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The Treatment of Hypospadias
The Treatment of Hypospadias DONALD W. MAcCOLLUM, M.D., F.A.C.S. * LUTHER A. LONGINO, M.D., F.A.C.S. ** 1. A. MEEKER, JR., M.D.t
THE term hypospadias is of Greek derivation and when literally translated means "to draw under." Unfortunately this "drawing under" results in a ventral bowing of the phallus. This downward curvature of the penis caused by the restriction of growth on the ventral surface is known as a chordee. Usually this chordee is accompanied by an abnormal position of the urinary meatus and is apt to be more severe in those cases in which the opening is in the perineal region as compared to those cases in which it is in the coronal region. In all forms of hypospadias there is a hooded foreskin. When this foreskin is redundant it may make one think that a great deal more of a chordee is present than actually exists. Occasionally in some severe forms of hypospadias a very much underdeveloped or diminutive phallus is found. EMBRYOLOGY
The external genitalia arise from a single or homologous anlage which appears about the sixth week of fetal life as an undifferentiated genital tubercle located midway between the attachment of the umbilical cord and the coccygeal tubercle. On the under surface of this rudimentary structure a shallow urethral groove is formed which is bordered on each side by parallel elevated urethral folds. Just lateral to these folds rounded prominences appear which represent the labioscrotal swellings. During the tenth to fourteenth week of gestation these structures begin to be differentiated in a pattern that eventually will develop into either the male or female organ. The masculinization of the undifferentiative external genitalia consists '" Assistant Clinical Professor of Surgery, Harvard Medical School; Surgeon, Children's Medical Center; Senior Associate Surgeon (Plastic), Peter Bent Brigham Hospital, Boston. ** Clinical Associate in Surgery, Harvard Medical School; Surgeon, Children's Medical Center, Boston. t Former Resident in Surgery, Children's Medical Center, Boston.
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of a marked elongation and downward growth of the tubercle until it assumes the shape of the male penis capped on its tip by the glans. As this is happening the parallel folds on either side of the ventral urethral groove unite to form a penile urethra that extends from the base of the penis to the tip of the glans. If there is an arrest in the normal development, the fusion of these urethral folds may be delayed so that the opening may occur at any point from the coronal to the perineal position. In addition to the failure of development of the normal urethra, there is also an arrest in the formation of the corpus spongiosum. As a result, the corpus spongiosum is abnormally shortened and fibrotic, which in turn causes the penis to be bound down to the perineum to some degree. METHODS OF MANAGEMENT
Numerous operative procedures have been proposed and employed for the correction of hypospadias. Noone procedure has been found to be ideal in every case or for every surgeon. An excellent review of the historical background and outline of the methods used in the repair of hypospadias has been given by Young and Benjamin. l Smith and Blackfield2 have also prepared an excellent critique on the repair of hypospadias. It is not the purpose of this paper to review in detail all of the operative procedures that have been recommended in the past for the correction of this deformity. However, most previous reports outline repairs that fall into two main categories: (a) those that employ local flaps of the penile skin to form a new urethral tube (Ombredanne,3 Bucknall,4 Duplay,S and Brown 6), and (b) those that resort to free grafts that are buried in the penis to form the new channel (N ove-Josserand, 7 McIndoe,8 Havens 9 ). Ombredanne3 fashioned a urethral extension by folding a scrotal flap forward and upward to unite with an isolated strip of skin on the ventral surface of the penis. The denuded surface left by the construction of these flaps was covered by scrotal and penile skin that had been undermined and drawn together over the defect. A wide patulous urethra usually was created that was prone to calculus formation around hairs which grew in the newly created tube. With the penis in a flaccid state the urethral meatus was often so wide that accurate control of the urinary stream was impossible. In erection the patient's penis tended to extend far beyond the constructed meatus so that it was no longer near. enough to the tip to make insemination of the female possible. This type of operation was carried out in 13 cases at the Children's Medical Center and was then discarded because the results were not satisfactory to either the patient or the surgeon. Buckna1l4 devised another procedure somewhat similar in principle to the Ombredanne repair, in which the penis was "hinged" downward at
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the level of the urethral opening while a penile and a perineal flap were sewn together to form a new urinary channel. The penis was detached from the perineum at a second operation and the denuded areas covered by skin from adjacent areas.' Because of the long suture lines, healing was not always as perfect as one would wish, so that fistulas occurred frequently. At puberty the growth of hair on the portion of the urethra obtained from the scrotal or perineal flap resulted in irritation and calculus formation in the new urethra. The Thiersch-Duplay procedure as modified by Cecipa consisted:of creating a ventral flap of tissue between two parallel incisions that extended from the ectopic meatus to the tip of the glans. This strip was undermined on each side so that the edges could be brought over a catheter and sewn together to form a new skin-lined channel. The remaining penile skin was undermined extensively and brought together over the new skin tube. Because of the wide undermining it was difficult to obtain complete hemostasis. Hematomas were common, as were fistulas along the long superimposed suture lines. The results in some cases were excellent but in others numerous complications occurred. More recently Browne 6 has developed an operation based somewhat on the principles of the Thiersch-Duplay method. Two parallel incisions isolate a strip of ventral skin from the meatus to the corona of the penis. The lateral margins of these two incisions are widely undermined and brought together over this strip of skin. This imbedded strip then produces a new urinary channel by growing epithelium from each edge until union is complete in the form of a tube. The chief advantage of this method is that it may be performed at an early age thereby eliminating any psychological embarrassment from this abnormality. It would seem well indicated for those cases with midshaft openings. For those severe cases in which the opening is in the penoscrotal or perineal region, again hair may form in the new urethra if hair-bearing skin has been buried under the penile flaps. In the severe case an excessive amount of deep scarring is present due to the tubulating of the original skin strip. It is believed that this may seriously limit extension of the penis in erection. Excellent early results have been reported from many clinics in which this method of repair has been followed. The construction of a penile urethra by means of free grafts was first described in 1897 by Nove-Josserand. 7 So many complications in the nature of incomplete "takes" of the graft, strictures and fistulas made this method fall into disrepute until it was revised and modified by McIndoe 8 , 11 in 1937 and Havens4 in 1949. This "free graft" method of repair as reported previously by Gross12 has been found to be most satisfactory and currently is being employed at the Children's Medical Center of Boston.
