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Ectopic scrotum—A case report
ECTOPIC S C R O T U M - - A C A S E R E P O R T By P. S. BAJM, M.S., F.R.C.S.Ed., F.R.C.S., and B. N. BAILEY,F.R.C.S.
Stoke Mandeville Hospital, Aylesbury, Bucks. THE scrotum is relatively immune to congenital anomalies. Bifid scrotum and transposition of penis and scrotum are the most common of these though only 13 cases have been reported in the literature (Flanagan et al., 1961 ; Remzi, 1966). When malformations occur they are often assgciated with other congenital conditions of a more serious nature (Daut and Daut, 1949). A syndrome consisting of genital anomalies associated with cleft lip and palate, popliteal pterygium and digital anomalies has been described (Gorlin and Pindborg, I964 ; Hecht and Jarvinen, 1967) but none of the cases quoted had an ectopic scrotum. Laterally ectopic scrotum has been described only twice. A case reported by Adair and Lewis (196o) had a unilateral ectopic scrotum on the anterior abdominal wall. This communicated directly with the external inguinal ring and contained an ectopic testis. This patient also had an accessory glans lying on the dorsum of the main shaft of penis. The other case reported (Williams, 1963) had a bilateral ectopic half scrotum containing a testis on the medial aspect of each thigh. The penis was in the middle line with the median raphe rotated 9°o to the patient's left. This patient had in addition a complete cleft of the lip and palate with maldevelopment of the maxilla and congenital web contracture behind the right knee. We wish to report an almost similar case with a unilateral ectopic scrotum and multiple associated developmental abnormalities.
Case Report.--M. B., born on I3th March I958, was admitted to Stoke Mandeville Hospital on the same day. The child (Fig. I) had a unilateral ectopic scrotum. The right half scrotum was attached to the medial side of the right thigh, with normal skin between it and the left half. The latter was normal but the penis which was in the middle line had a median raphe rotated 9°0 to the left. In addition the baby had trismus due to bands extending from upper to lower jaw, Group III cleft of lip and palate, minor interpalpebral bands and flexion webs extending from the adductor regions of the thighs to the heels on both sides with deformities of the feet. There was no history of any congenital anomaly in the family and the mother had an uncomplicated pregnancy, labour and delivery. No drugs other than iron, calcium and Supplementary vitamin C had been taken during pregnancy. Over a period of nine years he had been treated for these deformities, but it was only in I967 that we decided to correct the scrota1 anomaly. At this time the right half scrotum was empty ahhough it was better developed than the left half which contained a testis. The right testis was inguinal with an associated indirect inguinal hernia. The typical appearance of the ectopic scrotum left no doubt as to its morphological origin. Orchidopexy was performed, the testis being brought down into the scrotal pouch in the right thigh and anchored to the deep fascia there. The hernia was repaired at the same time. (Biopsy of the ectopic scrotal tissue confirmed normal scrotal skin with dartos muscle.) Post-operatively the right thigh which had been flexed to allow migration of the testis was allowed to straighten under the child's own control Six months later the scrotum with the contained testis and spermatic cord were transposed to the normal position by a simple Z-plasty, similar to that described by WiUiams (1963). There were no post-operative difficulties and the right half scrotum is more prominent than tile normal side (Fig. 2). Chromosome studies carried out by Dr Martin Bobrow (M.R.C. Population Genetic Research Unit, Oxford) at this stage showed a normal male pattern. 87
88
BRITISH JOURNAL OF PLASTIC SURGERY
FIG. I FIG. 2 Fig. I .---Photograph of the child at birth showing ectopic scrotum. Note the position of median raphe of the penis which is rotated to the left. Fig. 2 . - - T h e same child after scrotum had been transposed to its normal position.
