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Double bladder and related anomalies
DOUBLE
BLADDER
AND
RELATED
ANOMALIES
By ALEXANDERH. ELLENBERG,M.D. 1 and LAURENCEM. LINKER,M.D.
From the Department of Plastic Surgery, University of California School of Medicine, San Francisco, California, and the Department of Pediatric Surgery, Arizona State Crippled Children's Hospital, Phoenix, Arizona CONGENITAL defects simultaneously involving the genito-urinary, skeletal and musculocutaneous systems are rare. Recently a patient was seen who had congenital : (I) ectopic and bifid scrotum ; (2) exstrophy o f the bladder ; (3) bifid ectopic bladder ; (4) atrophic left kidney ; (5) congenital flexion contracture extending from left hip to Achilles t e n d o n ; (6) calcaneal valgus deformity o f the left f o o t ; (7) diastasis o f the symphysis p u b i s ; (8) right inguinal hernia and hydrocele ; and (9) abnormalities of the lumbar vertebrm, sacrum and pelvis. This combination of anomalies has not been previously reported. CASE REPORT A 4-day-old baby boy was admitted to the Arizona State Crippled Children's Hospital on 7th June 1963, because of multiple congenital defects. The prenatal course and birth had been uneventful. The birth was attended by a midwife and the baby weighed 7 lb. After birth a small amount of clear fluid constantly dripped from a midline sinus tract just below the umbilicus. The scrotum was bifid and the left leg was shorter than the right. The baby was vigorous and had good colour. The head, eyes, nose, throat, heart and lungs were normal. Mental development appeared to be normal. A funnel-shaped depression was present in the midline midway between the pubic symphysis and umbilicus. The right scrotum appeared to be in the normal position. The left scrotum was ectopic and was located to the left along Poupart's ligament (Fig. 1). There was no connection between the left and the right scrotum. Each scrotum contained a testicle that was normal on palpation. The symphysis pubis was widely separated. A flexion contracture was present behind the left knee and hip. This was associated with a tight band extending from the posterior ischial tuberosity to the lateral aspect of the lower leg, and thence to the os calcis. A calcaneal valgus deformity was present on the left foot. Urinalysis was normal except for 3 to 4 red, white and epithelial cells per high power field. The haemogram was within normal limits for the patient's age. Blood urea nitrogen was 9 mg./'ioo nal. (normal 1o to 20). Roentgenograms showed a wide diastasis of the symphysis pubis, valgus deformity of the left foot, gross malformations of the lower lumbar vertebrae and sacrum (L-4 and L-5), and congenital absence of portions of L-4 and L- 5. Congenital subluxation of the proximal left femur was associated with congenital malformation of the left innominate bone and acetabulum. A roentgenogram of the chest was normal. An intravenous pyelogram showed a slightly enlarged right collecting system and clubbing of the calyces, consistent with pyelonephritis. The left kidney could not be visualised. On a three-hour film the urinary bladder was dilated. Shortly after admission, orthopaedic treatment of the flexion contracture of the left knee and the calcaneal valgus deformity was instituted by long-leg casting. Two months of repeated cast applications failed to correct the calcaneal valgus deformity. Operation, performed on I9th August 1963, included bilateral iliac osteotomies (to correct the symphysis pubis diastasis), and release of the contracture band of the right leg. The fascial band extended from the ischial region to the heel, inserting into the Achilles tendon. It was somewhat tendinous and superficial to the musculature of the posterior thigh, and did not represent a normal posterior 1 Present Address : I333 Meridian Avenue, San Jose, California 95125. 338
DOUBLE BLADDER AND RELATED ANOMALIES
339
leg structure. Two muscles by-passed the knee and had a tendinous insertion into the fascial band near the heel. The tendon-like contracting band was incised, together with the muscles inserting into the band. The deep fascia of the thigh was divided. These manoeuvres permitted considerable extension of the knee, but there was no appreciable change in the calcaneal valgus deformity. At a second operation on 26th August I963, the pelvis was quite mobile and the pubic symphysis was easily brought together in the midline, demonstrating the effect of the previous bilateral iliac osteotomies. The anterior sinus tract, which was still draining clear fluid, was traced into a left ectopic urinary bladder measuring approximately 5 by 7 cm. The bladder was normal except for chronic ~cdema of the mucosa, probably due to infection. There was no connection between this bladder and a separate but distinctly larger urinary bladder on the right, nor was there an external opening suggesting a urethra. The only exit of this ectopic bladder was into the anterior abdominal wall through the sinus opening (Fig. 2). The peritoneum was opened and no abnormal intra-abdominal structures were noted, with the exception of the hypoplastic left