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Cleft Palate Deformity and the Bone-Flap Method of Repair
Cleft Palate Deformity and the Bone-Flap Method of Repair LYNDON A. PEER, M.D., F.A.C.S.*
EFFORTS to prevent cleft palate and other deformities have been hindered in the past by the assumption that most congenital defects are determined by chance arrangement of genes and chromosomes. This has served to direct the efforts of research workers to other areas where the possibilities for success seem more promising. The results of a number of extensive studies regarding the cause of deformities in animals, however, indicate that maternal disease and other critical stresses occurring at certain stages during pregnancy may be responsible for a number of congenital abnormalities including the cleft lip and cleft palate. Of particular interest are the studies made on pregnant mice and pregnant rats; In the mouse and rat, acquired abnormalities have been produced in the offspring by subjecting the pregnant animal to a number of treatments with various agencies all of which are capable of producing congenital deformity.
ETIOLOGY OF CONGENITAL ABNORMALITIES
It has been established by means of animal experimentation that after the first week of development a regular pattern of congenital deformities can be produced by critical stress. As an example, Ingalls! induced fusion of the ribs on the ninth day of pregnancy, the period that was also critical for defects of the brain, skull and vertebral column. This same stress on the fifteenth day resulted in cleft palate and, if exerted near the end of pregnancy, it caused partial or complete ablation of the eyelids. The stress factor is defined rather broadly and includes oxygen deficiency, exposure to x-ray, vitamin deficiency, cortisone intoxication, and virus infection. As examples of stresses inducing deformity in animals, Warkany2 has produced eye defects and cleft palate by rendering From the Department of Plastic Surgery, St. Barnabas Medical Center, Newark, New Jersey. Sponsored by the John A. Hartford Foundation, Inc. * Clinical and Research Director, St. Barnabas Rehabilitation Center, Newark, New Jersey; Consultant in Plastic Surgery, St. Albans Naval Hospital, Long Island, New York. 313
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the pregnant animal vitamin-deficient. Baxter and Fraser have induced a high incidence of cleft palate deformity in the offspring of pregnant mice which were given cortisone. Ingallsl produced cleft palate and other deformities by rendering the pregnant animal anoxemic, or oxygendeficient. Thus, there are many possible consequences of embryonic injury and they vary with the time at which the adverse stress occurs in human beings as well as in mice. This is shown by the relation between rubella at five weeks of gestation and congenital cataract of the infant, and at nine weeks, between rubella and deafness. From this, one can assume that many congenitally deformed children may have survived a temporary intrauterine stress. Etiology of Cleft Palate and Cleft Lip Deformity
The human individual undoubtedly has an hereditary tendency to produce occasional cleft palate offspring. Statistically, this is expressed by the incidence of harelip or cleft palate deformity, or both, occurring about once in 1000 births. Approximately 25 per cent of the families will have a history of similar deformities on the paternal or maternal side of the family going back as far as grandparents. In about 75 per cent of the cases there is a negative family history for harelip or cleft palate deformity. When a family history of the deformity is present, the number of offspring having harelip or cleft palate is scattered and irregular, following no definite pattern. There is, however, a greater tendency for more than one child to be affected in families in which a positive history exists than in families in which there is no history of similar deformities. A reasonable assumption would seem to be that, in the 75 per cent of patients with a negative family history, two factors may be required to result in cleft-palate or cleft-lip offspring-the genetic racial tendency for cleft palate or lip deformity strengthened or enforced by a stress factor during the mother's pregnancy. The stress factor to be effective must be exerted at the particular time during pregnancy when the palatal processes are undergoing their rapid period of development. This rapid period of development is termed "the critical period" and stresses applied earlier or later do not as a rule produce cleft palate. The exact period of rapid development for various organs and tissues has been rather accurately determined in a number of animal species. In the human, however, where investigative work is not possible on an exact scale, the critical period for development is less definitely known. This naturally is so because studies on humans have been necessarily confined to the examination of aborted fetuses. There is evidence, however, that in human embryos the palatal shelves begin to close at the end of the seventh week of ovulation age or near the end of the ninth week of gestation as calculated from the onset of the last menses. 4
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Both stress during pregnancy as well as a remote congenital tendency for cleft-palate deformity may be necessary in about 75 per cent of the patients who give birth to cleft-palate infants. Thus it appears that this deformity can be a problem of preventive medicine, and prophylactic measures may be instituted which substantially reduce the incidence of cleftpalate and cleft-lip defects. Psychological Stress. Psychic trauma or psychological stress as a causative agent of cleft-palate deformity has been investigated by our group, by Warkany, and by others. The theory predicates that emotional upset whether caused by death of a relative, marital difficulties, or accidents induces excessive production of ACTH in the pituitary gland of the pregnant mother. This, in turn, stimulates the cortex of her adrenal gland to excrete large amounts of cortisone. The excess of cortisone during the early months of pregnancy, which are critical for the development of the palate, may inhibit fusion of the palatal shelves. Critics of the theory point out that inhibitory substances retard the production of ACTH by the pituitary gland when cortisone is present in excessive amounts, but it is possible that this balance may be defective in some pregnant women. It is known that daily injection of ACTH, independent of the activity of a patient's pituitary gland, will stimulate the adrenal gland to secrete cortical steroids, as determined in the 24-hour urine. This is used as a laboratory test, because a prompt rise in these steroids indicates that the adrenal cortex is responsive to stimulation and therefore that the adrenal cortex is not destroyed by tuberculous or primary cortical atrophy." Strean 6 has emphasized that pregnancy per se constitutes a stressful situation and as a result gravid women usually produce more than normal quantities of hydrocortisone. In the absence of a genetic influence, severe stress alone between the eighth and eleventh week of pregnancy may produce an abundance of hydrocortisone capable of initiating teratogenic effects. Although it is possible that psychological stress may be a factor in the etiology of cleft palate in humans, we were unable to produce such results in mice. For example, pregnant mice subjected to noise or air jets, and shaken up in buckets at regular intervals produced no cleftpalate offspring. These findings are in agreement with Warkany's investigation4 in which pregnant mice exposed to the sound of ringing bells at regular intervals failed to produce cleft-palate offspring. The administration of ACTH to pregnant mice by our group also failed to produce cleft palate in the offspring. This, however, is not surprising since various animals among different species differ in sensitivity of their response to ACTH and to cortisone. Homografts in rabbits are virtually uninfluenced by ACTH but are by cortisone. In guinea pigs
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Fig. 52. Cleft palate in 19-day embryo mouse. The mother mouse was given cortisone acetate from the 11th through the 14th day of her pregnancy. Note that cleft involves only the palate proper and does not extend through the alveolus or lip.
the reverse is true. Men and monkeys seem to react only to very large dosage. 7 MEASURES TO PREVENT CLEFT PALATE
It is an interesting fact that certain animals have cleft lip or cleft palate as a constant and normal anatomic formation in postfetal life. Thus the hare has a partial cleft lip or harelip and the chicken and other fowls have a partial cleft palate. A hare without a cleft lip or a chicken without a cleft palate could properly be considered abnormal. Cleft palate deformity can be produced in the offspring of pregnant mice but, oddly enough, a cleft lip is not associated with this cleft-palate defect. In our laboratory we have repeated the work of Baxter and Fraser,3 inducing 85 per cent occurrence of cleft palate in the offspring by subjecting pregnant mice to injections of cortisone acetate between the eleventh and fourteenth day of pregnancy (Fig. 52). We then conducted a series of experiments in which we gave individual B-vitamins concomitantly with the cortisone acetate to neutralize or render ineffective its teratologic effect. Riboflavin, thiamine, nicotinamide, B12 , pantothenic acid, choline, D-biotin, and inositol were ineffectual. When vitamin B6 was given together with cortisone acetate, however, the incidence of cleft palate in the offspring was reduced to 45 per cent. When folic acid was given concomitantly with cortisone
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Fig. 53. Normal palate in 19-day embryo mouse. The mother mouse received cortisone acetate and folic acid from the 11th through the 14th day of her pregnancy. Folic acid and vitamin Be both give protection against cleft palate in cortisonetreated mice. Eight other vitamins in the B group failed to give protection. These vitamins were riboflavin, thiamine, nicotinamide, B II, pantothenic acid, D-biotin, inol!litol and choline.
acetate, the incidence was reduced to 25 per cent. The administration of folic acid and Ba together with cortisone acetate resulted in further lowering of the incidence of cleft palate deformity in the offspring 8 (Fig. 53). Since vitamin Ba is a coenzyme necessary for amino acid metabolism and folic acid is required for the metabolism of purines, it seems evident that the palatal processes cannot properly fuse when these coenzymes are not present or physiologically functioning during the critical period of palatal growth. * The cortisone-treated mice that gave birth to cleft-palate offspring had been on a. diet containing Ba and folic acid. The administration of additional B6 and folic acid reduced the number of cleft-palate deformities. This indicates that cortisone in the amount given (2.5 mg. daily) may interfere only with the functions of a given amount of Do and folic acid. It is possible that some pregnant women, as a result of stress in the early months of pregnancy, secrete cortisone in sufficient amounts to inhibit palatal fusion in the embryo, even though they are receiving Bo and folic acid in their diets. Larger amounts of Be and folic acid may be * Based on the work of Walter Bryan, M.S., St. Barnabas Rehabilitation Center. Newark, N. J.
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effective in promoting the normal progress of protein metabolism with subsequent fusion of the palatal processes. Consequently we are routinely administering Ba and folic acid during a subsequent pregnancy to patients who have had a child with cleft lip-palate. Thus far, all of the offspring of these mothers have been normal,9 but the series, consisting of 50 normal children, is not large enough to be significant. For a number of years Douglas lO has been routinely administering vitamins to pregnant women who have children with cleft lip-palate. Conwayll gave an interesting report on similar studies at the 1958 meeting of the American Association of Plastic Surgeons in Dallas, Texas. His work indicated that the administration of balanced vitamins may be partially effective in preventing cleft lip-palate deformities. It seems probable that work now under way at a number of medical centers will clarify many aspects of the intrauterine environment or stress factor in the etiology of cleft palate during the next few years. This may result in therapeutic measures which will materially reduce the incidence of the abnormality, especially in the group of offspring with a negative family history. HISTORICAL BACKGROUND OF SURGICAL TREATMENT*
Cleft palate was described by a number of early physicians but Ie Monnier of Rouen was possibly the first to actually repair a cleft in the soft palate successfully. Robert l2 in 1766 refers to Ie Monnier's operation in the following words. "A child had had the palate cleft from the velum to the incisor teeth. M. Ie Monnier, a skilful dentist, endeavored and succeeded in reuniting the two borders of the cleft. He first inserted several sutures for approximation and afterwards abraded the margins with the actual cautery. An inflammation supervened, which terminated in suppuration, and was followed by reunion of the two lips of the artificial wound. The child was perfectly cured." Although Dubois, Colombe and others suggested methods for closure it was Carl Ferdinand von Graefe (1787-1840) (Fig. 54) who first introduced a comprehensive surgical method for closing clefts of the velum. Three years later, independently of von Graefe, Roux (PhilibertJoseph, 1780-1854) of Paris popularized staphylorrhaphy or suture of the soft palate defect, following his success in closing a cleft of the velum in a Canadian student. In 1821 Thomas Alcock performed the operation for the first time ,in England. Stevens (1827) of New York and Warren (1828) of Boston were the first to close soft-palate clefts in America. Palatal operations were limited to closure of simple cleft of the velum or soft palate until the year 1826, when Dieffenbach introduced his boneflap operation for uranoplasty or closure of clefts in the bony palate. This
* The historical background of surgical treatment is partly taken from Dorrance, George M.: The Operative Story of Cleft Palate, Philadelphia, W. B. Saunders Co., 1933.
