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Harelip and Cleft Palate Defects: Surgical Technics
Harelip and Cleft Palate Defects Surgical Technics WAYNE B. SLAUGHTER, M.D.*
ONE of the common defects afflicting the human race is that of cleft lip, cleft palate or a combination of the two. The care of a person having such a defect is complex in the extreme. The complexity and inadequacy of satisfactory rehabilitation has been somewhat rectified in recent years as a result of the combined efforts of many classifications of specialists in basic research and clinical endeavor. These research findings combined with loosely related bits of information have resulted in improvement of the service aspect of rehabilitation of person with harelip or cleft palate. The real basis for a more optimistic outlook for those born with a cleft of the lip or palate has its foundation in fundamental research plus the more adequate use of associated clinical specialists whose services are extremely beneficial in the total rehabilitation of these patients. The picture of the normal growth pattern of the face as presented by Brodie,3 Broadbent,2 Krogman7 and others has pointed up hitherto unrecognized clues as to what can be accomplished from the standpoint of surgery. These basic works also indicate that there are specific limitations to be placed upon the surgeon's attempts to correct such defects. The timing of surgery in relation to the age of the patient and the extent of the surgical procedure itself are two closely interrelated factors in the successful outcome of such procedures as expressed in terms of normal development. It has been established that the growth pattern of an individual born with a cleft lip or palate endeavors to follow a pattern of growth the basis of which is completely laid down within a few months after birth. This growth pattern is very similar to that of a normal person irerespctive of the type or extent of the cleft. This growth pattern, or more specifically the growth potential of the tissues involved, can be dis-
* Head of Department of Plastic Surgery, University of Wisconsin School of Medicine, Madison, Wisconsin; Chairman, Department of Plastic and Reconstructive Surgery, Stritch School of Medicine, Loyola University and Chairman, Department of Maxillofacial Surgery, Chicago College of Dental Surgery, Loyola University, r:hicago. 165
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turbed enough to disrupt this predetermined pattern by only a few agents. These agents are practically limited to surgery, radiation, severe local trauma, and certain types of infectious processes. The action of these agents must be of a degree sufficient to destroy, damage or some way alter the individual cells having to do with growth and development of a given area. The surgical approach for ultimate closure of a cleft of the lip or palate must therefore be performed so as not to disturb this biologically determined normal growth pattern. In order to carry out a closure of a cleft of the lip or palate and still stay within bounds of the normal physiologic ability of the tissues to overcome the accompanying surgical trauma, and, at the same time establish normal anatomic relationships of the various structures lllvolved, the following principles are being tentatively advanced. UNILATERAL HARELIP
The closure of a simple or unilateral cleft of the lip is usually carried out rather early in life. The time cannot be rigidly defined because this is an elective operation carried out when the patient can best withstand such trauma. The only limitation that can attend such an operation is closure early enough to take advantage of the pliability of the underlying bony structures. For some this will mean surgical closure at two weeks of age while for some it may mean a delay for one to several months after birth. The general condition of the patient, the rate of weight gain and other indicative signs of satisfactory well-being are the determining factors for carrying out the procedure. At the present time all patients being operated upon for closure of a cleft lip receive a general inhalation anesthesia administered by means of an endotracheal tube. The tube is passed through the oral cavity to prevent distortion to the nose or upper lip. With the properly conducted endotracheal anesthesia, the mortality and morbidity rates have been greatly reduced. The rigid control of the airway at all times by the anesthetist, with the accompanying accurate knowledge of the plane of anesthesia, allow for a much more rapid completion of the operation itself. The ability to perform a more satisfactory postoperative endotracheal toilet and the rapid return to consciousness are also factors contributing to more acceptable results. Closure of the lip proper follows in general the procedures outlined some seventy years ago by Hagedorn 5 • 6 (Fig. 71, a, b, c). To this early contribution must be added the refinements and modifications introduced by Steffensen,12 Le Mesurier,8 Blair and Brown! and others. Specifically the operation calls for freshening the sides of the cleft by clean through-and-through incisions. On the medial side the anatomical boundaries of the normal philtrum are followed to facilitate adherence to normal tissue relationships as much as possible (Fig. 71, c, d, e). The medial portion of the lateral or short segment is freshened in such a
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manner as to loosely dovetail into the incision previously made in the philtrum. As will be noted in comparing the methods, the notch is made considerably lower than originally suggested by Hagedorn. This is done merely to more closely follow the dictates of accepted anatomic relationships. No incision or undermining is carried beyond the midline. This is in accordance with long established plastic surgery principles that to do so may be injurious to the flaps themselves in addition to the fact that this procedure may be a factor in changing the direction of the
Fig. 71. a, b, c, Closure of the lip. Redrawn from Hagedorn, Centralbl. fUr Chir. 19: 281,1892. d, e,!, Modification of Hagedorn's method in which the notching is nearer the mucocutaneous junction in order to more closely follow the normal configuration of the philtrum.