,
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Donald W. MacCollum, Luther A. Longino, I. A. Meeker CLINICAL MATERIAL
In our series of 443 patients with hypospadias the following positions of the urethral meatus have been observed: coronal, 263; penile midshaft, 47; penoscrotal or perineal, 133. When the urinary opening is at the level of the corona and is of adequate diameter, there is seldom any need for operative correction. In this type of deformity the shaft of the penis is usually straight. Micturition in a standing position can be carried out without difficulty. Intercourse with subsequent fertilization also should be accomplished quite normally. Occasionally this urethral opening may be pinpoint in size. If this is found, early dilatation or meatotomy is done to prevent the occurrence of chronic urinary obstruction. In all cases with inadequate urinary openings and in all cases with the more severe types of hypospadias, intravenous pyelography is carried out. Chronic obstruction is rarely demonstrated; associated anomalies of the upper urinary tract are rarely found. Even though other abnormalities of the urinary tract may be infrequent, associated anomalies of the reproductive tract may be many and varied. If anorchism or cryptorchidism is found to occur in conjunction with hypospadias, immediately the sex of the individual is under suspicion. A careful rectal examination will be helpful in ruling out the presence or absence of a uterus. However, this determination may not be definitive in a newborn baby and even may be equivocal in the first few years of life. Examination of the urethra by means of injection of radiopaque substances may also be helpful. Instead of a normal urethra a vaginal pouch may be shown to open directly into a dilated urogenital sinus. Collection of urine for the determination of the 17-ketosteroids is also done to rule out an adrenogenital syndrome in which the clitoris of the female patient is enlarged to resemble the hypospadiad penis of the male. If the rectal examination is negative, if the x-rays of the urethra are normal, and if the excretion of the 17-ketosteroids are also found to be normal one must still question the true gender of the individual. It is in the latter case that an exploratory laparotomy is indicated. In addition to inspection of the pelvic organs, the gonads should be biopsied, even though grossly they may resemble either testes or ovaries or both (testicular and ovarian structures in the same organ; e.g., ovo-testes). In our series of 133 patients with severe hypospadias we have performed abdominal explorations on 18. On these 18, eight were found to . have female internal genitalia and three more were found to be true hermaphrodites. It is obvious that an abdominal exploration should be made as soon after birth as it is possible to perform it without danger to the individual. To delay these sex determinations until early childhood or later, may impose a heavy psychological burden and an almost superhuman readjustment upon both the parents and the child.
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TREATMENT
In order to treat a hypospadias patient satisfactorily, it is necessary to create a penis that will function properly both for micturition and for procreation. In addition, it is necessary that both the patient and his parents be handled in such a fashion that no abnormal psychological traits develop. It is our opinion that parental pressure, exerted to avoid embarrassment for the child when he reaches school age, has forced many surgeons to attempt correction of this deformity at too early an age. Many of these early operations have not only been unsuccessful but frequently they have permanently mutilated the organ to such an extent that an adequate repair at a later age becomes impossible. It is felt in the Children's Medical Center that if proper and continuous individualized attention is given to such a patient that almost any unpleasant psychological reaction to this deformity can be avoided. This requires the establishment of a close relationship between not only the parents and the surgeon but most particularly between the patient and his doctor. The correction of the mild hypospadias deformity in the patient who has no appreciable chordee has been eliminated entirely at the Children's Medical Center. If the penis is of normal size, the movement of the urethral meatus to the tip of the penis has not been found to be necessary for either urination or insemination. In this mild type of deformity it might be wise to excise the hooded foreskin for both sanitary and cosmetic reasons. In the more severe hypospadias deformities, a series of operations has been found to produce very satisfactory results. In all cases a circumcision of the hooded prepuce is to be avoided. It may be necessary to transpose this redundant tissue to fill in the defect left on the ventral surface of the penis at the time of the correction of the chordee. This correction of the chordee constitutes the first of a three stage procedure. First Stage (Fig. 507) The child is admitted to the hospital when between nine and ten years of age for this first operation. In those cases in which the chordee is severe an earlier operation is occasionally carried out and a partial straightening accomplished. In the latter case a second chordee correction is carried out at the ten year age level and further lengthening of the ventral surface obtained. A Foley catheter is first placed in the bladder so as to divert the urinary flow during the early healing stage. An inverted "V"-shaped incision is then made just distal to the urethral opening. This incision is developed so that the tight fibrous midline cords of tissue are 61xposed. These cords, along with a narrow strip of the foreshortened ventral skin, are then excised from this point to the level of the normal meatal opening. The
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Fig. 507. Correction of chordee. a, Diagram of chordee with urethral orifice at penoscrotal junction. b, Penis sutured to anterior abdominal wall. Catheter in proximal urethra for constant drainage. c, d, Incisions for removal of fibrous bands and tight ventral midline contractures. e, Lateral incisions for relaxation of the ventral surface of the penis. f, g, h, Method of suturing to obtain the greatest possible length to the under surface of the penis. (Reprinted from R. E. Gross: Surgery of Infancy and Childhood, W. B. Saunders Co., 1953.)