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Fig. 54. Carl Ferdinand von Graefe (1787-1840). (Garrison from Dorrance.) This celebrated German surgeon introduced the first comprehensive surgical method for closing clefts of the velum or soft palate.
procedure was exceedingly simple and in harmony with embryological development in that it completed a union of the bony palatal processes which normally unite to form the hard palate. The later operation by von Langenbeck (1859) utilizing mucoperiosteal flaps, however, was favorably accepted by oral surgeons. Many of the designs in use even today are modifications of the von Langenbeck procedure. Von Langenbeck believed that bone regeneration occurred from the periosteum on the undersurface of his mucoperiosteal flaps and it probably does to some extent. That a considerable amount of anterior contracture or shortening of the palate occurs postoperatively, however, is evidenced by the numerous later modifications of von Langenbeck's technique such as push-back or lengthening maneuvers to compensate for the anterior contracture which is apt to occur. The Bone-Flap Method of Repair (Osteal Uranoplasty)
The operative story of the bone-flap procedure is rather easy to narrate since it can be accurately traced back to Dieffenbach (Fig. 55), to whom other writers, modifying his technique, gave due credit. Although the method was given up in Germany and other European countries, it later found a place in American surgery. In 1826 Dieffenbach13 demonstrated that a cleft involving the hard palate could be repaired by making lateral relaxation incisions through mucosa and bone. The denuded margins of the bone flaps were brought into firm apposition by means of wire sutures inserted through drill holes
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Fig. 55. Johann Friedrich Dieffenbach (1792-1847). (Courtesy of Dr. V. J. Bernbeck.) Palatal operations were limited to simple clefts of the velum until Dieffenbach introduced his bone-Hap procedure for closure of clefts in the hard palate.
in the bone. He described his suggested operation as actually performed in 1845 and advised that, if closure could not be accomplished at once, gradual approximation might be obtained by tightening the wire sutures at intervals. In 1850 Wutzer14 of Bonn combined Dieffenbach's boneflap closure of the hard palate defect (osteal uranoplasty) with closure of the soft palate defect in a single operation. In 1845 Dieffenbach described his management of hard and soft palate clefts in a comprehensive book, Die Operative Chirurgie. 16 He used no anesthesia and stressed the importance of sharp instruments to denude the free borders for relaxation through the hard and soft palate. He used the chisel to sever and infracture the bony palatal flaps, made lateral relaxation incisions in the soft palate, and inserted wire sutures through the drill holes in the bone. He also left an intentional anterior opening in all wide, complete palatal clefts. This technique is quite similar to that employed by Warren Davis. In Dieffenbach's words, "the purpose of the operation is to close the soft palate and as much of the hard palate as possible. The remaining anterior palate fistula may be covered by an obturator and later closed by further surgery. At the time of operation the patient is seated facing a window, his head being held by an assistant, and instructed to open his mouth and breathe in deeply, thereby pressing down on the floor of the mouth. After each incision he is allowed to recover, gasp for air and wash his mouth with cold water but not gargle because this would irritate the velum."* • Translated by V. J. Bernbeck, M.D., senior resident in plastic surgery at St. Barnabas Medical Center.
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Fig. 56. Bernhard Rudolf Konrad von Langenbeck (1810-1887). (Dorrance.) Langenbeck dissected the mucoperiosteum from the underlying bone in repairing cleft palate. The large majority of operations in use today are modifications of von Langenbeck's method.
Dieffenbach stated that the cleft-palate operation was first performed by von Graefe in 1816, and after him by Roux. He did not cite the single case reported by Ie Monnier described by Robert in 1766. The bone-flap method was used successfully by both Buhring 16 in 1850 and L6wenhardt 17 in 1857, but von Langenbeck18 (Fig. 56) in 1861 pointed out that the procedure was limited to cases of bilaterallip-jawpalate clefts. He thought that it was contraindicated in cases of unilateral lip-jaw-palate splits in which the vomer was attached to one side of the defect. From von Langenbeck's report, one is led to believe that the procedure was not successful in his hands. Billroth19 stated that the bone-flap procedure was no longer practiced, but Sir William Fergusson,2o Francis Mason,21 Lannelongue,22 Goodwillie23 and W oakes24 reported its successful use from 1873 to 1879. In the United States, in 1893, Mears26 of Philadelphia employed the bone-flap method for cleft palate repair and demonstrated his technique to G. V. 1. Brown (Fig. 57) and to Roe. Brown apparently was not impressed by the operation and employed the von Langenbeck method when he returned to practice in Milwaukee. Later, however, he became critical of his results with the von Langenbeck technique and began to use the bone-flap procedure almost exclusively in a one-stage type of repair. Roe utilized Mears' procedure in a large series of cleft palate patients and taught his modified technique to Warren Davis. Following Roe's death possibly the only two men in the world using the bone-flap tech-
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Fig. 57. George Van Ingen Brown (1862-1948). (Courtesy of Dr. V. P. Hyslop.) After Roe's death Brown and Warren Davis were probably the only men in the world using the bone-flap technique.
nique were Dr. G. V. 1. Brown of Milwaukee and Dr. Warren Davis of Philadelphia. These two men improved the operative method as devised by Dieffenbach and instructed other American surgeons .in its use. The author learned the bone-flap technique from Warren Davis (Fig. 58) and is currently demonstrating it to his senior resident, Dr. V. J. Bernbeck, who will reintroduce the procedure in Germany, where it originated. The bone-flap operation as used by our group has been modified to some extent but the technique is essentially the same as that of Warren Davis. The author first observed Warren Davis' technique some 20 years ago, when with Staige Davis, Kitlowski and Straatsma he was invited to visit Jefferson Hospital. Warren Davis* performed seven one- and twostage operations during the morning and in the afternoon demonstrated his postoperative results with some randomly selected patients in his office. We were all impressed by the speech of these patients and the
* Warren Davis was an exceptionally skillful cleft-palate operator and had developed a smooth operating team. His anesthetist, wearing a sterile glove on one hand, held a retractor and John Reese, of Reese Dermatome fame, served as first assistant. He controlled bleeding by packing the lateral relaxation incisions through the palatal shelves and posterior palatal artery with sponges saturated with Adrenalin solution (1 :1000). This quickly controlled bleeding, and so enabled him to complete his firststage procedure in 3 minutes and the second stage closure in 20 minutes. Davis, a gentleman of the old school, acknowledged indebtedness to his teacher Dr. Roe, whom he described as a skillful technician who had never written a paper describing the bone-flap repair.
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Fig. 58. Warren B. Davis (1881-1947). The present author learned the bone-flap technique from Warren Davis. Davis followed John Wheeler as President of the American Society of Plastic and Reconstructive Surgery and was the first Editor of the Society Journal.
normal appearance and function of the palates. The author has used Warren Davis' bone-flap technique with some modifications since that time. OPERATIVE TECHNIQUE
Complete Bilateral Palatal and Alveolar Clefts
The palatal defect is wide and the palatal shelves are often rather narrow. Consequently a two-stage procedure is always indicated to insure adequate blood supply for the flaps. Ali intentional fistula will remain anteriorly after the second operation, in which the palatal shelves are joined at the midline. In the first operation incisions are made medial and parallel to the alveolar ridges, leaving a rim of oral mucous membrane between the incision and the erupted or unerupted teeth. A Warren Davis chisel is then inserted into this incision and the bony palatal processes with their covering nasal mucous membrane are severed from their lateral attachments and infractured toward the midline. The hamular process and posterior palatal artery are separated during the chiseling maneuver but bleeding is quickly controlled by inserting gauze sponges soaked in dilute Adrenalin solution. When all bleeding has ceased, the relaxation openings at the sides are packed with Oxycel, and the patient sent to the recovery room with a temporary
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suture inserted in the tongue as an aid to nurses in opening the mouth and using suction for secretion or blood in the pharynx. The second operation is performed two weeks later. The free margins of the palatal flaps are denuded of mucous membrane and small drill holes are made in the bony palatal shelves. The fibrous adhesions which have formed in the lateral relaxation areas are separated and 000 silk sutures inserted through the drill holes. The soft palate processes are then freed from their lateral attachment by extending the relaxation incisions posteriorly until the soft palatal processes can be approximated without undue tension. Number 000 chromic catgut sutures are then inserted through nasal mucous membrane and muscle from near the posterior border of the bony palate to the tip of the uvula. These sutures are tied so that the knots are on the surface of the nasal mucous membrane. The three or four silk sutures which were previously inserted through drill holes in the bony palate are tied, which approximates about threefourths of the bony palate, leaving an intentional anterior fistula. This will be closed one year later. Number 000 chromic catgut sutures are then inserted into the oral surface of the soft palate from the tip of the uvula to the posterior border of the bony palate. These catgut sutures include mucous membrane and a generous portion of muscle. The relaxation openings at the sides are packed with Oxycel and a temporary suture is again inserted into the tongue as an aid for nurses in aspirating mucus and blood from the child's pharynx. The catgut sutures always loosen and are usually swallowed by the child about two weeks following operation. The silk sutures in the bone are removed three weeks after the operation. Complete Unilateral Palatal and Alveolar Clefts (Figs. 59 and 60)
In these cases also we perform the two-stage procedure two weeks apart. The technique is the same as that used for complete bilateral clefts except for the management of the vomer, which is attached to the bony palatal process on the non-cleft side. This vomer attachment is not disturbed at the time of the first operation, in which relaxation incisions are made through both bony palatal shelves. At the time of the second operation, however, the vomer is severed from the palatal process with a chisel, which permits infracture of the freed bony palatal process on the non-cleft side. The technique of closure is then exactly the same as that used for the second-stage repair of complete bilateral palatal and alveolar cleft palate. Severing the vomer from the bony palatal process, in effect, converts a unilateral cleft palate into a bilateral cleft palate. Warren Davis always used a mucoperiosteal flap on the non-cleft side in these patients. He joined the mucoperiosteal flap to a bone flap which
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Fig. 59. First operation in a two-stage bone-flap repair of a complete 'unilateral cleft palate. ' A, First inject mucous membrane with normal saline to which 8 drops of Adrenalin per ounce has been added. Incisions are then made parallel to the alveolar ridge through both oral mucous membrane and periosteum. Note that base of septum is attached to palatal process on the left side which classifies this case as a unilateral cleft palate. B, A Warren Davis chisel is inserted through the incision on the right side at an angle inward to avoid injuring tooth buds in the alveolar ridge. The chisel is tapped with a hammer to completely separate the bone and nasal mucous membrane from their lateral attachments. The hamular process and palatine artery are severed and the incision extended posteriorly in the soft palate. A sponge saturated in normal saline with 8 drops of Adrenalin to the ounce is packed in the relaxation incision. This should be held firmly for a few minutes to control bleeding before separating the opposite bony palatal process. C, Oxycel cotton is inserted in the lateral relaxation incision and held firmly until all bleeding has ceased. Child is sent to recovery room with a suture through the tongue as an aid to nurses for opening the mouth and sucking out blood or mucus. Endotracheal ether-oxygen anesthesia is used in all cases.