kidney which measured approximately 2 by 2 cm. Questionable dilatation or hypertrophy of the right ureter was present. A barely perceptible left ureter extended to the ectopic bladder. The ectopic scrotum in the left groin contained a testis witha spermatic cord adequate in length. The ectopic bladder was excised, together with its sinus tract, to the anterior abdominal wall. The small hypoplastic, nonFIG. I functioning left kidney was left in place since Pre-operative appearance of lower abdomen. its removal would have required extension of the original incision or a second incision. The ectopic scrotum in the left groin was excised and the testis was repositioned in the single, normally-placed scrotum after recanalising the inguinal canal (Fig. 3). A hip spica was applied to keep the pubic symphysis approximated. A post-operative urinary tract infection was treated with antibiotics. The patient also had post-operative vomiting and bowel distention. An upper gastro-intestinal series showed no signs of intestinal obstruction and he was treated symptomatically. Orthopmdic treatment was continued and a wedge cast was used to maintain knee extension and to attempt correction of the left foot. Physical therapy was also used. On 9th January I964, the patient developed a right hydrocele and communicating hernia. This was not surprising in view of the lax abdominal musculature, which suggested agenesis. The orthopmdic problem now consisted primarily of a persistent calcaneal valgus deformity as well as developmental shortening of the entire left lower extremity. A Steinmann pin was placed through the os calcis in an attempt to improve the foot alignment, although the left lower extremity would probably become progressively shorter and eventually a further procedure would be required. Urinary tract function was excellent and the repositioned left testis remained in a good position and was of normal size. Before additional corrective surgery could be performed, the patient developed varicella. When this subsided he was discharged to the care of his parents on ISt April I964, until he could be readmitted for further surgical therapy. However, the parents moved away and attempts to contact them to arrange for follow-up visits and treatment were unsuccessful. The patient has not been seen since.
340
B R I T I S H JOURNAL
OF PLASTIC SURGERY
FIG. 2
FIG. 3
Fig. 2.--Schematic representation of findings at operation. Fig. 3.--Post-operative appearance.
SUMMARY A newborn white male had multiple congenital anomalies involving the genitourinary system (exstrophic bladder, bifid and ectopic bladder, bifid and ectopic scrotum) ; musculoskeletal system (calcaneal valgus deformity of the left foot, diastasis, symphysis pubis, deformity of sacrum and fourth and fifth lumbar vertebra:, deformed left innominate bone and acetabulum with congenital subluxation of femur) ; and musculocutaneous system (congenital flexion contracture of fascial band extending from the ischial tuberosity to the calcaneus, communicating hydrocele with right inguinal hernia secondary to lax hypoplastic abdominal musculature). The various surgical approaches included orthopa:dic, pa:diatric, plastic and urological surgery.
Submitted for publication, September I965.
BLADDER
AND
RELATED
ANOMALIES
By ALEXANDERH. ELLENBERG,M.D. 1 and LAURENCEM. LINKER,M.D.
From the Department of Plastic Surgery, University of California School of Medicine, San Francisco, California, and the Department of Pediatric Surgery, Arizona State Crippled Children's Hospital, Phoenix, Arizona CONGENITAL defects simultaneously involving the genito-urinary, skeletal and musculocutaneous systems are rare. Recently a patient was seen who had congenital : (I) ectopic and bifid scrotum ; (2) exstrophy o f the bladder ; (3) bifid ectopic bladder ; (4) atrophic left kidney ; (5) congenital flexion contracture extending from left hip to Achilles t e n d o n ; (6) calcaneal valgus deformity o f the left f o o t ; (7) diastasis o f the symphysis p u b i s ; (8) right inguinal hernia and hydrocele ; and (9) abnormalities of the lumbar vertebrm, sacrum and pelvis. This combination of anomalies has not been previously reported. CASE REPORT A 4-day-old baby boy was admitted to the Arizona State Crippled Children's Hospital on 7th June 1963, because of multiple congenital defects. The prenatal course and birth had been uneventful. The birth was attended by a midwife and the baby weighed 7 lb. After birth a small amount of clear fluid constantly dripped from a midline sinus tract just below the umbilicus. The scrotum was bifid and the left leg was shorter than the right. The baby was vigorous and had good colour. The head, eyes, nose, throat, heart and lungs were normal. Mental development appeared to be normal. A funnel-shaped depression was present in the midline midway between the pubic symphysis and umbilicus. The right scrotum appeared to be in the normal position. The left scrotum was ectopic and was located to the left along Poupart's ligament (Fig. 1). There was no connection between the left and the right scrotum. Each scrotum contained a testicle that was normal on palpation. The