normal muscle forces resulting in irreversible abnormal structural relationships. Laterally the flaps are undermined in as limited a manner as possible over the anterior maxillary wall. Again this is in compliance with accepted dictates not to overextend the contained blood supply of a given flap. The attachment of the lower lateral or inferior alar cartilage is freed from the proximity of the maxilla to allow it to be rotated medially to aid in the formation of the floor of the nostril and re-establish a more normal nasolabial fold. These mobilized structures are moved into position and sutured in the desired relationship. This is done without any manual or forceful "molding," fracturing or contouring of t.he underlying delicate facial bones. The lip is closed over the defect
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in the bone so that it may act as a physiologic force to accomplish the desired change in contour. The gentle action of the closed lip exerted in the proper direction over a long period of time brings the structures into a more normal position by the interplay of muscular forces between the lip and tongue and their accessory supportive structures. The guiding or directing of the growth potential accomplishes the desired end result slowly and positively but does not interfere with growth factors of the involved structures by destroying existing or potential growth centers or by introducing damaging amounts of scar tissue. The floor of the nose is first closed using 4-0 silk. Care is taken to insure prop~r relationships of tissues at the nostril opening because of its importance both from a cosmetic and functional standpoint. The mucous membrane and muscle of the lip are brought into apposition by figure-of-8 sutures. The suture is started on the mucous membrane surface so that the knot may be placed on this surface thereby minimizing the scarring of the skin surface of the lip. This suture acts as a tension suture for the entire lip and the remaining sutures placed in the skin of the lip are purely for appositional purposes. As a rule a subcuticular suture is placed in the skin of the lip using fine horsehair. This practically eliminates any stitch marks on the skin of the lip itself. Again it must be emphasized that all unnecessary trauma in the form of crushing, clamping or molding of both hard and soft tissues is potentially harmful. The introduction of scar tissue into rapidly growing structures will eventually have a restraining action upon the development of the entire upper jaw. All too often the undue scarring of the lip acts as a powerful antagonist to the normal downward and forward growth of the upper jaw. This may be a major factor in producing a tight upper lip, one not possessing normal mobility because of the binding action of the contained scar tissue. It is well known that the surgical repair of any soft tissue structure has for its successful basis the introduction of some scar tissue as part of the normal healing process. A preponderance of scar tissue, however, will result in some limitation· of motion or gross physical deformity. In the lip this is expressed most often by a tight scarred lip that is usually anatomically longer than nature intended. A resulting discrepancy in growth between the upper and lower jaw results in the relatively prognathic or protruding jaw as an end result. Almost invariably the lower jaw is merely expressing its normal growth potential and the upper jaw fails to keep up with this growth picture because of the limitations imposed by the scarred lip plus possible interference with growth centers in the underlying bone itself.H It appears that the closure of the single cleft of the. lip must fulfill the following requirements: First, the congenitally separated tissues must be brought into relatively normal anatomic relationship. This can only be done surgically. As a rule the necessary ti5sues for such a
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reconstruction are present since the defect is not one of deficiency of tissue but merely one of displacement of tissue. Second, the closure of such a defect must be carried out in such a manner as not to interfere with the normal expression of growth potential of the tissues involved. Third, the position of the tissues in their new relationship must be such that even after rotation or medial deviation of the premaxilla (os incisivum) over a period of months or years the resulting lip is passable both cosmetically and functionally. Too often the stigma of a cleft lip facies is not the result of the shortcomings of nature but the result of overly zealous activities on the part of those sincerely trying to rehabilitate one so unfortunate as to be born with such a defect. BILATERAL HARELIP
The closure of a bilateral cleft of the lip presents all of the problems of the single cleft and frequently a disproportionate growth between the vomer, premaxilla and maxilla. The problem is not only that of closing two single clefts of the lip in one individual; there is the added problem of utilizing a philtrum that is not only displaced but also deficient in blood supply for the surgery that should be done. The closing of the lip over the existing bony openings has for its prime purpose the establishment of a physiological directing face on the subsequent development of the maxillary area. This should be carried out without resort to molding, fracturing or cutting of the rapidly developing structures involved any of which procedures are apt to interfere with the full expression of the growth potential. Since the blood supply of the philtrum is distributed in such a way that it is only logical to sever one side at a time, this is a major factor in the choice of operations to be carried out. Since the position of the premaxilla is frequently such that the soft tissues cannot be brought over it without causing undue tension on the lip structures or upon the underlying bony structures or both, it seems logical to close the lip in two stages. The double cleft is converted into a single cleft, then the single cleft is closed at a second operation. The initial operation is carried out early in life. If one side presents a wider defect it is closed first. The prerequisites for operation are the same as for the single cleft lip. The cleft is surgically closed using the simple principles as originally outlined by Hagedorn. The flaps made in the philtrum proper are altered slightly in order to conserve as much of the tissue as possible. This conservation of tissue is not carried to the point that tissue of the philtrum itself enters into the final lip below the mucocutaneous junction. To bring philtrum skin below this point results in a lip inadequate in functional capabilities. The first operation is carried out as outlined for the single cleft of the lip. This results in a change in position of the premaxilla due to the tension resulting from the closure. At first the closed side shows rapid