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Fig. 60. Second operation in a two-stage bone flap repair of a complete unilateral cleft palate. An interval of 14 days elapses between the first and second operation for development of an adequate blood supply in the flaps. A, The base of the septum is separated from the bony palatal process on the left side. This converts the unilateral cleft to a bilateral cleft. The relaxation incisions at the sides are reopened and the bony palatal shelves infractured so that the free margins can be approximated in the midline without undue tension. a, b, c, The free borders of the palatal shelves are denuded of their mucous membrane covering. 1, 2, 3, 4, Three drill holes have been made through the bony palatal processes on each side. Drawings illustrate technique used to insert 00 heavy silk sutures through these drill holes. B, First, the nasal and then the oral mucous membrane is drawn together with 000 chromic catgut. These sutures include the muscle layer. If the soft palate is short or the soft palatal processes unequal in length, the Z-plasty technique is used, as illustrated in Figure 61. This may be combined with the introduction of a dermal graft beneath the pharyngeal mucous membrane in the area of Passavant's cushion. The 3 heavy silk sutures through drill holes in the bone are then tied to bring the denuded surfaces of the bony palatal shelves together in the midline. In wide complete palatal and alveolar clefts it is often expedient not to attempt closure of the anterior onefifth of the bony palatal cleft. This leaves an intentional anterior fistula which may be closed about. 1 year later with rotation mucous membrane flaps. Oxycel gauze is introduced into the lateral relaxation incisions to control bleeding and the child sent to the recovery room with a suture in the tongue.
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he formed on the cleft side. I suggested the procedure of severing the vomer-palate connection to him a number of times but, although he approved of the principle, I do not recall that he ever used it. * IncOInplete and Complete Posterior Alveolar Cleft Palate (Fig. 61)
The repair of these clefts is essentially the same as that used for complete palatal and alveolar clefts except that they are usually closed in a single operation. The number of sutures inserted into the bony palatal shelves varies with the length of the cleft, and one can usually obtain complete closure of the bony palate without leaving an intentional anterior palatal fistula. The soft palate is never separated from the posterior ~argin of the bony palate. We prefer to infracture the bony palatal shelves and insert a single suture through the bone for approximation of even a small opening or notch in the bony palate. Z-Plasty for Short Palate
When the soft palate segments are short or unequal in length, a Z-plasty is routinely performed to obtain greater length in both segments or establish greater length on the short side. The incisions to form the Z-designs include the muscle and both oral and nasal mucous membrane. Hence, after the flaps have been sutured in position no raw surfaces exist which might give rise to anterior contraction and shortening of the palate. Careful examination of these patients postoperatively does not show any impairment in muscular movement. We believe that these children have less tendency to nasality than do a comparable group with short soft palates in which the short palatal segments are merely approximated. Variations in Technique
In extensive clefts of the palate we occasionally use a vomer flap combined with the bone-flap technique at the time of the second operation. Usually, however, we leave an intentional opening in front and close this later with overlapping detached flaps elevated from the underlying bone. In individual cases of incomplete cleft palate the decision whether to do a one- or two-stage procedure may present some difficulties. In complete palatal-alveolar clefts, either unilateral or bilateral, it is always safer to do a two-stage procedure. In older patients who have had no palatal repairs we also tend to use a two- or even a three-stage procedure because of the width of the palatal defect and the importance of an adequate blood supply for the flaps. In these older, unrepaired cleft-palate defects it is often possible to close only the soft palate and a small portion
* Dr. Hyslop has informed me that Dr. G. V. I. Brown always detached the vomer from the palatal process on the non-cleft side to facilitate infracture. Dr. Hyslop was at one time assistant to Dr. Brown and has continued to use his bone-flap technique.
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Fig. 61. Postalveolar short cleft palate with defect extending partly or completely through bony palate. A, The free margins of the palatal processes have been denuded of mucous membrane and relaxation incisions through the bony and soft palatal shelves have been made on each side. Right-angle incisions through the complete thickness of the soft palate form the pointed flaps 1 and 2. Variations in the angles of these flaps are used when one soft palatal process is shorter than the other to establish equal length. B, The bony palatal shelves are infractured and 2 silk sutures have been introduced through drill holes in the bone (see Fig. 60). C, D, Chromic catgut sutures (000) are inserted through the nasal mucous membrane and muscle. When these are tied the knots will be on the nasal surface. E, Chromic catgut sutures (000) through oral mucous membrane and muscle are tied to approximate the oral surface of the soft palate. The 2 heavy silk sutures through drill holes in the bone are tied to close the palatal cleft completely. When shortening of the velum is marked, the Z-plasty is combined with the introduction of a dermal graft beneath the pharyngeal mucous membrane in the region of Passavant's cushion.
of the bony palate. The large defect remaining anteriorly is covered with a dental prosthesis. Since all of these unrepaired cleft palates are short, we do the pharyngeal flap or a Z-plasty procedure at a later date and follow with speech therapy. The advisability of operating on older, unrepaired cleft palates is an individual problem. When the defect is so
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wide that closure of the soft palate seems problematical, then it is best to advise the patient to consult a prosthodontist and have an artificial appliance made. Orthodontia
Consultation with an orthodontist should go hand-in-hand with surgical repair of the palate, especially in the cases of palatal clefts of a complete type extending through the alveolus and disrupting the integrity of the alveolar arch. Bilateral palatal clefts involving the alveolar arch which are associated with a complete double cleft lip present unusually difficult problems, and the assistance of an orthodontist is essential to place and maintain the middle segment or prolabium properly in alignment as the anterior central segment of the alveolar arch. RESULTS WITH THE BONE-FLAP TECHNIQUE
It is extremely difficult to compare the results of various types of operation for closure of cleft-palate defects. This is so because individua1 plastic surgeons mainly use a single technique which they have learned from their preceptor during their training period. Consequently, they have no basis for comparison excepting occasional cases in which repair has been carried out elsewhere by other methods, and these are generally not seen in large numbers. In evaluating the bone-flap technique, which has been employed by the author for 20 years, it may be said to be a rather simple procedure, which gives uniformly good results in respect to closure of the palate and good functional movement of the soft palate. Because a segment of bone with oral and nasal mucous-membrane covering is brought over on each side to the midline and the soft palate attachment to the posterior edge of the bony palate is left intact, there is little, if any, tendency to anterior contracture or shortening of the palate following operation. The procedure, however, does not provide for additional lengthening, and if one starts with a short palate, one ends up with a palate of the same length. If the shortening is marked, we lengthen the soft palate with a Z-plasty, as described under operative technique. The incisions for this Z-plasty include muscle and both mucous-membrane surfaces. When the children are older, secondary operations to close the intentional anterior fistula, which could not be closed at the time of the primary operation, demonstrate that the bone-flap shift results in a firm bony union of the palatal shelves in the midline. This facilitates closure of the anterior fistula, since mucous-membrane flaps can be elevated from the bone and sutured to cover the palatal opening. The area at the side where the bony palate was separated and infractured for relaxation purposes also fills in with bone. Thus, in all areas of the hard palate
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where bone normally should be, bone is ultimately present except in the area of the anterior fistula. This may be of some advantage as a soundingboard effect in speech; certainly it is a distinct advantage in closing the intentional anterior palatal fistula, since any flaps raised will expose a surface of bone rather than create an opening into the nasal cavity. Speech Evaluation
Comparison of the quality of speech following the bone-flap technique with that following other types of palatal operations presents numerous problems. The only type of repair made by other techniques elsewhere which we see in any considerable number at the St. Barnabas Rehabilitation Center is the von Langenbeck procedure. All of these patients seem to have considerably more shortening and scarring in the palate, and the over-all quality of speech is not as good as that obtained following the bone-flap procedure. This is in conformity with the author's early experience regarding the quality of speech in his own patients repaired by the von Langenbeck method. Comparison of the bone-flap method with the Wardell type of operation or other operations involving closure and lengthening in the same procedure cannot be made with any degree of accuracy. Critical evaluation by our speech therapists of all cleft palate patients repaired on our service, either by staff members or senior residents, demonstrates that 30 per cent require no speech therapy; 70 per cent do. A large number of the patients who are judged to require speech therapy have only minor speech defects, which are readily corrected by therapy. On the small number of patients who do not respond adequately to the therapy we do a pharyngeal flap procedure, as described by Moran, or an extension Z-plasty if the palate is short. In most instances this improves the quality of speech; speech therapy is always instituted following the lengthening operation. OTHER CLINICAL CONSIDERATIONS
Age of Child at Operation
A few years ago considerable controversy prevailed, especially in the United States, regarding the proper age for operation on palatal clefts. One group with a considerable following claimed that cephalometric examinations of the maxillae of patients operated on before the fifth year of age demonstrated considerable underdevelopment of the upper jaw and they advised against early operation. Cephalometric examinations were made by the orthodontic department at St. Barnabas Rehabilitation Center on 94 patients operated upon by the bone-flap technique before the age of two years. Similar studies were made on 44 patients operated upon elsewhere by the von
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Langenbeck method before the age of two years.26 In neither group did we find a significant underdevelopment of the maxillae. Accordingly, we operate on all cleft-palate patients who are in good physical condition between the ages of seven to 14 months, depending on the width of the cleft and the development of the palatal shelves. It is of considerable advantage to close the cleft before the child begins to talk. Malocclusion in Cleft Palate Deform.ity
Studies by our orthodontist, Dr. Michael Collito, indicate that crowding and crossbite are typical malocclusions associated with cleft palate. whether surgically treated or not. Only one patient in six is free of malocclusion, which suggests the need for orthodontia regardless of the type of surgical repair. Neither crowding nor crossbite is related to surgical trauma. Both are related, however, to the type of original defect, since the relative incidence of both deformities increases sharply with the increasing severity of the original defect. 26 Change in surgical technique cannot be expected to affect the incidence of malocclusion. Nor does age at operation affect either its incidence or its severity. Orthodontic management in general should begin after eruption of the secondary teeth except in patients with complete bilateral alveolar clefts. These require very early orthodontic treatment to maintain the middle segment in proper relationship with the alveolar arch. Hearing of Cleft Palate Patients
Sixty per cent of the children with all types of cleft palate deformity appearing at our clinic have a significant hearing loss. The deafness is usually of the conductive type and is associated with a history of repeated colds and the presence of enlarged and often infected tonsils and adenoids. Young children who do not hear well will not learn to speak well, and the child with a cleft palate and a significant hearing loss presents a serious problem. Our management has consisted of first attempting to immunize children against infection of the upper respiratory tract with vaccines and general preventive measures. When this fails, we remove the tonsils and the adenoid tissue around the eustachian tubes, leaving the centra adenoid mass in the region of "Passavant's cushion." Recently we have routinely sutured the tonsillar pillars together to avoid excess scar formation in the denuded tonsillar fossae. Patients who have established good speech before operation are not usually affected; children who have poor speech before operation may be adversely affected by. removal of too much adenoid tissue especially in the region of Passavant's cushion.