corpus spongiosum is largely absent and, if present at all, is represented by a group of tight fibrotic bands. All of these firm constricting elements are removed. Mter all the deep fibrous tissue that caused the ventral bowing has been removed, cross incisions are made at different levels in the ventral skin so as to produce either a single Z-plasty flap, or a series
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of them in the severest cases. These flaps are interposed and sewn in their new positions with both mattress and single sutures of 5-0 plain catgut. It is important that no tension whatever exists on this suture line when the flaps are united. To produce sufficient relaxation it may be necessary to undermine the skin quite extensively up over the dorsum of the penis. It is at this point that the redundant skin of the prepuce becomes of importance and allows a shift in the skin coverings in such a fashion that all tension will be avoided on the ventral suture line. This single or multiple Z type of closure has the tendency to prevent any subsequent scar contracture which one is apt to get if there has been a straight line closure. A heavy gauze dressing is applied which is held in position under mild pressure with strips of elastoplast. Because of the extensive undermining during this first stage, bleeding is sometimes difficult to control. Hot packs, multiple sutures and pressure dressings are always necessary. If hemostasis is not absolute, hematomas will form beneath the flaps and lead to either rupture of the suture line or deep scarring, both of which will produce some tightening of the ventral surface and defeat the whole purpose of the first stage. Second Stage (Fig. 508)
This stage consists of creating a new penile urethra by means of a free, inlay, split thickness graft. So as to be certain of making a urethra of adequate length, this second stage is usually delayed until the penis reaches more adult proportions (14 to 16 years of age). The four to six year interval between the first and second stage is sometimes difficult for the boy psychologically, because it is necessary for most patients to sit down each time that they urinate. Usually a detailed and careful explanation of the entire problem, emphasizing particularly the goal to be obtained, is sufficient to avoid permanent psychological damage. Again a Foley catheter is placed in the bladder and again an inverted "V"-shaped incision is made just distal to the urethral opening. Through this incision a large metal trocar is passed up through the length of the penis to emerge at the position where the normal meatus should be. The tip and handle of the trocar are then unscrewed, leaving the hollow metal shaft of the trocar imbedded in the penis. A thin razor graft is next taken from the thigh and is rolled, raw side out, around a No. 16 French catheter. Each end of the graft is then sewn to the catheter so that it will not be displaced or wrinkled as the tube is being drawn into the metal trocar left in the penis. After the catheter carrying the graft has been placed inside the trocar shaft, the metal tube is removed leaving the catheter bearing the graft in the position of the new urethra. The graft is tacked to the glans with interrupted sutures of 5-0 plain catgut and a small safety-pin put through the catheter at each end so as to orevent displacement with movement. A firm compression dressing which
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Fig. 508. a, b, c, Method of tunneling the ventral surface of the penis to receive graft. d, e, Split thickness graft wrapped around and tied to No. 16 rubber catheter. " Dismantling of trocar for insertion of graft. (Continued on facing page.)
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Fig. 508 (Continued). g, h, i, Placement of graft through hollow barrel of trocar. k, Split thickness graft in position to make a new channel in the penis. It is anchored to the glans only. (Reprinted from Gross: Surgery of Infancy and Childhood, W. B. Saunders Co., 1953).
i, Removal of metal cylinder with graft remaining in place.