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changes in the relationship of the alveolar borders of the cleft in that they move toward one another. This may continue until there is contact between the two segments. While this is happening the cleft on the unrepaired side at first becomes more prOIlOunced. After a few weeks, however, there are compensatory changes well back in the septum that eventually manifest themselves in the premaxilla. The entire premaxilla moves into a more no.rmal position in relation to the frontal facial plane. This is thought to be due to the lack of displacement forces previously exerted by the tongue directly and the lateral pull of the lip muscles indirectly. Another change also takes place while the above mentioned rotation and displacement process is occurring. This is expressed as an apparent forward movement of the maxilla. Actually it is the addition of bone to the maxilla making the true anterior-posterior dimension of that bone greater. Therefore, what appears to be a greatly displaced premaxilla on first examination may be only slightly displaced after growth allowances are made Pruzansky and Ricketts are of the opinion that this additive growth on the maxilla, if liot interfered with, may in many instances be sufficient to produce a fairly normal upper arch irrespective of the width of the cleft. 9. 10 The operation for closure of the second side of the double cleft of the lip may be any time from six weeks to six months or longer after the first operation. The position of the segments and the general condition of the patient determine the actual time for closure. When the second side of the lip cleft can be closed without undue tension then the procedure is carried out. One of the unfortunate aspects of surgery on bilateral clefts of the lip is that the immediate cosmetic results are usually far from satisfactory. The lip must be closed with the degree of migration of the underlying bony segments kept in mind. Usually the nostrils appear flared and the tip of the nose is flat and the lip appears to be short. But with the knowledge that this will eventually assume a more normal position the program for rehabilitation can be carried on without undue apprehension. In closing a double cleft of the lip none of the principles of plastic surgery are violated. There is no molding, shaping, contouring, cutting or fracturing of bony or cartilaginous structures on the part of the operator. This is all left to the normal muscle interplay that will take place after the soft tissues are brought into proper apposition. 4 There is no substitution of tissues or structures for the sake of immediate cosmetic results. The normal relationship of the philtrum to the columella is maintained together with the relationship of these structures to the nose itself. It is admitted that the nose appears flat after the operation but substitution of the philtrum for a portion of the columella in order to elevate the nose is not a satisfactory answer in the final analysis.
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Adequate growth of the alar cartilages is just as important as growth of other structures if a normal face is to be expected. After this growth has taken place the necessary cosmetic procedures can be carried out without unforeseen disfiguring results. The tremendous changes obtained by competent orthodontists in directing the growth in the double cleft patient cannot be overemphasized. This point will be enlarged upon later. CLEFT PALATE
The problem of closing a congenital cleft of the palate is so well known and wrought with such controversies that an attempt to delve into only a few of the difficulties would only complicate the picture. On the basis of measurements of cephalometric x-rays, plus those taken of plaster casts over a period of years, an impression of some of the steps to be avoided can be gained, while on the positive side some of the things to do may also be considered. It is known that areas of growth and rates of development of membraneous bone vary at different times during the formative stage of the palate. Growth along the suture line or along a cleft at the normal position of the suture can be anticipated. Also, growth in the region of the tuberosity of the maxilla is such that the end result is that of displacing the maxilla foward. The actual part played by the pterygoid plate and hamulus is not so clearly understood except that its relationship to the posterior portion of the maxilla is significant. Its removal or displacement seems to be a factor in the failure of downward and forward growth of the entire maxilla. Severance of the arterial supply to an area or the introduction of masses of scar all have their adverse effects in the normal growth pattern of the maxilla. Other bones and soft tissue structures may be of prime importance in its ultimate position, but for immediate practical purposes the factors influencing development of the maxilla are a reflection of the same trend in other bones. This does not mean that surgery should not be done upon the palate as has been intimated by some on the basis of cross-sectional studies, nor does it mean that all palates should be closed surgically. In some cases where there is an actual deficiency of tissue or where the tissue is so placed that it cannot be utilized in the proper reconstruction, the defect had better be closed by means of an obturator or false palate. This type of case is frequently observed. In other cases closure of the entire palate is feasible as a one stage operation. Still other patients have palates in which changes in the bone are effected more rapidly by closure of the posterior palate only as a first step. This induces a repositioning of the tongue which, in turn, seems to influence the development of a functional bony architectural pattern. After a lapse of time the remaining portion of the cleft may be repaired or the opening may be allowed to persist in which event t.he
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opening can be closed with an obturator, allowing the closed soft palate to function. In rare instances it is advisable to open the closed posterior cleft if the patient cannot use the palate for adequate closure of the nasopharynx. In such cases the palate is closed by means of an obturator substituting for both the hard and soft palate. The unilateral cleft of the palate and the bilateral cleft of the palate give the most satisfactory responses to surgery in the average case. The most difficult defects are those with a posterior cleft possessing only an intact alveolar ridge. These are usually associated with a congenitally flhort mandible that mayor may not respond to normal stimuli and grow more rapidly than anticipated and, so to speak, catch up with the accepted position of this bone in relationship to the maxilla. The mechanical procedure of closure of the palatal opening is not sufficient to give a functioning palate in terms of adequate closure of the nasopharynx. The structure is basically inadequate as it presents itself at birth. The percentage of failures are far higher in this group than in any other and at the