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A high percentage of our cleft palate children in the New Jersey area have a low-grade or chronic sinusitis. This is treated by local medication to promote drainage and general measures to increase the child's resistance. The outlook for most of the 60 per cent of cleft palate children with significant hearing losses is not a happy one, since the deafness usually is progressive despite therapy. Psychological Screening of Cleft Palate Children
Our patients with cleft palate defects, as a group, had a mean intelligence quotient (by Stanford-Binet tests) slightly higher than that of a comparable group of children without deformity. Social maturity was low, which we attributed to the effect of imperfect speech and the concomitant cleft-lip deformity (when this was present) on the adjustment of these children with their playmates. In many instances patients become most aware of the fact that they are different from other children after entering school, usually in the first or second grade. This indicates the importance of doing everything possible to have them at their best regarding speech and appearance before the school period begins. CONCLUSIONS
1. The results of a number of studies on animals indicate that maternal disease and other critical stresses occurring at certain stages during pregnancy may be responsible for various types of congenital abnormalities including cleft palate. 2. Cortisone given to pregnant mice induces a high incidence of cleft palate deformity in the offspring. Specific vitamins were administered to pregnant mice concomitantly with cortisone acetate to render ineffective its teratologic effect. Of the vitamins tested only B6 and folic acid reduced the incidence of cleft palate in the offspring. 3. Based on the protective effects of vitamins B6 and folic acid in cortisone-treated mice, we are routinely giving these two vitamins during the first four months of a subsequent pregnancy to patients who have had a cleft lip-palate child. Thus far, all of the offspring of these mothers have been normal but the series of 50 normal infants is not large enough to be significant. 4. The bone-flap method of repair originated by Dieffenbach in 1826 was possibly the first technique to close a cleft in the hard or bony palate successfully. 5. In our experience the bone-flap technique of the late Dr. Warren Davis is a very satisfactory method of repair for cleft palate. 6. When the soft palatal processes are short or unequal in length a Z-plasty is useful to obtain greater length in both .segments or establish greater length on the short side.
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7. Postoperative evaluation studies on a varied age group of our cleft palate patients is summarized as follows: (a) Speech. Thirty per cent had normal speech and 70 per cent had speech defects. A large majority of the patients with defective speech responded favorably to therapy before the school age (five to six years). (b) Hearing. Sixty per cent had a significant hearing loss. (c) Psychological Evaluation. The LQ. (by Stanford-Binet tests) of children with cleft palate at our clinic was slightly higher than that of a comparable group of children without congenital deformity. (d) Facial Bone Development. Operative repair by the bone-flap technique before the age of 20 months was not associated with significant underdevelopment of the maxillae. (e) Orthodontia. Malocclusion is very frequently associated with cleft palate deformity whether surgically treated or not. Malocclusion is related to the type of original defect, since its relative incidence increases sharply with the increasing severity of the cleft palate deformity. REFERENCES 1. Ingalls, T. H.: Causes and Prevention of Developmental Mental Defects,
J.A.M.A. 161: 1047 (July 14) 1956. 2. Warkany, J.: Congenital Malformations Induced by Matgrnal Nutritional Deficiency. J. Pediat. 25: 476 (Dec.) 1944. 3. Baxter, H. and Fraser, R. G.: Production of Congenital Defects in Offspring of Female Mice Treated with Cortisone. McGill M.J. 19: 245, 1950. 4. Warkany, Josef: Personal communication regarding his unpublished experimental work. 5. Hurxthal, Lewis M.: Practical Endocrinology. New York, McGraw-Hill Book Co., 1955, p. 148. 6. Strean, L. P., and Peer, L. A.: Stress as an Etiologic Factor in the Development of Cleft Palate. Plast. & Reconstruct. Surg. 18: 1 (July) 1956. 7. Medawar, P. B.: Zoological Laws. In Vol. II, Transplantation of Tissues, Lyndon A. Peer, Ed., Baltimore, Williams & Wilkins Co. In press. 8. Peer, L. A. and others: The Induction of Cleft Palate in Mice by Cortisone and Its Reduction by Vitamins. J. Internat. Coll. Surgeons 30: 249 (Aug.) 1958. 9. Peer, L. A. and others: Study of 400 Pregnancies with Birth of Cleft Lip-Palate Infants. Plast. & Reconstruct. Surg. 22: 442 (Nov.) 1958. 10. Douglas, Beverly: Monograph: Harelip and Cleft Palate. From Division of Plastic Surgery, School of Medicine, Vanderbilt University, Nashville, Tennessee. Privately printed. 11. Conway, H.: Effect of Supplemental Vitamin Therapy on the Limitation of Incidence of Cleft Lip and Cleft Palate in Humans. Plast. & Reconstruct. Surg., 22: 450 (Nov.) 1958. 12. Robert: Traite de principaux objets de medecine avec un summaire de la plupart des tMses soutenues aux ecoles de Paris, depuis 1752 jusqu'en 1764: on y a joint des observations de pratique, 1: 8, premier observation, Paris, 1766, Lecombe. See also errata appended to vol. ii. 13. Dieffenbach, J. F.: "Ober'das Gaumensegel des Menschen und der Saeugethiere. Litt. Ann. d. ges. Heilk. 4: 298, 1826, abstr. Lancet 10: 811,1826. Idem: Beitrage zur Gaumennath. Litt. Ann. d. ges. Heilk. 4: 145, 1826. Idem: Beitrii.ge zur Gaumennath. Litt. Ann. d. ges. Heilk. 6: 305,1826. Cited by Dorrance. 14. Wutzer, C. W.: Heilung der angeborenen SpaUe des weichen und harten Gaumens durch Operation. Deutsche Klin. 2: 260, 1850.
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15. Dieffenbach, J. F.: Die Operative Chirurgie. Vol. 1. Leipzig, F. A. Brockhaus, 1845. Translated by V. J. Bernbeck, M.D., senior resident at St. Barnabas Medical Center. 16. BUhring, J.: Die organische Schliessung des durchbrochenen harten Gaumens vermittelst Knochensubstanz. J. d. Chir. u. Augenh. n.s. 9: 329, 1850. Idem: Beitrage zum organischen Wiederersatz der Defecte des harten Gaumens vermittelst Knochensubstanz. Deutsche Klin. 2: 472,1850. 17. Lowenhardt: Staphyloraphie und versuchte Staphyloplastik. Deutsche Klin. 9: 137, 1857. 18. von Langenbeck, Bernhard: Professor Langenbeck on UranopIastica. Med. Times & Gaz. 2: 69, 1861. Idem: Operation der angeborenen totalen Spaltung des harten Gaumens nach einer neuer Methode. Deutsche Klin. 8: 231, 1861. 19. Billroth, Theodore: tl"ber osteoplastische Operationen. Wien. med. Presse 9: 1057, 1058, 1073-1076, 1868. . 20. Fergusson, Sir William: Observations on Harelip and Cleft Palate. Retrospect of Practical Med. & Surg. 23: 158, 1852. Idem: Cleft Palate. M. Times & Gaz. n.s. 4: 433, 1852. Idem: A New Operation for Cleft Palate. Lancet 2: 784,1873; Month. Rev. Dent. Surg. 311, 1873-1874. Idem: Observations on Harelip and Cleft Palate. Brit. M.J. 1: 403, 437, 1874; Month. Rev. Dent. Surg. 2: 481, 1873-1874. 21. Mason, Francis: Clinical Remarks on Cleft Palate. Lancet 2: 578,1874. Idem: The Treatment of Cleft Palate. Lancet 2: 170, 1876. Idem: On Harelip and Cleft Palate. London, 1877. Idem: Cleft Palate. In: On Surgery of the Face. London, 1878, p. 104. 22. Lannelongue: Restauration de la vo'O.te palatine. Bull. et m~m. Soc. de chir. n.s. 2: 469, 1876. 23. Goodwillie, D. H.: A Case of Congenital Cleft of the Hard and Soft Palate, with Extensive Hypertrophy of the Left Inferior Turbinate Bone. Tr. Med. Soc. New York, 93,1877. 24. Woakes, Edwards: Case of Operation for Cleft Palate Illustrating aNew Method of Performing Osteoplasty. M. Times & Gaz. 2: 526,1879. 25. Mears, J. E.: Clefts of the Hard and Soft Palates. Read before the Philadelphia Academy of Surgery, 1893. South. M. Rec. 24: 59, 1894. 26. Peer, L. A., Hagerty, R., Hoffmeister, F. S. and Collito, M. D.: Repair of Cleft Palate by the Bone Flap Method. J. Internat. ColI. Surgeons 22: 463 (Oct.) 1954. 120 Halstead Street East Orange, New Jersey
EFFORTS to prevent cleft palate and other deformities have been hindered in the past by the assumption that most congenital defects are determined by chance arrangement of genes and chromosomes. This has served to direct the efforts of research workers to other areas where the possibilities for success seem more promising. The results of a number of extensive studies regarding the cause of deformities in animals, however, indicate that maternal disease and other critical stresses occurring at certain stages during pregnancy may be responsible for a number of congenital abnormalities including the cleft lip and cleft palate. Of particular interest are the studies made on pregnant mice and pregnant rats; In the mouse and rat, acquired abnormalities have been produced in the offspring by subjecting the pregnant animal to a number of treatments with various agencies all of which are capable of producing congenital deformity.