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holds the penis up against the abdominal wall is then applied. After a week, the Foley catheter in the bladder is removed. At this same time the urethral catheter is changed and a new tube of the same diameter reinserted. After a few more days the patient is shown how to
Fig. 509. a-f. Establishment of perineal urethrotomy. (Cemtinued em facing page.)
carry out the daily changing of this tube which now acts as a urethral splint and prevents any contracture of the lumen. During the next six months, this strut is worn for decreasing periods of time. At the end of the:six month period all tendency for contracture of the graft should have passed so the patient is now ready for the final stage of the procedure.
k
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Third Stage (Fig. 509)
As in the previous two stages, we find it advisable to divert the urinary flow so that there will be no contamination of the operative site during the healing period. A perineal urethrotomy is first performed. If there are any objections to placing the urethrotomy in the perineal region, a
Fig. 509 (Continued). g-l, Method of anastomosis of the skin graft channel to the proximal urethra. (Reprinted from Gross: Surgery of Infancy and Childhood, W. B. Saunders Co., 1953).
suprapubic cystotomy may be carried out. After this has been accomplished, a catheter is inserted through the newly constructed urethra and into the normal urethra so that it will bridge the gap between the two. An oval incision is made around this site and the innermost flaps undermined so they may be closed over the bridging catheter. This closure is
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accomplished by a continuous Connell suture of 5-0 plain catgut. A reinforcing layer of tissue is brought over this first suture line and is held here by interrupted sutures of 5-0 arterial silk. The lateral edges of the oval incision are then undermined and the closure carried out in two
Fig. 510. A, Photograph showing chordee with arrow pointing to urethral orifice at penoscrotal junction. B, After correction of chordee with lengthening of the penis. C, Graft in place with a No. 16 rubber catheter through the newly formed skin-lined channel. D, Six months after completion of Stage III showing the patient voiding through the newly created urethra.
layers using either 5-0 plain catgut or 5-0 chromic catgut. At the end of the procedure the urethral catheter is removed and a light compression dressing is applied. The perineal urethrotomy or suprapubic cystotomy catheter is removed on the tenth postoperative day at which time the
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patient now begins to use his new urethra. The urethrotomy or cystotomy openings will close spontaneously within 48 hours. RESULTS AND COMPLICATIONS
To date, 68 patients have been operated upon for the relief of the chordee with highly satisfactory results. Three of these patients developed hematomas following the release of the chordee and required a second operation for the evacuation of the clot and resuturing of the flaps. None of these three developed unusual scarring subsequently, so there was no permanent damage to the penis. In 33 of these 68 patients, all three stages described above for the creation of a new urethra have been completed. Two of these 33 patients developed fistulas after the third stage and each required another operative procedure to close the fistulas. One patient developed a stricture at the junction of the skin tube and the natural urethra. Repeated dilatations were not successful in relieving it, so an exploratory operation was then done to remove the scar tissue and reanastomose the ends. This was successful in every respect. In each of these four cases the complications were corrected easily so that the end result in all 33 cases was completely satisfactory. SUMMARY
Four hundred and forty-three patients with hypospadias have been seen in the Plastic Surgery Clinic of the Children's Medical Center of Boston. A great many of these patients had only mild deformities which required no surgical correction. For those patients with severe hypospadias deformities, a three stage corrective procedure has been outlined. The first stage consists in release of the chordee sometime before ten years of age. The second stage is performed between 14 and 16 years of age. By means of a free inlay graft this stage embodies the creation of a new penile urethra of sufficient diameter and length for an adult sized penis. The third stage is performed six months to a year after stage two and consists in uniting the new urethra with the natural urethra. The principal disadvantage to this method of hypospadias repair is the delay encountered before the three stages are completed. Many observers believe that the psychological damage from this delay is excessive. In our own clinic we have observed that individualized attention and establishment of a close doctor-patient relationship, plus a thorough understanding of the problem by the patient, has done much to minimize this psychological trauma. The advantages of this method of repair are many. The three stages of the repair are simple and require no complicated reconstructive steps. Complications following each stage have been infrequent and when they did occur were corrected by minor secondary operative procedures. To
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date, every patient whose repair has been completed now has a penile organ that is satisfactory both cosmetically and functionally. REFERENCES 1. Young, F. and Benjamin, J. A.: Preschool Age Repair of Hypospadias with Free Inlay Skin Graft. Surgery 26: 384-404, 1949. 2. Smith, D. R. and Blackfield, H. M.: A Critique on the Repair of Hypospadias. . Surgery 31: 885, 1952. 3. Ombredanne: Hypospadias Penien Chez. I'enfant. Bull. et memo Soc. de Chu. de Paris 37: 1076-1078, 1911. 4. BucknaII, R. T. H.: A New Operation for Penile Hypospadias. Lancet 2: 887890,1907. 5. Duplay, Simon: Nouvelle methode operatoire applicable au traitement de l'hypospadias perin eo-scrotal. Arch. gen. de me.d. 23(VIe ser): 657-682, 1874. 6. Browne, Denis: An Operation for Hypospadias. Proc. Roy. Soc. Med. 42: 466468, 1949. 7. Nove-Josserand, Gabriel: Traitement de l'hypospadias, Nouvelle methode. Lyon med. 10: 184, 1907. 8. McIndoe, A. H.: Deformities of the Male Urethra. Brit. J. Plastic Surg. 1: 29-47, 1948. 9. Havens, F. Z. and Black, A. S.: The Treatment of Hypospadias. J. Urol. 61: 1053-1064, 1949. 10. Cecil, A. B.: Surgery of Hypospadias and Epispadias in the Male. J. Urol. 27: 507-537, 1932. 11. McIndoe, A. H.: The Treatment of Hypospadias. Am. J. Surg. 38: 176-185,1937. 12. Gross, R. E.: Surgery of Infancy and Childhood. Philadelphia, W. B. Saunders Co., 1953. 300 Longwood Avenue Boston, Massachusetts
THE term hypospadias is of Greek derivation and when literally translated means "to draw under." Unfortunately this "drawing under" results in a ventral bowing of the phallus. This downward curvature of the penis caused by the restriction of growth on the ventral surface is known as a chordee. Usually this chordee is accompanied by an abnormal position of the urinary meatus and is apt to be more severe in those cases in which the opening is in the perineal region as compared to those cases in which it is in the coronal region. In all forms of hypospadias there is a hooded foreskin. When this foreskin is redundant it may make one think that a great deal more of a chordee is present than actually exists. Occasionally in some severe forms of hypospadias a very much underdeveloped or diminutive phallus is found. EMBRYOLOGY