same time are the most misleading to the casual observer in terms of repair. Technically the closure of a cleft of the palate consists of freshening the edges of the defect throughout its entire extent. After this the soft tissues of the palate are elevated from the underlying bone. The extent of the undermining is limited for the same reasons put forth in discussions of closure of the clefts of the lip. Extensive undermining may injure potential growth centers or allow the introduction of scar tissue to the extent that it is an inhibiting factor both by reason of limitation of blood supply and the actual limiting force of the scar itself. As actually practiced no relaxing incisions are used to facilitate apposition of the flaps. No plates or wires are introduced to relieve tension or approximate flaps. The hamular process of the pterygoid is not surgically exposed nor is it fractured. No bone flaps are used. The medial borders of the cleft throughout its entire extent are simply freshened and sutured in the midline with fine silk, dermal or nylon, utilizing a mattress suture as much as possible except in the uvula proper. Here a simple interrupted suture is used. If the cleft cannot be closed by this procedure it is not closed. Either the operation is postponed until more growth has taken place or surgery as a means of closure is abandoned altogether. In certain cases partial closure is done with the plan in mind for a second stage closure. In these cases only the areas that are thought capable of being closed are freshened or their mesial border in order to reduce the amount of scar tissue to be encountered at a subsequent attempt at completion of the closure. Obturators
An obturator or artificial palate is frequently used as a temporary
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or permanent appliance to aid in deglutition and speech. Obturators are placed as early as three years of age in selected cases. These may be placed in order to close off the nasopharynx and obtain a more normal position for the tongue to allow growth to be expressed in a more acceptable manner. Extreme care must be exercised by the prosthodontist to prevent the obturator from hindering development by holding the cleft open. These appliances must be rebuilt often in the growing individual to keep the tissues from forcibly impinging upon the nonresilient obturator. In some cases the obturator is used to maintain spaces for the teeth or to carry false teeth in cases where they are lost or have failed to develop. If the obturator can be anchored to existing teeth it usually functions much better. In some instances they are constructed for completely edentulous mouths. The obturator itself is simple in construction. It is merely an extension of the palatal curve in such a manner that the existing pharyngeal musculature can have something to contact in order to close off the nasopharynx. There are no hinges, bulbs or other complex mechanical devices used in the final product. The success of an obturator depends upon the skill of the operator in measuring the exact amount of function that is present and utilizing it to the utmost. Orthodontia
Given a case of repaired cleft palate that has not been unduly scarred and one possessing a fairly normal complement of teeth and normal developmental growth, the orthodontist can attain a high degree of rehabilit~tion. The age favorable for orthodontic treatment varies. Many of the patients are given treatment on the deciduous teeth. This is a tremendous aid in establishing a normal relationship between the upper and lower jaw. During the transition from deciduous to permanent dentition some of the result is lost but is quickly regained on the permanent dentition. This rapidity of completion of acceptable upper and lower jaw relationship could not be obtained in many cases had not the preliminalY work been carried out on the deciduous teeth. In this respect it might be worth mentioning that the obturator may again play a big part in maintaining the improvement or relationships obtained by treatment on the deciduous dentition, or on the permanent dentition. Speech Training
Almost all persons suffering from a cleft palate defect can benefit from speech training regardless of the success of the surgical closure. Speech training is usually started at 4 years of age so that the patient will have the advantage of some instruction before entering school.
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Treatment integrated with the school curriculum or special intense summer courses or a combination of the two are utilized in order to obtain the maximum results. The mentality of the child, the degree of the defect, intelligence of the parents and .the success of the surgeon, the orthodontist and the prosthodontist are all factors in determining the results that will be obtainei by the speech therapist. SUMMARY
Some of the complexities of the problem of the rehabilitation of persons born with clefts of the lip or palate have been presented. Attention has been directed to the risks involved in operating upon tissues on whose potentialities rest its expression of full normal growth. At the same time, it has been pointed. out that there is no justification for the inferences that have been made recently, on the basis of purely cross sectional studies; viz., that cleft palate surgery is to be entirely avoidei. The surgeon alone cannot completely rehabilitate many individuals born with these deformities; this requires the intelligent cooperation of the orthodontist, the prosthodontist, the speech therapist and other trained specialists. These workers, however, cannot be expected to overcome the handicaps imposed by scarred, denervated and ischemic tissues. REFERENCES 1. Blair, V. P. and Brown, J. B.: Mirault Operation for Single Hare Lip, Surg., Gynec. & Obst. 51: 81-98, 1930. 2. Broadbent, B. H.: The Face of the Normal Child. Angle Orthodontist 7: 209,1937. 3. Brodie, A. G.: On the Growth Pattern of the Human Head from the Third Month to the Eighth Year of Life. Am. J. Anat. 68: 209-262,1\)..41. 4. Davis, Albert D.: Unoperated Bilateral Complete Cleft Lip on l>alate in the Adult. Plast. & Reconstruct. Surg. 7: 482, 1951. 5. Hagedorn: Uber eine Modifikation der Hasenschartenoperation. Centralbl. f. Chir. 11: 756-758, 1884. 6. Hagedorn: Die Operation der Hasenscharte mit Zickzachnacht. Centralbl. f. Chir. 19: 281-285, 1892. 7. Krogman, W. M.: Studies in Growth Changes in the Skull and Face of Anthropoids. Am. J. Anat. 4-6: 303-315, 1930; 4-7: 89, 325, 1931. 8. Le Mesurier, A. B.: A Method of Cutting and Suturing the Lip in the Treatment of Complete Unilateral Clefts. Plast. & Reconstruct.Surg. 4-: 1, 1949. 9. Pruzansky, Samuel: Personal communication. 10. Ricketts, Robert M.: Personal communication. 11. Slaughter, W. B. and Brodie, A. G.: Facial Clefts and Their Surgical Management In View of Recent Research. J. Plast. & Reconstruct. Surg. 4-: 311-332, 1949. 12. Steffensen, W. H.: A Method for Repair of the Unilateral Cleft Lip. Plast. & Reconstruct. Surg. 4-: 144, 1949.