ETIOLOGY OF CONGENITAL ABNORMALITIES
It has been established by means of animal experimentation that after the first week of development a regular pattern of congenital deformities can be produced by critical stress. As an example, Ingalls! induced fusion of the ribs on the ninth day of pregnancy, the period that was also critical for defects of the brain, skull and vertebral column. This same stress on the fifteenth day resulted in cleft palate and, if exerted near the end of pregnancy, it caused partial or complete ablation of the eyelids. The stress factor is defined rather broadly and includes oxygen deficiency, exposure to x-ray, vitamin deficiency, cortisone intoxication, and virus infection. As examples of stresses inducing deformity in animals, Warkany2 has produced eye defects and cleft palate by rendering From the Department of Plastic Surgery, St. Barnabas Medical Center, Newark, New Jersey. Sponsored by the John A. Hartford Foundation, Inc. * Clinical and Research Director, St. Barnabas Rehabilitation Center, Newark, New Jersey; Consultant in Plastic Surgery, St. Albans Naval Hospital, Long Island, New York. 313
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the pregnant animal vitamin-deficient. Baxter and Fraser have induced a high incidence of cleft palate deformity in the offspring of pregnant mice which were given cortisone. Ingallsl produced cleft palate and other deformities by rendering the pregnant animal anoxemic, or oxygendeficient. Thus, there are many possible consequences of embryonic injury and they vary with the time at which the adverse stress occurs in human beings as well as in mice. This is shown by the relation between rubella at five weeks of gestation and congenital cataract of the infant, and at nine weeks, between rubella and deafness. From this, one can assume that many congenitally deformed children may have survived a temporary intrauterine stress. Etiology of Cleft Palate and Cleft Lip Deformity
The human individual undoubtedly has an hereditary tendency to produce occasional cleft palate offspring. Statistically, this is expressed by the incidence of harelip or cleft palate deformity, or both, occurring about once in 1000 births. Approximately 25 per cent of the families will have a history of similar deformities on the paternal or maternal side of the family going back as far as grandparents. In about 75 per cent of the cases there is a negative family history for harelip or cleft palate deformity. When a family history of the deformity is present, the number of offspring having harelip or cleft palate is scattered and irregular, following no definite pattern. There is, however, a greater tendency for more than one child to be affected in families in which a positive history exists than in families in which there is no history of similar deformities. A reasonable assumption would seem to be that, in the 75 per cent of patients with a negative family history, two factors may be required to result in cleft-palate or cleft-lip offspring-the genetic racial tendency for cleft palate or lip deformity strengthened or enforced by a stress factor during the mother's pregnancy. The stress factor to be effective must be exerted at the particular time during pregnancy when the palatal processes are undergoing their rapid period of development. This rapid period of development is termed "the critical period" and stresses applied earlier or later do not as a rule produce cleft palate. The exact period of rapid development for various organs and tissues has been rather accurately determined in a number of animal species. In the human, however, where investigative work is not possible on an exact scale, the critical period for development is less definitely known. This naturally is so because studies on humans have been necessarily confined to the examination of aborted fetuses. There is evidence, however, that in human embryos the palatal shelves begin to close at the end of the seventh week of ovulation age or near the end of the ninth week of gestation as calculated from the onset of the last menses. 4
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Both stress during pregnancy as well as a remote congenital tendency for cleft-palate deformity may be necessary in about 75 per cent of the patients who give birth to cleft-palate infants. Thus it appears that this deformity can be a problem of preventive medicine, and prophylactic measures may be instituted which substantially reduce the incidence of cleftpalate and cleft-lip defects. Psychological Stress. Psychic trauma or psychological stress as a causative agent of cleft-palate deformity has been investigated by our group, by Warkany, and by others. The theory predicates that emotional upset whether caused by death of a relative, marital difficulties, or accidents induces excessive production of ACTH in the pituitary gland of the pregnant mother. This, in turn, stimulates the cortex of her adrenal gland to excrete large amounts of cortisone. The excess of cortisone during the early months of pregnancy, which are critical for the development of the palate, may inhibit fusion of the palatal shelves. Critics of the theory point out that inhibitory substances retard the production of ACTH by the pituitary gland when cortisone is present in excessive amounts, but it is possible that this balance may be defective in some pregnant women. It is known that daily injection of ACTH, independent of the activity of a patient's pituitary gland, will stimulate the adrenal gland to secrete cortical steroids, as determined in the 24-hour urine. This is used as a laboratory test, because a prompt rise in these steroids indicates that the adrenal cortex is responsive to stimulation and therefore that the adrenal cortex is not destroyed by tuberculous or primary cortical atrophy." Strean 6 has emphasized that pregnancy per se constitutes a stressful situation and as a result gravid women usually produce more than normal quantities of hydrocortisone. In the absence of a genetic influence, severe stress alone between the eighth and eleventh week of pregnancy may produce an abundance of hydrocortisone capable of initiating teratogenic effects. Although it is possible that psychological stress may be a factor in the etiology of cleft palate in humans, we were unable to produce such results in mice. For example, pregnant mice subjected to noise or air jets, and shaken up in buckets at regular intervals produced no cleftpalate offspring. These findings are in agreement with Warkany's investigation4 in which pregnant mice exposed to the sound of ringing bells at regular intervals failed to produce cleft-palate offspring. The administration of ACTH to pregnant mice by our group also failed to produce cleft palate in the offspring. This, however, is not surprising since various animals among different species differ in sensitivity of their response to ACTH and to cortisone. Homografts in rabbits are virtually uninfluenced by ACTH but are by cortisone. In guinea pigs
to
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Fig. 52. Cleft palate in 19-day embryo mouse. The mother mouse was given cortisone acetate from the 11th through the 14th day of her pregnancy. Note that cleft involves only the palate proper and does not extend through the alveolus or lip.
the reverse is true. Men and monkeys seem to react only to very large dosage. 7 MEASURES TO PREVENT CLEFT PALATE
It is an interesting fact that certain animals have cleft lip or cleft palate as a constant and normal anatomic formation in postfetal life. Thus the hare has a partial cleft lip or harelip and the chicken and other fowls have a partial cleft palate. A hare without a cleft lip or a chicken without a cleft palate could properly be considered abnormal. Cleft palate deformity can be produced in the offspring of pregnant mice but, oddly enough, a cleft lip is not associated with this cleft-palate defect. In our laboratory we have repeated the work of Baxter and Fraser,3 inducing 85 per cent occurrence of cleft palate in the offspring by subjecting pregnant mice to injections of cortisone acetate between the eleventh and fourteenth day of pregnancy (Fig. 52). We then conducted a series of experiments in which we gave individual B-vitamins concomitantly with the cortisone acetate to neutralize or render ineffective its teratologic effect. Riboflavin, thiamine, nicotinamide, B12 , pantothenic acid, choline, D-biotin, and inositol were ineffectual. When vitamin B6 was given together with cortisone acetate, however, the incidence of cleft palate in the offspring was reduced to 45 per cent. When folic acid was given concomitantly with cortisone
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i'
Fig. 53. Normal palate in 19-day embryo mouse. The mother mouse received cortisone acetate and folic acid from the 11th through the 14th day of her pregnancy. Folic acid and vitamin Be both give protection against cleft palate in cortisonetreated mice. Eight other vitamins in the B group failed to give protection. These vitamins were riboflavin, thiamine, nicotinamide, B II, pantothenic acid, D-biotin, inol!litol and choline.
acetate, the incidence was reduced to 25 per cent. The administration of folic acid and Ba together with cortisone acetate resulted in further lowering of the incidence of cleft palate deformity in the offspring 8 (Fig. 53). Since vitamin Ba is a coenzyme necessary for amino acid metabolism and folic acid is required for the metabolism of purines, it seems evident that the palatal processes cannot properly fuse when these coenzymes are not present or physiologically functioning during the critical period of palatal growth. * The cortisone-treated mice that gave birth to cleft-palate offspring had been on a. diet containing Ba and folic acid. The administration of additional B6 and folic acid reduced the number of cleft-palate deformities. This indicates that cortisone in the amount given (2.5 mg. daily) may interfere only with the functions of a given amount of Do and folic acid. It is possible that some pregnant women, as a result of stress in the early months of pregnancy, secrete cortisone in sufficient amounts to inhibit palatal fusion in the embryo, even though they are receiving Bo and folic acid in their diets. Larger amounts of Be and folic acid may be * Based on the work of Walter Bryan, M.S., St. Barnabas Rehabilitation Center. Newark, N. J.
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effective in promoting the normal progress of protein metabolism with subsequent fusion of the palatal processes. Consequently we are routinely administering Ba and folic acid during a subsequent pregnancy to patients who have had a child with cleft lip-palate. Thus far, all of the offspring of these mothers have been normal,9 but the series, consisting of 50 normal children, is not large enough to be significant. For a number of years Douglas lO has been routinely administering vitamins to pregnant women who have children with cleft lip-palate. Conwayll gave an interesting report on similar studies at the 1958 meeting of the American Association of Plastic Surgeons in Dallas, Texas. His work indicated that the administration of balanced vitamins may be partially effective in preventing cleft lip-palate deformities. It seems probable that work now under way at a number of medical centers will clarify many aspects of the intrauterine environment or stress factor in the etiology of cleft palate during the next few years. This may result in therapeutic measures which will materially reduce the incidence of the abnormality, especially in the group of offspring with a negative family history. HISTORICAL BACKGROUND OF SURGICAL TREATMENT*
Cleft palate was described by a number of early physicians but Ie Monnier of Rouen was possibly the first to actually repair a cleft in the soft palate successfully. Robert l2 in 1766 refers to Ie Monnier's operation in the following words. "A child had had the palate cleft from the velum to the incisor teeth. M. Ie Monnier, a skilful dentist, endeavored and succeeded in reuniting the two borders of the cleft. He first inserted several sutures for approximation and afterwards abraded the margins with the actual cautery. An inflammation supervened, which terminated in suppuration, and was followed by reunion of the two lips of the artificial wound. The child was perfectly cured." Although Dubois, Colombe and others suggested methods for closure it was Carl Ferdinand von Graefe (1787-1840) (Fig. 54) who first introduced a comprehensive surgical method for closing clefts of the velum. Three years later, independently of von Graefe, Roux (PhilibertJoseph, 1780-1854) of Paris popularized staphylorrhaphy or suture of the soft palate defect, following his success in closing a cleft of the velum in a Canadian student. In 1821 Thomas Alcock performed the operation for the first time ,in England. Stevens (1827) of New York and Warren (1828) of Boston were the first to close soft-palate clefts in America. Palatal operations were limited to closure of simple cleft of the velum or soft palate until the year 1826, when Dieffenbach introduced his boneflap operation for uranoplasty or closure of clefts in the bony palate. This
* The historical background of surgical treatment is partly taken from Dorrance, George M.: The Operative Story of Cleft Palate, Philadelphia, W. B. Saunders Co., 1933.