The external genitalia arise from a single or homologous anlage which appears about the sixth week of fetal life as an undifferentiated genital tubercle located midway between the attachment of the umbilical cord and the coccygeal tubercle. On the under surface of this rudimentary structure a shallow urethral groove is formed which is bordered on each side by parallel elevated urethral folds. Just lateral to these folds rounded prominences appear which represent the labioscrotal swellings. During the tenth to fourteenth week of gestation these structures begin to be differentiated in a pattern that eventually will develop into either the male or female organ. The masculinization of the undifferentiative external genitalia consists '" Assistant Clinical Professor of Surgery, Harvard Medical School; Surgeon, Children's Medical Center; Senior Associate Surgeon (Plastic), Peter Bent Brigham Hospital, Boston. ** Clinical Associate in Surgery, Harvard Medical School; Surgeon, Children's Medical Center, Boston. t Former Resident in Surgery, Children's Medical Center, Boston.
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of a marked elongation and downward growth of the tubercle until it assumes the shape of the male penis capped on its tip by the glans. As this is happening the parallel folds on either side of the ventral urethral groove unite to form a penile urethra that extends from the base of the penis to the tip of the glans. If there is an arrest in the normal development, the fusion of these urethral folds may be delayed so that the opening may occur at any point from the coronal to the perineal position. In addition to the failure of development of the normal urethra, there is also an arrest in the formation of the corpus spongiosum. As a result, the corpus spongiosum is abnormally shortened and fibrotic, which in turn causes the penis to be bound down to the perineum to some degree. METHODS OF MANAGEMENT
Numerous operative procedures have been proposed and employed for the correction of hypospadias. Noone procedure has been found to be ideal in every case or for every surgeon. An excellent review of the historical background and outline of the methods used in the repair of hypospadias has been given by Young and Benjamin. l Smith and Blackfield2 have also prepared an excellent critique on the repair of hypospadias. It is not the purpose of this paper to review in detail all of the operative procedures that have been recommended in the past for the correction of this deformity. However, most previous reports outline repairs that fall into two main categories: (a) those that employ local flaps of the penile skin to form a new urethral tube (Ombredanne,3 Bucknall,4 Duplay,S and Brown 6), and (b) those that resort to free grafts that are buried in the penis to form the new channel (N ove-Josserand, 7 McIndoe,8 Havens 9 ). Ombredanne3 fashioned a urethral extension by folding a scrotal flap forward and upward to unite with an isolated strip of skin on the ventral surface of the penis. The denuded surface left by the construction of these flaps was covered by scrotal and penile skin that had been undermined and drawn together over the defect. A wide patulous urethra usually was created that was prone to calculus formation around hairs which grew in the newly created tube. With the penis in a flaccid state the urethral meatus was often so wide that accurate control of the urinary stream was impossible. In erection the patient's penis tended to extend far beyond the constructed meatus so that it was no longer near. enough to the tip to make insemination of the female possible. This type of operation was carried out in 13 cases at the Children's Medical Center and was then discarded because the results were not satisfactory to either the patient or the surgeon. Buckna1l4 devised another procedure somewhat similar in principle to the Ombredanne repair, in which the penis was "hinged" downward at
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the level of the urethral opening while a penile and a perineal flap were sewn together to form a new urinary channel. The penis was detached from the perineum at a second operation and the denuded areas covered by skin from adjacent areas.' Because of the long suture lines, healing was not always as perfect as one would wish, so that fistulas occurred frequently. At puberty the growth of hair on the portion of the urethra obtained from the scrotal or perineal flap resulted in irritation and calculus formation in the new urethra. The Thiersch-Duplay procedure as modified by Cecipa consisted:of creating a ventral flap of tissue between two parallel incisions that extended from the ectopic meatus to the tip of the glans. This strip was undermined on each side so that the edges could be brought over a catheter and sewn together to form a new skin-lined channel. The remaining penile skin was undermined extensively and brought together over the new skin tube. Because of the wide undermining it was difficult to obtain complete hemostasis. Hematomas were common, as were fistulas along the long superimposed suture lines. The results in some cases were excellent but in others numerous complications occurred. More recently Browne 6 has developed an operation based somewhat on the principles of the Thiersch-Duplay method. Two parallel incisions isolate a strip of ventral skin from the meatus to the corona of the penis. The lateral margins of these two incisions are widely undermined and brought together over this strip of skin. This imbedded strip then produces a new urinary channel by growing epithelium from each edge until union is complete in the form of a tube. The chief advantage of this method is that it may be performed at an early age thereby eliminating any psychological embarrassment from this abnormality. It would seem well indicated for those cases with midshaft openings. For those severe cases in which the opening is in the penoscrotal or perineal region, again hair may form in the new urethra if hair-bearing skin has been buried under the penile flaps. In the severe case an excessive amount of deep scarring is present due to the tubulating of the original skin strip. It is believed that this may seriously limit extension of the penis in erection. Excellent early results have been reported from many clinics in which this method of repair has been followed. The construction of a penile urethra by means of free grafts was first described in 1897 by Nove-Josserand. 7 So many complications in the nature of incomplete "takes" of the graft, strictures and fistulas made this method fall into disrepute until it was revised and modified by McIndoe 8 , 11 in 1937 and Havens4 in 1949. This "free graft" method of repair as reported previously by Gross12 has been found to be most satisfactory and currently is being employed at the Children's Medical Center of Boston.