ONE of the common defects afflicting the human race is that of cleft lip, cleft palate or a combination of the two. The care of a person having such a defect is complex in the extreme. The complexity and inadequacy of satisfactory rehabilitation has been somewhat rectified in recent years as a result of the combined efforts of many classifications of specialists in basic research and clinical endeavor. These research findings combined with loosely related bits of information have resulted in improvement of the service aspect of rehabilitation of person with harelip or cleft palate. The real basis for a more optimistic outlook for those born with a cleft of the lip or palate has its foundation in fundamental research plus the more adequate use of associated clinical specialists whose services are extremely beneficial in the total rehabilitation of these patients. The picture of the normal growth pattern of the face as presented by Brodie,3 Broadbent,2 Krogman7 and others has pointed up hitherto unrecognized clues as to what can be accomplished from the standpoint of surgery. These basic works also indicate that there are specific limitations to be placed upon the surgeon's attempts to correct such defects. The timing of surgery in relation to the age of the patient and the extent of the surgical procedure itself are two closely interrelated factors in the successful outcome of such procedures as expressed in terms of normal development. It has been established that the growth pattern of an individual born with a cleft lip or palate endeavors to follow a pattern of growth the basis of which is completely laid down within a few months after birth. This growth pattern is very similar to that of a normal person irerespctive of the type or extent of the cleft. This growth pattern, or more specifically the growth potential of the tissues involved, can be dis-
* Head of Department of Plastic Surgery, University of Wisconsin School of Medicine, Madison, Wisconsin; Chairman, Department of Plastic and Reconstructive Surgery, Stritch School of Medicine, Loyola University and Chairman, Department of Maxillofacial Surgery, Chicago College of Dental Surgery, Loyola University, r:hicago. 165
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turbed enough to disrupt this predetermined pattern by only a few agents. These agents are practically limited to surgery, radiation, severe local trauma, and certain types of infectious processes. The action of these agents must be of a degree sufficient to destroy, damage or some way alter the individual cells having to do with growth and development of a given area. The surgical approach for ultimate closure of a cleft of the lip or palate must therefore be performed so as not to disturb this biologically determined normal growth pattern. In order to carry out a closure of a cleft of the lip or palate and still stay within bounds of the normal physiologic ability of the tissues to overcome the accompanying surgical trauma, and, at the same time establish normal anatomic relationships of the various structures lllvolved, the following principles are being tentatively advanced. UNILATERAL HARELIP
The closure of a simple or unilateral cleft of the lip is usually carried out rather early in life. The time cannot be rigidly defined because this is an elective operation carried out when the patient can best withstand such trauma. The only limitation that can attend such an operation is closure early enough to take advantage of the pliability of the underlying bony structures. For some this will mean surgical closure at two weeks of age while for some it may mean a delay for one to several months after birth. The general condition of the patient, the rate of weight gain and other indicative signs of satisfactory well-being are the determining factors for carrying out the procedure. At the present time all patients being operated upon for closure of a cleft lip receive a general inhalation anesthesia administered by means of an endotracheal tube. The tube is passed through the oral cavity to prevent distortion to the nose or upper lip. With the properly conducted endotracheal anesthesia, the mortality and morbidity rates have been greatly reduced. The rigid control of the airway at all times by the anesthetist, with the accompanying accurate knowledge of the plane of anesthesia, allow for a much more rapid completion of the operation itself. The ability to perform a more satisfactory postoperative endotracheal toilet and the rapid return to consciousness are also factors contributing to more acceptable results. Closure of the lip proper follows in general the procedures outlined some seventy years ago by Hagedorn 5 • 6 (Fig. 71, a, b, c). To this early contribution must be added the refinements and modifications introduced by Steffensen,12 Le Mesurier,8 Blair and Brown! and others. Specifically the operation calls for freshening the sides of the cleft by clean through-and-through incisions. On the medial side the anatomical boundaries of the normal philtrum are followed to facilitate adherence to normal tissue relationships as much as possible (Fig. 71, c, d, e). The medial portion of the lateral or short segment is freshened in such a
Harelip and Cleft Palate Defects
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manner as to loosely dovetail into the incision previously made in the philtrum. As will be noted in comparing the methods, the notch is made considerably lower than originally suggested by Hagedorn. This is done merely to more closely follow the dictates of accepted anatomic relationships. No incision or undermining is carried beyond the midline. This is in accordance with long established plastic surgery principles that to do so may be injurious to the flaps themselves in addition to the fact that this procedure may be a factor in changing the direction of the
Fig. 71. a, b, c, Closure of the lip. Redrawn from Hagedorn, Centralbl. fUr Chir. 19: 281,1892. d, e,!, Modification of Hagedorn's method in which the notching is nearer the mucocutaneous junction in order to more closely follow the normal configuration of the philtrum.