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Fig. 54. Carl Ferdinand von Graefe (1787-1840). (Garrison from Dorrance.) This celebrated German surgeon introduced the first comprehensive surgical method for closing clefts of the velum or soft palate.
procedure was exceedingly simple and in harmony with embryological development in that it completed a union of the bony palatal processes which normally unite to form the hard palate. The later operation by von Langenbeck (1859) utilizing mucoperiosteal flaps, however, was favorably accepted by oral surgeons. Many of the designs in use even today are modifications of the von Langenbeck procedure. Von Langenbeck believed that bone regeneration occurred from the periosteum on the undersurface of his mucoperiosteal flaps and it probably does to some extent. That a considerable amount of anterior contracture or shortening of the palate occurs postoperatively, however, is evidenced by the numerous later modifications of von Langenbeck's technique such as push-back or lengthening maneuvers to compensate for the anterior contracture which is apt to occur. The Bone-Flap Method of Repair (Osteal Uranoplasty)
The operative story of the bone-flap procedure is rather easy to narrate since it can be accurately traced back to Dieffenbach (Fig. 55), to whom other writers, modifying his technique, gave due credit. Although the method was given up in Germany and other European countries, it later found a place in American surgery. In 1826 Dieffenbach13 demonstrated that a cleft involving the hard palate could be repaired by making lateral relaxation incisions through mucosa and bone. The denuded margins of the bone flaps were brought into firm apposition by means of wire sutures inserted through drill holes
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Fig. 55. Johann Friedrich Dieffenbach (1792-1847). (Courtesy of Dr. V. J. Bernbeck.) Palatal operations were limited to simple clefts of the velum until Dieffenbach introduced his bone-Hap procedure for closure of clefts in the hard palate.
in the bone. He described his suggested operation as actually performed in 1845 and advised that, if closure could not be accomplished at once, gradual approximation might be obtained by tightening the wire sutures at intervals. In 1850 Wutzer14 of Bonn combined Dieffenbach's boneflap closure of the hard palate defect (osteal uranoplasty) with closure of the soft palate defect in a single operation. In 1845 Dieffenbach described his management of hard and soft palate clefts in a comprehensive book, Die Operative Chirurgie. 16 He used no anesthesia and stressed the importance of sharp instruments to denude the free borders for relaxation through the hard and soft palate. He used the chisel to sever and infracture the bony palatal flaps, made lateral relaxation incisions in the soft palate, and inserted wire sutures through the drill holes in the bone. He also left an intentional anterior opening in all wide, complete palatal clefts. This technique is quite similar to that employed by Warren Davis. In Dieffenbach's words, "the purpose of the operation is to close the soft palate and as much of the hard palate as possible. The remaining anterior palate fistula may be covered by an obturator and later closed by further surgery. At the time of operation the patient is seated facing a window, his head being held by an assistant, and instructed to open his mouth and breathe in deeply, thereby pressing down on the floor of the mouth. After each incision he is allowed to recover, gasp for air and wash his mouth with cold water but not gargle because this would irritate the velum."* • Translated by V. J. Bernbeck, M.D., senior resident in plastic surgery at St. Barnabas Medical Center.
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Fig. 56. Bernhard Rudolf Konrad von Langenbeck (1810-1887). (Dorrance.) Langenbeck dissected the mucoperiosteum from the underlying bone in repairing cleft palate. The large majority of operations in use today are modifications of von Langenbeck's method.
Dieffenbach stated that the cleft-palate operation was first performed by von Graefe in 1816, and after him by Roux. He did not cite the single case reported by Ie Monnier described by Robert in 1766. The bone-flap method was used successfully by both Buhring 16 in 1850 and L6wenhardt 17 in 1857, but von Langenbeck18 (Fig. 56) in 1861 pointed out that the procedure was limited to cases of bilaterallip-jawpalate clefts. He thought that it was contraindicated in cases of unilateral lip-jaw-palate splits in which the vomer was attached to one side of the defect. From von Langenbeck's report, one is led to believe that the procedure was not successful in his hands. Billroth19 stated that the bone-flap procedure was no longer practiced, but Sir William Fergusson,2o Francis Mason,21 Lannelongue,22 Goodwillie23 and W oakes24 reported its successful use from 1873 to 1879. In the United States, in 1893, Mears26 of Philadelphia employed the bone-flap method for cleft palate repair and demonstrated his technique to G. V. 1. Brown (Fig. 57) and to Roe. Brown apparently was not impressed by the operation and employed the von Langenbeck method when he returned to practice in Milwaukee. Later, however, he became critical of his results with the von Langenbeck technique and began to use the bone-flap procedure almost exclusively in a one-stage type of repair. Roe utilized Mears' procedure in a large series of cleft palate patients and taught his modified technique to Warren Davis. Following Roe's death possibly the only two men in the world using the bone-flap tech-
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Fig. 57. George Van Ingen Brown (1862-1948). (Courtesy of Dr. V. P. Hyslop.) After Roe's death Brown and Warren Davis were probably the only men in the world using the bone-flap technique.
nique were Dr. G. V. 1. Brown of Milwaukee and Dr. Warren Davis of Philadelphia. These two men improved the operative method as devised by Dieffenbach and instructed other American surgeons .in its use. The author learned the bone-flap technique from Warren Davis (Fig. 58) and is currently demonstrating it to his senior resident, Dr. V. J. Bernbeck, who will reintroduce the procedure in Germany, where it originated. The bone-flap operation as used by our group has been modified to some extent but the technique is essentially the same as that of Warren Davis. The author first observed Warren Davis' technique some 20 years ago, when with Staige Davis, Kitlowski and Straatsma he was invited to visit Jefferson Hospital. Warren Davis* performed seven one- and twostage operations during the morning and in the afternoon demonstrated his postoperative results with some randomly selected patients in his office. We were all impressed by the speech of these patients and the
* Warren Davis was an exceptionally skillful cleft-palate operator and had developed a smooth operating team. His anesthetist, wearing a sterile glove on one hand, held a retractor and John Reese, of Reese Dermatome fame, served as first assistant. He controlled bleeding by packing the lateral relaxation incisions through the palatal shelves and posterior palatal artery with sponges saturated with Adrenalin solution (1 :1000). This quickly controlled bleeding, and so enabled him to complete his firststage procedure in 3 minutes and the second stage closure in 20 minutes. Davis, a gentleman of the old school, acknowledged indebtedness to his teacher Dr. Roe, whom he described as a skillful technician who had never written a paper describing the bone-flap repair.
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Fig. 58. Warren B. Davis (1881-1947). The present author learned the bone-flap technique from Warren Davis. Davis followed John Wheeler as President of the American Society of Plastic and Reconstructive Surgery and was the first Editor of the Society Journal.
normal appearance and function of the palates. The author has used Warren Davis' bone-flap technique with some modifications since that time. OPERATIVE TECHNIQUE
Complete Bilateral Palatal and Alveolar Clefts
The palatal defect is wide and the palatal shelves are often rather narrow. Consequently a two-stage procedure is always indicated to insure adequate blood supply for the flaps. Ali intentional fistula will remain anteriorly after the second operation, in which the palatal shelves are joined at the midline. In the first operation incisions are made medial and parallel to the alveolar ridges, leaving a rim of oral mucous membrane between the incision and the erupted or unerupted teeth. A Warren Davis chisel is then inserted into this incision and the bony palatal processes with their covering nasal mucous membrane are severed from their lateral attachments and infractured toward the midline. The hamular process and posterior palatal artery are separated during the chiseling maneuver but bleeding is quickly controlled by inserting gauze sponges soaked in dilute Adrenalin solution. When all bleeding has ceased, the relaxation openings at the sides are packed with Oxycel, and the patient sent to the recovery room with a temporary
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suture inserted in the tongue as an aid to nurses in opening the mouth and using suction for secretion or blood in the pharynx. The second operation is performed two weeks later. The free margins of the palatal flaps are denuded of mucous membrane and small drill holes are made in the bony palatal shelves. The fibrous adhesions which have formed in the lateral relaxation areas are separated and 000 silk sutures inserted through the drill holes. The soft palate processes are then freed from their lateral attachment by extending the relaxation incisions posteriorly until the soft palatal processes can be approximated without undue tension. Number 000 chromic catgut sutures are then inserted through nasal mucous membrane and muscle from near the posterior border of the bony palate to the tip of the uvula. These sutures are tied so that the knots are on the surface of the nasal mucous membrane. The three or four silk sutures which were previously inserted through drill holes in the bony palate are tied, which approximates about threefourths of the bony palate, leaving an intentional anterior fistula. This will be closed one year later. Number 000 chromic catgut sutures are then inserted into the oral surface of the soft palate from the tip of the uvula to the posterior border of the bony palate. These catgut sutures include mucous membrane and a generous portion of muscle. The relaxation openings at the sides are packed with Oxycel and a temporary suture is again inserted into the tongue as an aid for nurses in aspirating mucus and blood from the child's pharynx. The catgut sutures always loosen and are usually swallowed by the child about two weeks following operation. The silk sutures in the bone are removed three weeks after the operation. Complete Unilateral Palatal and Alveolar Clefts (Figs. 59 and 60)
In these cases also we perform the two-stage procedure two weeks apart. The technique is the same as that used for complete bilateral clefts except for the management of the vomer, which is attached to the bony palatal process on the non-cleft side. This vomer attachment is not disturbed at the time of the first operation, in which relaxation incisions are made through both bony palatal shelves. At the time of the second operation, however, the vomer is severed from the palatal process with a chisel, which permits infracture of the freed bony palatal process on the non-cleft side. The technique of closure is then exactly the same as that used for the second-stage repair of complete bilateral palatal and alveolar cleft palate. Severing the vomer from the bony palatal process, in effect, converts a unilateral cleft palate into a bilateral cleft palate. Warren Davis always used a mucoperiosteal flap on the non-cleft side in these patients. He joined the mucoperiosteal flap to a bone flap which
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Fig. 59. First operation in a two-stage bone-flap repair of a complete 'unilateral cleft palate. ' A, First inject mucous membrane with normal saline to which 8 drops of Adrenalin per ounce has been added. Incisions are then made parallel to the alveolar ridge through both oral mucous membrane and periosteum. Note that base of septum is attached to palatal process on the left side which classifies this case as a unilateral cleft palate. B, A Warren Davis chisel is inserted through the incision on the right side at an angle inward to avoid injuring tooth buds in the alveolar ridge. The chisel is tapped with a hammer to completely separate the bone and nasal mucous membrane from their lateral attachments. The hamular process and palatine artery are severed and the incision extended posteriorly in the soft palate. A sponge saturated in normal saline with 8 drops of Adrenalin to the ounce is packed in the relaxation incision. This should be held firmly for a few minutes to control bleeding before separating the opposite bony palatal process. C, Oxycel cotton is inserted in the lateral relaxation incision and held firmly until all bleeding has ceased. Child is sent to recovery room with a suture through the tongue as an aid to nurses for opening the mouth and sucking out blood or mucus. Endotracheal ether-oxygen anesthesia is used in all cases.