DISCUSSION
Development of the scrotum is well described by Lowsley and Kirwin(I956). They state--" When the embryo is about 2I mm. long, a pair of rounded lateral ridges--the labioscrotal swellings, or outer genital folds--ar~ visible one on each side of the base of phallus from which they are separated by a Y-shaped grove. During the 38 to 45 ram. stage, the phallus elongates and the primitive urogenital opening is removed farther and farther from the anus by the fusing of the margins of the urethral folds in the midline. The fused margins of these folds constitute the perineal and penile raphe. During the elongation of the phallus, the labioscrotal swellings gradually migrate downwards and assume their permanent position between the base of the penis and perineal body and now become scrotal swellings." Failure of fusion is a common congenital anomaly and while there are many conflicting theories of its cause, no great effort of imagination is needed to visualise the immediate mechanics of the error. Failure of migration is a similar situation and, as with failure of fusion, one is inclined to marvel at the frequency with which this complex process proceeds to a satisfactory conclusion rather than to dwell on the few developmental errors. In this case we are faced with an abnormal migration, the final position of the half scrotum lying far from any position through which it should have passed in its normal development. We are not able to offer any explanation based on the embryology of this area, but it is significant that the only other recorded case of incorrect migration (Williams) had very similar associated defects, i.e. cleft of lip and palate, rotated median raphe, and popliteal pterygium. A major intrauterine catastrophe affecting all these organs at an early stage at the time of their development might be a reasonable conjecture. SUMMARY A case of unilateral ectopic scrotum present on the medial side of the right thigh is reported. Its association with Group III cleft of lip and palate and web contracture of lower limbs is stressed.
ECTOPIC S C R O T U M - - A CASE REPORT REFERENCES ADAIR, E. L. and LEwxs, E. L. (I96O). ~. Urol. 84, xI5. DAUT, W. W. and DAUT, R. V. (I949). J. Iowa St. meal. Soc. 39, I94. FLANAG^N, M. J., McDoNALD, J. H. and Kx~.F~R,J. H. (I96I). J . Urol. 86, 273. GOl~IN, R. J. and PmDBORG, J. J. (x964). " Syndromes of Head and Neck." New York : McGraw-Hill. HECHT, F. and JARVIN~N,J. M. (x967)..7. Pediat. 7o, 927. LOWSLEY, O. S. and KmWIN, T. J. (I956). " Clinical Urology." Baltimore : Williams & Wilkins. RE/vml, D. (x966)..7. Urol. 95, 555. WmrIAMS, D. W. (I963). ~. Urol. 89, 860.
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Stoke Mandeville Hospital, Aylesbury, Bucks. THE scrotum is relatively immune to congenital anomalies. Bifid scrotum and transposition of penis and scrotum are the most common of these though only 13 cases have been reported in the literature (Flanagan et al., 1961 ; Remzi, 1966). When malformations occur they are often assgciated with other congenital conditions of a more serious nature (Daut and Daut, 1949). A syndrome consisting of genital anomalies associated with cleft lip and palate, popliteal pterygium and digital anomalies has been described (Gorlin and Pindborg, I964 ; Hecht and Jarvinen, 1967) but none of the cases quoted had an ectopic scrotum. Laterally ectopic scrotum has been described only twice. A case reported by Adair and Lewis (196o) had a unilateral ectopic scrotum on the anterior abdominal wall. This communicated directly with the external inguinal ring and contained an ectopic testis. This patient also had an accessory glans lying on the dorsum of the main shaft of penis. The other case reported (Williams, 1963) had a bilateral ectopic half scrotum containing a testis on the medial aspect of each thigh. The penis was in the middle line with the median raphe rotated 9°o to the patient's left. This patient had in addition a complete cleft of the lip and palate with maldevelopment of the maxilla and congenital web contracture behind the right knee. We wish to report an almost similar case with a unilateral ectopic scrotum and multiple associated developmental abnormalities.