normal muscle forces resulting in irreversible abnormal structural relationships. Laterally the flaps are undermined in as limited a manner as possible over the anterior maxillary wall. Again this is in compliance with accepted dictates not to overextend the contained blood supply of a given flap. The attachment of the lower lateral or inferior alar cartilage is freed from the proximity of the maxilla to allow it to be rotated medially to aid in the formation of the floor of the nostril and re-establish a more normal nasolabial fold. These mobilized structures are moved into position and sutured in the desired relationship. This is done without any manual or forceful "molding," fracturing or contouring of t.he underlying delicate facial bones. The lip is closed over the defect
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in the bone so that it may act as a physiologic force to accomplish the desired change in contour. The gentle action of the closed lip exerted in the proper direction over a long period of time brings the structures into a more normal position by the interplay of muscular forces between the lip and tongue and their accessory supportive structures. The guiding or directing of the growth potential accomplishes the desired end result slowly and positively but does not interfere with growth factors of the involved structures by destroying existing or potential growth centers or by introducing damaging amounts of scar tissue. The floor of the nose is first closed using 4-0 silk. Care is taken to insure prop~r relationships of tissues at the nostril opening because of its importance both from a cosmetic and functional standpoint. The mucous membrane and muscle of the lip are brought into apposition by figure-of-8 sutures. The suture is started on the mucous membrane surface so that the knot may be placed on this surface thereby minimizing the scarring of the skin surface of the lip. This suture acts as a tension suture for the entire lip and the remaining sutures placed in the skin of the lip are purely for appositional purposes. As a rule a subcuticular suture is placed in the skin of the lip using fine horsehair. This practically eliminates any stitch marks on the skin of the lip itself. Again it must be emphasized that all unnecessary trauma in the form of crushing, clamping or molding of both hard and soft tissues is potentially harmful. The introduction of scar tissue into rapidly growing structures will eventually have a restraining action upon the development of the entire upper jaw. All too often the undue scarring of the lip acts as a powerful antagonist to the normal downward and forward growth of the upper jaw. This may be a major factor in producing a tight upper lip, one not possessing normal mobility because of the binding action of the contained scar tissue. It is well known that the surgical repair of any soft tissue structure has for its successful basis the introduction of some scar tissue as part of the normal healing process. A preponderance of scar tissue, however, will result in some limitation· of motion or gross physical deformity. In the lip this is expressed most often by a tight scarred lip that is usually anatomically longer than nature intended. A resulting discrepancy in growth between the upper and lower jaw results in the relatively prognathic or protruding jaw as an end result. Almost invariably the lower jaw is merely expressing its normal growth potential and the upper jaw fails to keep up with this growth picture because of the limitations imposed by the scarred lip plus possible interference with growth centers in the underlying bone itself.H It appears that the closure of the single cleft of the. lip must fulfill the following requirements: First, the congenitally separated tissues must be brought into relatively normal anatomic relationship. This can only be done surgically. As a rule the necessary ti5sues for such a
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reconstruction are present since the defect is not one of deficiency of tissue but merely one of displacement of tissue. Second, the closure of such a defect must be carried out in such a manner as not to interfere with the normal expression of growth potential of the tissues involved. Third, the position of the tissues in their new relationship must be such that even after rotation or medial deviation of the premaxilla (os incisivum) over a period of months or years the resulting lip is passable both cosmetically and functionally. Too often the stigma of a cleft lip facies is not the result of the shortcomings of nature but the result of overly zealous activities on the part of those sincerely trying to rehabilitate one so unfortunate as to be born with such a defect. BILATERAL HARELIP
The closure of a bilateral cleft of the lip presents all of the problems of the single cleft and frequently a disproportionate growth between the vomer, premaxilla and maxilla. The problem is not only that of closing two single clefts of the lip in one individual; there is the added problem of utilizing a philtrum that is not only displaced but also deficient in blood supply for the surgery that should be done. The closing of the lip over the existing bony openings has for its prime purpose the establishment of a physiological directing face on the subsequent development of the maxillary area. This should be carried out without resort to molding, fracturing or cutting of the rapidly developing structures involved any of which procedures are apt to interfere with the full expression of the growth potential. Since the blood supply of the philtrum is distributed in such a way that it is only logical to sever one side at a time, this is a major factor in the choice of operations to be carried out. Since the position of the premaxilla is frequently such that the soft tissues cannot be brought over it without causing undue tension on the lip structures or upon the underlying bony structures or both, it seems logical to close the lip in two stages. The double cleft is converted into a single cleft, then the single cleft is closed at a second operation. The initial operation is carried out early in life. If one side presents a wider defect it is closed first. The prerequisites for operation are the same as for the single cleft lip. The cleft is surgically closed using the simple principles as originally outlined by Hagedorn. The flaps made in the philtrum proper are altered slightly in order to conserve as much of the tissue as possible. This conservation of tissue is not carried to the point that tissue of the philtrum itself enters into the final lip below the mucocutaneous junction. To bring philtrum skin below this point results in a lip inadequate in functional capabilities. The first operation is carried out as outlined for the single cleft of the lip. This results in a change in position of the premaxilla due to the tension resulting from the closure. At first the closed side shows rapid
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changes in the relationship of the alveolar borders of the cleft in that they move toward one another. This may continue until there is contact between the two segments. While this is happening the cleft on the unrepaired side at first becomes more prOIlOunced. After a few weeks, however, there are compensatory changes well back in the septum that eventually manifest themselves in the premaxilla. The entire premaxilla moves into a more no.rmal position in relation to the frontal facial plane. This is thought to be due to the lack of displacement forces previously exerted by the tongue directly and the lateral pull of the lip muscles indirectly. Another change also takes place while the above mentioned rotation and displacement process is occurring. This is expressed as an apparent forward movement of the maxilla. Actually it is the addition of bone to the maxilla making the true anterior-posterior dimension of that bone greater. Therefore, what appears to be a greatly displaced premaxilla on first examination may be only slightly displaced after growth allowances are made Pruzansky and Ricketts are of the opinion that this additive growth on the maxilla, if liot interfered with, may in many instances be sufficient to produce a fairly normal upper arch irrespective of the width of the cleft. 9. 10 The operation for closure of the second side of the double cleft of the lip may be any time from six weeks to six months or longer after the first operation. The position of the segments and the general condition of the patient determine the actual time for closure. When the second side of the lip cleft can be closed without undue tension then the procedure is carried out. One of the unfortunate aspects of surgery on bilateral clefts of the lip is that the immediate cosmetic results are usually far from satisfactory. The lip must be closed with the degree of migration of the underlying bony segments kept in mind. Usually the nostrils appear flared and the tip of the nose is flat and the lip appears to be short. But with the knowledge that this will eventually assume a more normal position the program for rehabilitation can be carried on without undue apprehension. In closing a double cleft of the lip none of the principles of plastic surgery are violated. There is no molding, shaping, contouring, cutting or fracturing of bony or cartilaginous structures on the part of the operator. This is all left to the normal muscle interplay that will take place after the soft tissues are brought into proper apposition. 4 There is no substitution of tissues or structures for the sake of immediate cosmetic results. The normal relationship of the philtrum to the columella is maintained together with the relationship of these structures to the nose itself. It is admitted that the nose appears flat after the operation but substitution of the philtrum for a portion of the columella in order to elevate the nose is not a satisfactory answer in the final analysis.