,
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Donald W. MacCollum, Luther A. Longino, I. A. Meeker CLINICAL MATERIAL
In our series of 443 patients with hypospadias the following positions of the urethral meatus have been observed: coronal, 263; penile midshaft, 47; penoscrotal or perineal, 133. When the urinary opening is at the level of the corona and is of adequate diameter, there is seldom any need for operative correction. In this type of deformity the shaft of the penis is usually straight. Micturition in a standing position can be carried out without difficulty. Intercourse with subsequent fertilization also should be accomplished quite normally. Occasionally this urethral opening may be pinpoint in size. If this is found, early dilatation or meatotomy is done to prevent the occurrence of chronic urinary obstruction. In all cases with inadequate urinary openings and in all cases with the more severe types of hypospadias, intravenous pyelography is carried out. Chronic obstruction is rarely demonstrated; associated anomalies of the upper urinary tract are rarely found. Even though other abnormalities of the urinary tract may be infrequent, associated anomalies of the reproductive tract may be many and varied. If anorchism or cryptorchidism is found to occur in conjunction with hypospadias, immediately the sex of the individual is under suspicion. A careful rectal examination will be helpful in ruling out the presence or absence of a uterus. However, this determination may not be definitive in a newborn baby and even may be equivocal in the first few years of life. Examination of the urethra by means of injection of radiopaque substances may also be helpful. Instead of a normal urethra a vaginal pouch may be shown to open directly into a dilated urogenital sinus. Collection of urine for the determination of the 17-ketosteroids is also done to rule out an adrenogenital syndrome in which the clitoris of the female patient is enlarged to resemble the hypospadiad penis of the male. If the rectal examination is negative, if the x-rays of the urethra are normal, and if the excretion of the 17-ketosteroids are also found to be normal one must still question the true gender of the individual. It is in the latter case that an exploratory laparotomy is indicated. In addition to inspection of the pelvic organs, the gonads should be biopsied, even though grossly they may resemble either testes or ovaries or both (testicular and ovarian structures in the same organ; e.g., ovo-testes). In our series of 133 patients with severe hypospadias we have performed abdominal explorations on 18. On these 18, eight were found to . have female internal genitalia and three more were found to be true hermaphrodites. It is obvious that an abdominal exploration should be made as soon after birth as it is possible to perform it without danger to the individual. To delay these sex determinations until early childhood or later, may impose a heavy psychological burden and an almost superhuman readjustment upon both the parents and the child.
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TREATMENT
In order to treat a hypospadias patient satisfactorily, it is necessary to create a penis that will function properly both for micturition and for procreation. In addition, it is necessary that both the patient and his parents be handled in such a fashion that no abnormal psychological traits develop. It is our opinion that parental pressure, exerted to avoid embarrassment for the child when he reaches school age, has forced many surgeons to attempt correction of this deformity at too early an age. Many of these early operations have not only been unsuccessful but frequently they have permanently mutilated the organ to such an extent that an adequate repair at a later age becomes impossible. It is felt in the Children's Medical Center that if proper and continuous individualized attention is given to such a patient that almost any unpleasant psychological reaction to this deformity can be avoided. This requires the establishment of a close relationship between not only the parents and the surgeon but most particularly between the patient and his doctor. The correction of the mild hypospadias deformity in the patient who has no appreciable chordee has been eliminated entirely at the Children's Medical Center. If the penis is of normal size, the movement of the urethral meatus to the tip of the penis has not been found to be necessary for either urination or insemination. In this mild type of deformity it might be wise to excise the hooded foreskin for both sanitary and cosmetic reasons. In the more severe hypospadias deformities, a series of operations has been found to produce very satisfactory results. In all cases a circumcision of the hooded prepuce is to be avoided. It may be necessary to transpose this redundant tissue to fill in the defect left on the ventral surface of the penis at the time of the correction of the chordee. This correction of the chordee constitutes the first of a three stage procedure. First Stage (Fig. 507) The child is admitted to the hospital when between nine and ten years of age for this first operation. In those cases in which the chordee is severe an earlier operation is occasionally carried out and a partial straightening accomplished. In the latter case a second chordee correction is carried out at the ten year age level and further lengthening of the ventral surface obtained. A Foley catheter is first placed in the bladder so as to divert the urinary flow during the early healing stage. An inverted "V"-shaped incision is then made just distal to the urethral opening. This incision is developed so that the tight fibrous midline cords of tissue are 61xposed. These cords, along with a narrow strip of the foreshortened ventral skin, are then excised from this point to the level of the normal meatal opening. The
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Donald W. MacCollum, Luther A. Longino, I. A. Meeker
Fig. 507. Correction of chordee. a, Diagram of chordee with urethral orifice at penoscrotal junction. b, Penis sutured to anterior abdominal wall. Catheter in proximal urethra for constant drainage. c, d, Incisions for removal of fibrous bands and tight ventral midline contractures. e, Lateral incisions for relaxation of the ventral surface of the penis. f, g, h, Method of suturing to obtain the greatest possible length to the under surface of the penis. (Reprinted from R. E. Gross: Surgery of Infancy and Childhood, W. B. Saunders Co., 1953.)