Case Report.--M. B., born on I3th March I958, was admitted to Stoke Mandeville Hospital on the same day. The child (Fig. I) had a unilateral ectopic scrotum. The right half scrotum was attached to the medial side of the right thigh, with normal skin between it and the left half. The latter was normal but the penis which was in the middle line had a median raphe rotated 9°0 to the left. In addition the baby had trismus due to bands extending from upper to lower jaw, Group III cleft of lip and palate, minor interpalpebral bands and flexion webs extending from the adductor regions of the thighs to the heels on both sides with deformities of the feet. There was no history of any congenital anomaly in the family and the mother had an uncomplicated pregnancy, labour and delivery. No drugs other than iron, calcium and Supplementary vitamin C had been taken during pregnancy. Over a period of nine years he had been treated for these deformities, but it was only in I967 that we decided to correct the scrota1 anomaly. At this time the right half scrotum was empty ahhough it was better developed than the left half which contained a testis. The right testis was inguinal with an associated indirect inguinal hernia. The typical appearance of the ectopic scrotum left no doubt as to its morphological origin. Orchidopexy was performed, the testis being brought down into the scrotal pouch in the right thigh and anchored to the deep fascia there. The hernia was repaired at the same time. (Biopsy of the ectopic scrotal tissue confirmed normal scrotal skin with dartos muscle.) Post-operatively the right thigh which had been flexed to allow migration of the testis was allowed to straighten under the child's own control Six months later the scrotum with the contained testis and spermatic cord were transposed to the normal position by a simple Z-plasty, similar to that described by WiUiams (1963). There were no post-operative difficulties and the right half scrotum is more prominent than tile normal side (Fig. 2). Chromosome studies carried out by Dr Martin Bobrow (M.R.C. Population Genetic Research Unit, Oxford) at this stage showed a normal male pattern. 87
88
BRITISH JOURNAL OF PLASTIC SURGERY
FIG. I FIG. 2 Fig. I .---Photograph of the child at birth showing ectopic scrotum. Note the position of median raphe of the penis which is rotated to the left. Fig. 2 . - - T h e same child after scrotum had been transposed to its normal position.
DISCUSSION
Development of the scrotum is well described by Lowsley and Kirwin(I956). They state--" When the embryo is about 2I mm. long, a pair of rounded lateral ridges--the labioscrotal swellings, or outer genital folds--ar~ visible one on each side of the base of phallus from which they are separated by a Y-shaped grove. During the 38 to 45 ram. stage, the phallus elongates and the primitive urogenital opening is removed farther and farther from the anus by the fusing of the margins of the urethral folds in the midline. The fused margins of these folds constitute the perineal and penile raphe. During the elongation of the phallus, the labioscrotal swellings gradually migrate downwards and assume their permanent position between the base of the penis and perineal body and now become scrotal swellings." Failure of fusion is a common congenital anomaly and while there are many conflicting theories of its cause, no great effort of imagination is needed to visualise the immediate mechanics of the error. Failure of migration is a similar situation and, as with failure of fusion, one is inclined to marvel at the frequency with which this complex process proceeds to a satisfactory conclusion rather than to dwell on the few developmental errors. In this case we are faced with an abnormal migration, the final position of the half scrotum lying far from any position through which it should have passed in its normal development. We are not able to offer any explanation based on the embryology of this area, but it is significant that the only other recorded case of incorrect migration (Williams) had very similar associated defects, i.e. cleft of lip and palate, rotated median raphe, and popliteal pterygium. A major intrauterine catastrophe affecting all these organs at an early stage at the time of their development might be a reasonable conjecture. SUMMARY A case of unilateral ectopic scrotum present on the medial side of the right thigh is reported. Its association with Group III cleft of lip and palate and web contracture of lower limbs is stressed.
ECTOPIC S C R O T U M - - A CASE REPORT REFERENCES ADAIR, E. L. and LEwxs, E. L. (I96O). ~. Urol. 84, xI5. DAUT, W. W. and DAUT, R. V. (I949). J. Iowa St. meal. Soc. 39, I94. FLANAG^N, M. J., McDoNALD, J. H. and Kx~.F~R,J. H. (I96I). J . Urol. 86, 273. GOl~IN, R. J. and PmDBORG, J. J. (x964). " Syndromes of Head and Neck." New York : McGraw-Hill. HECHT, F. and JARVIN~N,J. M. (x967)..7. Pediat. 7o, 927. LOWSLEY, O. S. and KmWIN, T. J. (I956). " Clinical Urology." Baltimore : Williams & Wilkins. RE/vml, D. (x966)..7. Urol. 95, 555. WmrIAMS, D. W. (I963). ~. Urol. 89, 860.
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