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Adequate growth of the alar cartilages is just as important as growth of other structures if a normal face is to be expected. After this growth has taken place the necessary cosmetic procedures can be carried out without unforeseen disfiguring results. The tremendous changes obtained by competent orthodontists in directing the growth in the double cleft patient cannot be overemphasized. This point will be enlarged upon later. CLEFT PALATE
The problem of closing a congenital cleft of the palate is so well known and wrought with such controversies that an attempt to delve into only a few of the difficulties would only complicate the picture. On the basis of measurements of cephalometric x-rays, plus those taken of plaster casts over a period of years, an impression of some of the steps to be avoided can be gained, while on the positive side some of the things to do may also be considered. It is known that areas of growth and rates of development of membraneous bone vary at different times during the formative stage of the palate. Growth along the suture line or along a cleft at the normal position of the suture can be anticipated. Also, growth in the region of the tuberosity of the maxilla is such that the end result is that of displacing the maxilla foward. The actual part played by the pterygoid plate and hamulus is not so clearly understood except that its relationship to the posterior portion of the maxilla is significant. Its removal or displacement seems to be a factor in the failure of downward and forward growth of the entire maxilla. Severance of the arterial supply to an area or the introduction of masses of scar all have their adverse effects in the normal growth pattern of the maxilla. Other bones and soft tissue structures may be of prime importance in its ultimate position, but for immediate practical purposes the factors influencing development of the maxilla are a reflection of the same trend in other bones. This does not mean that surgery should not be done upon the palate as has been intimated by some on the basis of cross-sectional studies, nor does it mean that all palates should be closed surgically. In some cases where there is an actual deficiency of tissue or where the tissue is so placed that it cannot be utilized in the proper reconstruction, the defect had better be closed by means of an obturator or false palate. This type of case is frequently observed. In other cases closure of the entire palate is feasible as a one stage operation. Still other patients have palates in which changes in the bone are effected more rapidly by closure of the posterior palate only as a first step. This induces a repositioning of the tongue which, in turn, seems to influence the development of a functional bony architectural pattern. After a lapse of time the remaining portion of the cleft may be repaired or the opening may be allowed to persist in which event t.he
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opening can be closed with an obturator, allowing the closed soft palate to function. In rare instances it is advisable to open the closed posterior cleft if the patient cannot use the palate for adequate closure of the nasopharynx. In such cases the palate is closed by means of an obturator substituting for both the hard and soft palate. The unilateral cleft of the palate and the bilateral cleft of the palate give the most satisfactory responses to surgery in the average case. The most difficult defects are those with a posterior cleft possessing only an intact alveolar ridge. These are usually associated with a congenitally flhort mandible that mayor may not respond to normal stimuli and grow more rapidly than anticipated and, so to speak, catch up with the accepted position of this bone in relationship to the maxilla. The mechanical procedure of closure of the palatal opening is not sufficient to give a functioning palate in terms of adequate closure of the nasopharynx. The structure is basically inadequate as it presents itself at birth. The percentage of failures are far higher in this group than in any other and at the same time are the most misleading to the casual observer in terms of repair. Technically the closure of a cleft of the palate consists of freshening the edges of the defect throughout its entire extent. After this the soft tissues of the palate are elevated from the underlying bone. The extent of the undermining is limited for the same reasons put forth in discussions of closure of the clefts of the lip. Extensive undermining may injure potential growth centers or allow the introduction of scar tissue to the extent that it is an inhibiting factor both by reason of limitation of blood supply and the actual limiting force of the scar itself. As actually practiced no relaxing incisions are used to facilitate apposition of the flaps. No plates or wires are introduced to relieve tension or approximate flaps. The hamular process of the pterygoid is not surgically exposed nor is it fractured. No bone flaps are used. The medial borders of the cleft throughout its entire extent are simply freshened and sutured in the midline with fine silk, dermal or nylon, utilizing a mattress suture as much as possible except in the uvula proper. Here a simple interrupted suture is used. If the cleft cannot be closed by this procedure it is not closed. Either the operation is postponed until more growth has taken place or surgery as a means of closure is abandoned altogether. In certain cases partial closure is done with the plan in mind for a second stage closure. In these cases only the areas that are thought capable of being closed are freshened or their mesial border in order to reduce the amount of scar tissue to be encountered at a subsequent attempt at completion of the closure. Obturators