corpus spongiosum is largely absent and, if present at all, is represented by a group of tight fibrotic bands. All of these firm constricting elements are removed. Mter all the deep fibrous tissue that caused the ventral bowing has been removed, cross incisions are made at different levels in the ventral skin so as to produce either a single Z-plasty flap, or a series
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of them in the severest cases. These flaps are interposed and sewn in their new positions with both mattress and single sutures of 5-0 plain catgut. It is important that no tension whatever exists on this suture line when the flaps are united. To produce sufficient relaxation it may be necessary to undermine the skin quite extensively up over the dorsum of the penis. It is at this point that the redundant skin of the prepuce becomes of importance and allows a shift in the skin coverings in such a fashion that all tension will be avoided on the ventral suture line. This single or multiple Z type of closure has the tendency to prevent any subsequent scar contracture which one is apt to get if there has been a straight line closure. A heavy gauze dressing is applied which is held in position under mild pressure with strips of elastoplast. Because of the extensive undermining during this first stage, bleeding is sometimes difficult to control. Hot packs, multiple sutures and pressure dressings are always necessary. If hemostasis is not absolute, hematomas will form beneath the flaps and lead to either rupture of the suture line or deep scarring, both of which will produce some tightening of the ventral surface and defeat the whole purpose of the first stage. Second Stage (Fig. 508)
This stage consists of creating a new penile urethra by means of a free, inlay, split thickness graft. So as to be certain of making a urethra of adequate length, this second stage is usually delayed until the penis reaches more adult proportions (14 to 16 years of age). The four to six year interval between the first and second stage is sometimes difficult for the boy psychologically, because it is necessary for most patients to sit down each time that they urinate. Usually a detailed and careful explanation of the entire problem, emphasizing particularly the goal to be obtained, is sufficient to avoid permanent psychological damage. Again a Foley catheter is placed in the bladder and again an inverted "V"-shaped incision is made just distal to the urethral opening. Through this incision a large metal trocar is passed up through the length of the penis to emerge at the position where the normal meatus should be. The tip and handle of the trocar are then unscrewed, leaving the hollow metal shaft of the trocar imbedded in the penis. A thin razor graft is next taken from the thigh and is rolled, raw side out, around a No. 16 French catheter. Each end of the graft is then sewn to the catheter so that it will not be displaced or wrinkled as the tube is being drawn into the metal trocar left in the penis. After the catheter carrying the graft has been placed inside the trocar shaft, the metal tube is removed leaving the catheter bearing the graft in the position of the new urethra. The graft is tacked to the glans with interrupted sutures of 5-0 plain catgut and a small safety-pin put through the catheter at each end so as to orevent displacement with movement. A firm compression dressing which
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Fig. 508. a, b, c, Method of tunneling the ventral surface of the penis to receive graft. d, e, Split thickness graft wrapped around and tied to No. 16 rubber catheter. " Dismantling of trocar for insertion of graft. (Continued on facing page.)
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Fig. 508 (Continued). g, h, i, Placement of graft through hollow barrel of trocar. k, Split thickness graft in position to make a new channel in the penis. It is anchored to the glans only. (Reprinted from Gross: Surgery of Infancy and Childhood, W. B. Saunders Co., 1953).
i, Removal of metal cylinder with graft remaining in place.
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holds the penis up against the abdominal wall is then applied. After a week, the Foley catheter in the bladder is removed. At this same time the urethral catheter is changed and a new tube of the same diameter reinserted. After a few more days the patient is shown how to
Fig. 509. a-f. Establishment of perineal urethrotomy. (Cemtinued em facing page.)
carry out the daily changing of this tube which now acts as a urethral splint and prevents any contracture of the lumen. During the next six months, this strut is worn for decreasing periods of time. At the end of the:six month period all tendency for contracture of the graft should have passed so the patient is now ready for the final stage of the procedure.
k
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Third Stage (Fig. 509)
As in the previous two stages, we find it advisable to divert the urinary flow so that there will be no contamination of the operative site during the healing period. A perineal urethrotomy is first performed. If there are any objections to placing the urethrotomy in the perineal region, a
Fig. 509 (Continued). g-l, Method of anastomosis of the skin graft channel to the proximal urethra. (Reprinted from Gross: Surgery of Infancy and Childhood, W. B. Saunders Co., 1953).