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Fig. 60. Second operation in a two-stage bone flap repair of a complete unilateral cleft palate. An interval of 14 days elapses between the first and second operation for development of an adequate blood supply in the flaps. A, The base of the septum is separated from the bony palatal process on the left side. This converts the unilateral cleft to a bilateral cleft. The relaxation incisions at the sides are reopened and the bony palatal shelves infractured so that the free margins can be approximated in the midline without undue tension. a, b, c, The free borders of the palatal shelves are denuded of their mucous membrane covering. 1, 2, 3, 4, Three drill holes have been made through the bony palatal processes on each side. Drawings illustrate technique used to insert 00 heavy silk sutures through these drill holes. B, First, the nasal and then the oral mucous membrane is drawn together with 000 chromic catgut. These sutures include the muscle layer. If the soft palate is short or the soft palatal processes unequal in length, the Z-plasty technique is used, as illustrated in Figure 61. This may be combined with the introduction of a dermal graft beneath the pharyngeal mucous membrane in the area of Passavant's cushion. The 3 heavy silk sutures through drill holes in the bone are then tied to bring the denuded surfaces of the bony palatal shelves together in the midline. In wide complete palatal and alveolar clefts it is often expedient not to attempt closure of the anterior onefifth of the bony palatal cleft. This leaves an intentional anterior fistula which may be closed about. 1 year later with rotation mucous membrane flaps. Oxycel gauze is introduced into the lateral relaxation incisions to control bleeding and the child sent to the recovery room with a suture in the tongue.
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he formed on the cleft side. I suggested the procedure of severing the vomer-palate connection to him a number of times but, although he approved of the principle, I do not recall that he ever used it. * IncOInplete and Complete Posterior Alveolar Cleft Palate (Fig. 61)
The repair of these clefts is essentially the same as that used for complete palatal and alveolar clefts except that they are usually closed in a single operation. The number of sutures inserted into the bony palatal shelves varies with the length of the cleft, and one can usually obtain complete closure of the bony palate without leaving an intentional anterior palatal fistula. The soft palate is never separated from the posterior ~argin of the bony palate. We prefer to infracture the bony palatal shelves and insert a single suture through the bone for approximation of even a small opening or notch in the bony palate. Z-Plasty for Short Palate
When the soft palate segments are short or unequal in length, a Z-plasty is routinely performed to obtain greater length in both segments or establish greater length on the short side. The incisions to form the Z-designs include the muscle and both oral and nasal mucous membrane. Hence, after the flaps have been sutured in position no raw surfaces exist which might give rise to anterior contraction and shortening of the palate. Careful examination of these patients postoperatively does not show any impairment in muscular movement. We believe that these children have less tendency to nasality than do a comparable group with short soft palates in which the short palatal segments are merely approximated. Variations in Technique
In extensive clefts of the palate we occasionally use a vomer flap combined with the bone-flap technique at the time of the second operation. Usually, however, we leave an intentional opening in front and close this later with overlapping detached flaps elevated from the underlying bone. In individual cases of incomplete cleft palate the decision whether to do a one- or two-stage procedure may present some difficulties. In complete palatal-alveolar clefts, either unilateral or bilateral, it is always safer to do a two-stage procedure. In older patients who have had no palatal repairs we also tend to use a two- or even a three-stage procedure because of the width of the palatal defect and the importance of an adequate blood supply for the flaps. In these older, unrepaired cleft-palate defects it is often possible to close only the soft palate and a small portion
* Dr. Hyslop has informed me that Dr. G. V. I. Brown always detached the vomer from the palatal process on the non-cleft side to facilitate infracture. Dr. Hyslop was at one time assistant to Dr. Brown and has continued to use his bone-flap technique.
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Fig. 61. Postalveolar short cleft palate with defect extending partly or completely through bony palate. A, The free margins of the palatal processes have been denuded of mucous membrane and relaxation incisions through the bony and soft palatal shelves have been made on each side. Right-angle incisions through the complete thickness of the soft palate form the pointed flaps 1 and 2. Variations in the angles of these flaps are used when one soft palatal process is shorter than the other to establish equal length. B, The bony palatal shelves are infractured and 2 silk sutures have been introduced through drill holes in the bone (see Fig. 60). C, D, Chromic catgut sutures (000) are inserted through the nasal mucous membrane and muscle. When these are tied the knots will be on the nasal surface. E, Chromic catgut sutures (000) through oral mucous membrane and muscle are tied to approximate the oral surface of the soft palate. The 2 heavy silk sutures through drill holes in the bone are tied to close the palatal cleft completely. When shortening of the velum is marked, the Z-plasty is combined with the introduction of a dermal graft beneath the pharyngeal mucous membrane in the region of Passavant's cushion.
of the bony palate. The large defect remaining anteriorly is covered with a dental prosthesis. Since all of these unrepaired cleft palates are short, we do the pharyngeal flap or a Z-plasty procedure at a later date and follow with speech therapy. The advisability of operating on older, unrepaired cleft palates is an individual problem. When the defect is so
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wide that closure of the soft palate seems problematical, then it is best to advise the patient to consult a prosthodontist and have an artificial appliance made. Orthodontia
Consultation with an orthodontist should go hand-in-hand with surgical repair of the palate, especially in the cases of palatal clefts of a complete type extending through the alveolus and disrupting the integrity of the alveolar arch. Bilateral palatal clefts involving the alveolar arch which are associated with a complete double cleft lip present unusually difficult problems, and the assistance of an orthodontist is essential to place and maintain the middle segment or prolabium properly in alignment as the anterior central segment of the alveolar arch. RESULTS WITH THE BONE-FLAP TECHNIQUE
It is extremely difficult to compare the results of various types of operation for closure of cleft-palate defects. This is so because individua1 plastic surgeons mainly use a single technique which they have learned from their preceptor during their training period. Consequently, they have no basis for comparison excepting occasional cases in which repair has been carried out elsewhere by other methods, and these are generally not seen in large numbers. In evaluating the bone-flap technique, which has been employed by the author for 20 years, it may be said to be a rather simple procedure, which gives uniformly good results in respect to closure of the palate and good functional movement of the soft palate. Because a segment of bone with oral and nasal mucous-membrane covering is brought over on each side to the midline and the soft palate attachment to the posterior edge of the bony palate is left intact, there is little, if any, tendency to anterior contracture or shortening of the palate following operation. The procedure, however, does not provide for additional lengthening, and if one starts with a short palate, one ends up with a palate of the same length. If the shortening is marked, we lengthen the soft palate with a Z-plasty, as described under operative technique. The incisions for this Z-plasty include muscle and both mucous-membrane surfaces. When the children are older, secondary operations to close the intentional anterior fistula, which could not be closed at the time of the primary operation, demonstrate that the bone-flap shift results in a firm bony union of the palatal shelves in the midline. This facilitates closure of the anterior fistula, since mucous-membrane flaps can be elevated from the bone and sutured to cover the palatal opening. The area at the side where the bony palate was separated and infractured for relaxation purposes also fills in with bone. Thus, in all areas of the hard palate
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where bone normally should be, bone is ultimately present except in the area of the anterior fistula. This may be of some advantage as a soundingboard effect in speech; certainly it is a distinct advantage in closing the intentional anterior palatal fistula, since any flaps raised will expose a surface of bone rather than create an opening into the nasal cavity. Speech Evaluation
Comparison of the quality of speech following the bone-flap technique with that following other types of palatal operations presents numerous problems. The only type of repair made by other techniques elsewhere which we see in any considerable number at the St. Barnabas Rehabilitation Center is the von Langenbeck procedure. All of these patients seem to have considerably more shortening and scarring in the palate, and the over-all quality of speech is not as good as that obtained following the bone-flap procedure. This is in conformity with the author's early experience regarding the quality of speech in his own patients repaired by the von Langenbeck method. Comparison of the bone-flap method with the Wardell type of operation or other operations involving closure and lengthening in the same procedure cannot be made with any degree of accuracy. Critical evaluation by our speech therapists of all cleft palate patients repaired on our service, either by staff members or senior residents, demonstrates that 30 per cent require no speech therapy; 70 per cent do. A large number of the patients who are judged to require speech therapy have only minor speech defects, which are readily corrected by therapy. On the small number of patients who do not respond adequately to the therapy we do a pharyngeal flap procedure, as described by Moran, or an extension Z-plasty if the palate is short. In most instances this improves the quality of speech; speech therapy is always instituted following the lengthening operation. OTHER CLINICAL CONSIDERATIONS
Age of Child at Operation
A few years ago considerable controversy prevailed, especially in the United States, regarding the proper age for operation on palatal clefts. One group with a considerable following claimed that cephalometric examinations of the maxillae of patients operated on before the fifth year of age demonstrated considerable underdevelopment of the upper jaw and they advised against early operation. Cephalometric examinations were made by the orthodontic department at St. Barnabas Rehabilitation Center on 94 patients operated upon by the bone-flap technique before the age of two years. Similar studies were made on 44 patients operated upon elsewhere by the von
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Langenbeck method before the age of two years.26 In neither group did we find a significant underdevelopment of the maxillae. Accordingly, we operate on all cleft-palate patients who are in good physical condition between the ages of seven to 14 months, depending on the width of the cleft and the development of the palatal shelves. It is of considerable advantage to close the cleft before the child begins to talk. Malocclusion in Cleft Palate Deform.ity
Studies by our orthodontist, Dr. Michael Collito, indicate that crowding and crossbite are typical malocclusions associated with cleft palate. whether surgically treated or not. Only one patient in six is free of malocclusion, which suggests the need for orthodontia regardless of the type of surgical repair. Neither crowding nor crossbite is related to surgical trauma. Both are related, however, to the type of original defect, since the relative incidence of both deformities increases sharply with the increasing severity of the original defect. 26 Change in surgical technique cannot be expected to affect the incidence of malocclusion. Nor does age at operation affect either its incidence or its severity. Orthodontic management in general should begin after eruption of the secondary teeth except in patients with complete bilateral alveolar clefts. These require very early orthodontic treatment to maintain the middle segment in proper relationship with the alveolar arch. Hearing of Cleft Palate Patients
Sixty per cent of the children with all types of cleft palate deformity appearing at our clinic have a significant hearing loss. The deafness is usually of the conductive type and is associated with a history of repeated colds and the presence of enlarged and often infected tonsils and adenoids. Young children who do not hear well will not learn to speak well, and the child with a cleft palate and a significant hearing loss presents a serious problem. Our management has consisted of first attempting to immunize children against infection of the upper respiratory tract with vaccines and general preventive measures. When this fails, we remove the tonsils and the adenoid tissue around the eustachian tubes, leaving the centra adenoid mass in the region of "Passavant's cushion." Recently we have routinely sutured the tonsillar pillars together to avoid excess scar formation in the denuded tonsillar fossae. Patients who have established good speech before operation are not usually affected; children who have poor speech before operation may be adversely affected by. removal of too much adenoid tissue especially in the region of Passavant's cushion.