An obturator or artificial palate is frequently used as a temporary
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or permanent appliance to aid in deglutition and speech. Obturators are placed as early as three years of age in selected cases. These may be placed in order to close off the nasopharynx and obtain a more normal position for the tongue to allow growth to be expressed in a more acceptable manner. Extreme care must be exercised by the prosthodontist to prevent the obturator from hindering development by holding the cleft open. These appliances must be rebuilt often in the growing individual to keep the tissues from forcibly impinging upon the nonresilient obturator. In some cases the obturator is used to maintain spaces for the teeth or to carry false teeth in cases where they are lost or have failed to develop. If the obturator can be anchored to existing teeth it usually functions much better. In some instances they are constructed for completely edentulous mouths. The obturator itself is simple in construction. It is merely an extension of the palatal curve in such a manner that the existing pharyngeal musculature can have something to contact in order to close off the nasopharynx. There are no hinges, bulbs or other complex mechanical devices used in the final product. The success of an obturator depends upon the skill of the operator in measuring the exact amount of function that is present and utilizing it to the utmost. Orthodontia
Given a case of repaired cleft palate that has not been unduly scarred and one possessing a fairly normal complement of teeth and normal developmental growth, the orthodontist can attain a high degree of rehabilit~tion. The age favorable for orthodontic treatment varies. Many of the patients are given treatment on the deciduous teeth. This is a tremendous aid in establishing a normal relationship between the upper and lower jaw. During the transition from deciduous to permanent dentition some of the result is lost but is quickly regained on the permanent dentition. This rapidity of completion of acceptable upper and lower jaw relationship could not be obtained in many cases had not the preliminalY work been carried out on the deciduous teeth. In this respect it might be worth mentioning that the obturator may again play a big part in maintaining the improvement or relationships obtained by treatment on the deciduous dentition, or on the permanent dentition. Speech Training
Almost all persons suffering from a cleft palate defect can benefit from speech training regardless of the success of the surgical closure. Speech training is usually started at 4 years of age so that the patient will have the advantage of some instruction before entering school.
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Treatment integrated with the school curriculum or special intense summer courses or a combination of the two are utilized in order to obtain the maximum results. The mentality of the child, the degree of the defect, intelligence of the parents and .the success of the surgeon, the orthodontist and the prosthodontist are all factors in determining the results that will be obtainei by the speech therapist. SUMMARY
Some of the complexities of the problem of the rehabilitation of persons born with clefts of the lip or palate have been presented. Attention has been directed to the risks involved in operating upon tissues on whose potentialities rest its expression of full normal growth. At the same time, it has been pointed. out that there is no justification for the inferences that have been made recently, on the basis of purely cross sectional studies; viz., that cleft palate surgery is to be entirely avoidei. The surgeon alone cannot completely rehabilitate many individuals born with these deformities; this requires the intelligent cooperation of the orthodontist, the prosthodontist, the speech therapist and other trained specialists. These workers, however, cannot be expected to overcome the handicaps imposed by scarred, denervated and ischemic tissues. REFERENCES 1. Blair, V. P. and Brown, J. B.: Mirault Operation for Single Hare Lip, Surg., Gynec. & Obst. 51: 81-98, 1930. 2. Broadbent, B. H.: The Face of the Normal Child. Angle Orthodontist 7: 209,1937. 3. Brodie, A. G.: On the Growth Pattern of the Human Head from the Third Month to the Eighth Year of Life. Am. J. Anat. 68: 209-262,1\)..41. 4. Davis, Albert D.: Unoperated Bilateral Complete Cleft Lip on l>alate in the Adult. Plast. & Reconstruct. Surg. 7: 482, 1951. 5. Hagedorn: Uber eine Modifikation der Hasenschartenoperation. Centralbl. f. Chir. 11: 756-758, 1884. 6. Hagedorn: Die Operation der Hasenscharte mit Zickzachnacht. Centralbl. f. Chir. 19: 281-285, 1892. 7. Krogman, W. M.: Studies in Growth Changes in the Skull and Face of Anthropoids. Am. J. Anat. 4-6: 303-315, 1930; 4-7: 89, 325, 1931. 8. Le Mesurier, A. B.: A Method of Cutting and Suturing the Lip in the Treatment of Complete Unilateral Clefts. Plast. & Reconstruct.Surg. 4-: 1, 1949. 9. Pruzansky, Samuel: Personal communication. 10. Ricketts, Robert M.: Personal communication. 11. Slaughter, W. B. and Brodie, A. G.: Facial Clefts and Their Surgical Management In View of Recent Research. J. Plast. & Reconstruct. Surg. 4-: 311-332, 1949. 12. Steffensen, W. H.: A Method for Repair of the Unilateral Cleft Lip. Plast. & Reconstruct. Surg. 4-: 144, 1949.