suprapubic cystotomy may be carried out. After this has been accomplished, a catheter is inserted through the newly constructed urethra and into the normal urethra so that it will bridge the gap between the two. An oval incision is made around this site and the innermost flaps undermined so they may be closed over the bridging catheter. This closure is
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accomplished by a continuous Connell suture of 5-0 plain catgut. A reinforcing layer of tissue is brought over this first suture line and is held here by interrupted sutures of 5-0 arterial silk. The lateral edges of the oval incision are then undermined and the closure carried out in two
Fig. 510. A, Photograph showing chordee with arrow pointing to urethral orifice at penoscrotal junction. B, After correction of chordee with lengthening of the penis. C, Graft in place with a No. 16 rubber catheter through the newly formed skin-lined channel. D, Six months after completion of Stage III showing the patient voiding through the newly created urethra.
layers using either 5-0 plain catgut or 5-0 chromic catgut. At the end of the procedure the urethral catheter is removed and a light compression dressing is applied. The perineal urethrotomy or suprapubic cystotomy catheter is removed on the tenth postoperative day at which time the
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patient now begins to use his new urethra. The urethrotomy or cystotomy openings will close spontaneously within 48 hours. RESULTS AND COMPLICATIONS
To date, 68 patients have been operated upon for the relief of the chordee with highly satisfactory results. Three of these patients developed hematomas following the release of the chordee and required a second operation for the evacuation of the clot and resuturing of the flaps. None of these three developed unusual scarring subsequently, so there was no permanent damage to the penis. In 33 of these 68 patients, all three stages described above for the creation of a new urethra have been completed. Two of these 33 patients developed fistulas after the third stage and each required another operative procedure to close the fistulas. One patient developed a stricture at the junction of the skin tube and the natural urethra. Repeated dilatations were not successful in relieving it, so an exploratory operation was then done to remove the scar tissue and reanastomose the ends. This was successful in every respect. In each of these four cases the complications were corrected easily so that the end result in all 33 cases was completely satisfactory. SUMMARY
Four hundred and forty-three patients with hypospadias have been seen in the Plastic Surgery Clinic of the Children's Medical Center of Boston. A great many of these patients had only mild deformities which required no surgical correction. For those patients with severe hypospadias deformities, a three stage corrective procedure has been outlined. The first stage consists in release of the chordee sometime before ten years of age. The second stage is performed between 14 and 16 years of age. By means of a free inlay graft this stage embodies the creation of a new penile urethra of sufficient diameter and length for an adult sized penis. The third stage is performed six months to a year after stage two and consists in uniting the new urethra with the natural urethra. The principal disadvantage to this method of hypospadias repair is the delay encountered before the three stages are completed. Many observers believe that the psychological damage from this delay is excessive. In our own clinic we have observed that individualized attention and establishment of a close doctor-patient relationship, plus a thorough understanding of the problem by the patient, has done much to minimize this psychological trauma. The advantages of this method of repair are many. The three stages of the repair are simple and require no complicated reconstructive steps. Complications following each stage have been infrequent and when they did occur were corrected by minor secondary operative procedures. To
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date, every patient whose repair has been completed now has a penile organ that is satisfactory both cosmetically and functionally. REFERENCES 1. Young, F. and Benjamin, J. A.: Preschool Age Repair of Hypospadias with Free Inlay Skin Graft. Surgery 26: 384-404, 1949. 2. Smith, D. R. and Blackfield, H. M.: A Critique on the Repair of Hypospadias. . Surgery 31: 885, 1952. 3. Ombredanne: Hypospadias Penien Chez. I'enfant. Bull. et memo Soc. de Chu. de Paris 37: 1076-1078, 1911. 4. BucknaII, R. T. H.: A New Operation for Penile Hypospadias. Lancet 2: 887890,1907. 5. Duplay, Simon: Nouvelle methode operatoire applicable au traitement de l'hypospadias perin eo-scrotal. Arch. gen. de me.d. 23(VIe ser): 657-682, 1874. 6. Browne, Denis: An Operation for Hypospadias. Proc. Roy. Soc. Med. 42: 466468, 1949. 7. Nove-Josserand, Gabriel: Traitement de l'hypospadias, Nouvelle methode. Lyon med. 10: 184, 1907. 8. McIndoe, A. H.: Deformities of the Male Urethra. Brit. J. Plastic Surg. 1: 29-47, 1948. 9. Havens, F. Z. and Black, A. S.: The Treatment of Hypospadias. J. Urol. 61: 1053-1064, 1949. 10. Cecil, A. B.: Surgery of Hypospadias and Epispadias in the Male. J. Urol. 27: 507-537, 1932. 11. McIndoe, A. H.: The Treatment of Hypospadias. Am. J. Surg. 38: 176-185,1937. 12. Gross, R. E.: Surgery of Infancy and Childhood. Philadelphia, W. B. Saunders Co., 1953. 300 Longwood Avenue Boston, Massachusetts