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A high percentage of our cleft palate children in the New Jersey area have a low-grade or chronic sinusitis. This is treated by local medication to promote drainage and general measures to increase the child's resistance. The outlook for most of the 60 per cent of cleft palate children with significant hearing losses is not a happy one, since the deafness usually is progressive despite therapy. Psychological Screening of Cleft Palate Children
Our patients with cleft palate defects, as a group, had a mean intelligence quotient (by Stanford-Binet tests) slightly higher than that of a comparable group of children without deformity. Social maturity was low, which we attributed to the effect of imperfect speech and the concomitant cleft-lip deformity (when this was present) on the adjustment of these children with their playmates. In many instances patients become most aware of the fact that they are different from other children after entering school, usually in the first or second grade. This indicates the importance of doing everything possible to have them at their best regarding speech and appearance before the school period begins. CONCLUSIONS
1. The results of a number of studies on animals indicate that maternal disease and other critical stresses occurring at certain stages during pregnancy may be responsible for various types of congenital abnormalities including cleft palate. 2. Cortisone given to pregnant mice induces a high incidence of cleft palate deformity in the offspring. Specific vitamins were administered to pregnant mice concomitantly with cortisone acetate to render ineffective its teratologic effect. Of the vitamins tested only B6 and folic acid reduced the incidence of cleft palate in the offspring. 3. Based on the protective effects of vitamins B6 and folic acid in cortisone-treated mice, we are routinely giving these two vitamins during the first four months of a subsequent pregnancy to patients who have had a cleft lip-palate child. Thus far, all of the offspring of these mothers have been normal but the series of 50 normal infants is not large enough to be significant. 4. The bone-flap method of repair originated by Dieffenbach in 1826 was possibly the first technique to close a cleft in the hard or bony palate successfully. 5. In our experience the bone-flap technique of the late Dr. Warren Davis is a very satisfactory method of repair for cleft palate. 6. When the soft palatal processes are short or unequal in length a Z-plasty is useful to obtain greater length in both .segments or establish greater length on the short side.
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7. Postoperative evaluation studies on a varied age group of our cleft palate patients is summarized as follows: (a) Speech. Thirty per cent had normal speech and 70 per cent had speech defects. A large majority of the patients with defective speech responded favorably to therapy before the school age (five to six years). (b) Hearing. Sixty per cent had a significant hearing loss. (c) Psychological Evaluation. The LQ. (by Stanford-Binet tests) of children with cleft palate at our clinic was slightly higher than that of a comparable group of children without congenital deformity. (d) Facial Bone Development. Operative repair by the bone-flap technique before the age of 20 months was not associated with significant underdevelopment of the maxillae. (e) Orthodontia. Malocclusion is very frequently associated with cleft palate deformity whether surgically treated or not. Malocclusion is related to the type of original defect, since its relative incidence increases sharply with the increasing severity of the cleft palate deformity. REFERENCES 1. Ingalls, T. H.: Causes and Prevention of Developmental Mental Defects,
J.A.M.A. 161: 1047 (July 14) 1956. 2. Warkany, J.: Congenital Malformations Induced by Matgrnal Nutritional Deficiency. J. Pediat. 25: 476 (Dec.) 1944. 3. Baxter, H. and Fraser, R. G.: Production of Congenital Defects in Offspring of Female Mice Treated with Cortisone. McGill M.J. 19: 245, 1950. 4. Warkany, Josef: Personal communication regarding his unpublished experimental work. 5. Hurxthal, Lewis M.: Practical Endocrinology. New York, McGraw-Hill Book Co., 1955, p. 148. 6. Strean, L. P., and Peer, L. A.: Stress as an Etiologic Factor in the Development of Cleft Palate. Plast. & Reconstruct. Surg. 18: 1 (July) 1956. 7. Medawar, P. B.: Zoological Laws. In Vol. II, Transplantation of Tissues, Lyndon A. Peer, Ed., Baltimore, Williams & Wilkins Co. In press. 8. Peer, L. A. and others: The Induction of Cleft Palate in Mice by Cortisone and Its Reduction by Vitamins. J. Internat. Coll. Surgeons 30: 249 (Aug.) 1958. 9. Peer, L. A. and others: Study of 400 Pregnancies with Birth of Cleft Lip-Palate Infants. Plast. & Reconstruct. Surg. 22: 442 (Nov.) 1958. 10. Douglas, Beverly: Monograph: Harelip and Cleft Palate. From Division of Plastic Surgery, School of Medicine, Vanderbilt University, Nashville, Tennessee. Privately printed. 11. Conway, H.: Effect of Supplemental Vitamin Therapy on the Limitation of Incidence of Cleft Lip and Cleft Palate in Humans. Plast. & Reconstruct. Surg., 22: 450 (Nov.) 1958. 12. Robert: Traite de principaux objets de medecine avec un summaire de la plupart des tMses soutenues aux ecoles de Paris, depuis 1752 jusqu'en 1764: on y a joint des observations de pratique, 1: 8, premier observation, Paris, 1766, Lecombe. See also errata appended to vol. ii. 13. Dieffenbach, J. F.: "Ober'das Gaumensegel des Menschen und der Saeugethiere. Litt. Ann. d. ges. Heilk. 4: 298, 1826, abstr. Lancet 10: 811,1826. Idem: Beitrage zur Gaumennath. Litt. Ann. d. ges. Heilk. 4: 145, 1826. Idem: Beitrii.ge zur Gaumennath. Litt. Ann. d. ges. Heilk. 6: 305,1826. Cited by Dorrance. 14. Wutzer, C. W.: Heilung der angeborenen SpaUe des weichen und harten Gaumens durch Operation. Deutsche Klin. 2: 260, 1850.
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15. Dieffenbach, J. F.: Die Operative Chirurgie. Vol. 1. Leipzig, F. A. Brockhaus, 1845. Translated by V. J. Bernbeck, M.D., senior resident at St. Barnabas Medical Center. 16. BUhring, J.: Die organische Schliessung des durchbrochenen harten Gaumens vermittelst Knochensubstanz. J. d. Chir. u. Augenh. n.s. 9: 329, 1850. Idem: Beitrage zum organischen Wiederersatz der Defecte des harten Gaumens vermittelst Knochensubstanz. Deutsche Klin. 2: 472,1850. 17. Lowenhardt: Staphyloraphie und versuchte Staphyloplastik. Deutsche Klin. 9: 137, 1857. 18. von Langenbeck, Bernhard: Professor Langenbeck on UranopIastica. Med. Times & Gaz. 2: 69, 1861. Idem: Operation der angeborenen totalen Spaltung des harten Gaumens nach einer neuer Methode. Deutsche Klin. 8: 231, 1861. 19. Billroth, Theodore: tl"ber osteoplastische Operationen. Wien. med. Presse 9: 1057, 1058, 1073-1076, 1868. . 20. Fergusson, Sir William: Observations on Harelip and Cleft Palate. Retrospect of Practical Med. & Surg. 23: 158, 1852. Idem: Cleft Palate. M. Times & Gaz. n.s. 4: 433, 1852. Idem: A New Operation for Cleft Palate. Lancet 2: 784,1873; Month. Rev. Dent. Surg. 311, 1873-1874. Idem: Observations on Harelip and Cleft Palate. Brit. M.J. 1: 403, 437, 1874; Month. Rev. Dent. Surg. 2: 481, 1873-1874. 21. Mason, Francis: Clinical Remarks on Cleft Palate. Lancet 2: 578,1874. Idem: The Treatment of Cleft Palate. Lancet 2: 170, 1876. Idem: On Harelip and Cleft Palate. London, 1877. Idem: Cleft Palate. In: On Surgery of the Face. London, 1878, p. 104. 22. Lannelongue: Restauration de la vo'O.te palatine. Bull. et m~m. Soc. de chir. n.s. 2: 469, 1876. 23. Goodwillie, D. H.: A Case of Congenital Cleft of the Hard and Soft Palate, with Extensive Hypertrophy of the Left Inferior Turbinate Bone. Tr. Med. Soc. New York, 93,1877. 24. Woakes, Edwards: Case of Operation for Cleft Palate Illustrating aNew Method of Performing Osteoplasty. M. Times & Gaz. 2: 526,1879. 25. Mears, J. E.: Clefts of the Hard and Soft Palates. Read before the Philadelphia Academy of Surgery, 1893. South. M. Rec. 24: 59, 1894. 26. Peer, L. A., Hagerty, R., Hoffmeister, F. S. and Collito, M. D.: Repair of Cleft Palate by the Bone Flap Method. J. Internat. ColI. Surgeons 22: 463 (Oct.) 1954. 120 Halstead Street East Orange, New Jersey