symphysis pubis was widely separated. A flexion contracture was present behind the left knee and hip. This was associated with a tight band extending from the posterior ischial tuberosity to the lateral aspect of the lower leg, and thence to the os calcis. A calcaneal valgus deformity was present on the left foot. Urinalysis was normal except for 3 to 4 red, white and epithelial cells per high power field. The haemogram was within normal limits for the patient's age. Blood urea nitrogen was 9 mg./'ioo nal. (normal 1o to 20). Roentgenograms showed a wide diastasis of the symphysis pubis, valgus deformity of the left foot, gross malformations of the lower lumbar vertebrae and sacrum (L-4 and L-5), and congenital absence of portions of L-4 and L- 5. Congenital subluxation of the proximal left femur was associated with congenital malformation of the left innominate bone and acetabulum. A roentgenogram of the chest was normal. An intravenous pyelogram showed a slightly enlarged right collecting system and clubbing of the calyces, consistent with pyelonephritis. The left kidney could not be visualised. On a three-hour film the urinary bladder was dilated. Shortly after admission, orthopaedic treatment of the flexion contracture of the left knee and the calcaneal valgus deformity was instituted by long-leg casting. Two months of repeated cast applications failed to correct the calcaneal valgus deformity. Operation, performed on I9th August 1963, included bilateral iliac osteotomies (to correct the symphysis pubis diastasis), and release of the contracture band of the right leg. The fascial band extended from the ischial region to the heel, inserting into the Achilles tendon. It was somewhat tendinous and superficial to the musculature of the posterior thigh, and did not represent a normal posterior 1 Present Address : I333 Meridian Avenue, San Jose, California 95125. 338
DOUBLE BLADDER AND RELATED ANOMALIES
339
leg structure. Two muscles by-passed the knee and had a tendinous insertion into the fascial band near the heel. The tendon-like contracting band was incised, together with the muscles inserting into the band. The deep fascia of the thigh was divided. These manoeuvres permitted considerable extension of the knee, but there was no appreciable change in the calcaneal valgus deformity. At a second operation on 26th August I963, the pelvis was quite mobile and the pubic symphysis was easily brought together in the midline, demonstrating the effect of the previous bilateral iliac osteotomies. The anterior sinus tract, which was still draining clear fluid, was traced into a left ectopic urinary bladder measuring approximately 5 by 7 cm. The bladder was normal except for chronic ~cdema of the mucosa, probably due to infection. There was no connection between this bladder and a separate but distinctly larger urinary bladder on the right, nor was there an external opening suggesting a urethra. The only exit of this ectopic bladder was into the anterior abdominal wall through the sinus opening (Fig. 2). The peritoneum was opened and no abnormal intra-abdominal structures were noted, with the exception of the hypoplastic left kidney which measured approximately 2 by 2 cm. Questionable dilatation or hypertrophy of the right ureter was present. A barely perceptible left ureter extended to the ectopic bladder. The ectopic scrotum in the left groin contained a testis witha spermatic cord adequate in length. The ectopic bladder was excised, together with its sinus tract, to the anterior abdominal wall. The small hypoplastic, nonFIG. I functioning left kidney was left in place since Pre-operative appearance of lower abdomen. its removal would have required extension of the original incision or a second incision. The ectopic scrotum in the left groin was excised and the testis was repositioned in the single, normally-placed scrotum after recanalising the inguinal canal (Fig. 3). A hip spica was applied to keep the pubic symphysis approximated. A post-operative urinary tract infection was treated with antibiotics. The patient also had post-operative vomiting and bowel distention. An upper gastro-intestinal series showed no signs of intestinal obstruction and he was treated symptomatically. Orthopmdic treatment was continued and a wedge cast was used to maintain knee extension and to attempt correction of the left foot. Physical therapy was also used. On 9th January I964, the patient developed a right hydrocele and communicating hernia. This was not surprising in view of the lax abdominal musculature, which suggested agenesis. The orthopmdic problem now consisted primarily of a persistent calcaneal valgus deformity as well as developmental shortening of the entire left lower extremity. A Steinmann pin was placed through the os calcis in an attempt to improve the foot alignment, although the left lower extremity would probably become progressively shorter and eventually a further procedure would be required. Urinary tract function was excellent and the repositioned left testis remained in a good position and was of normal size. Before additional corrective surgery could be performed, the patient developed varicella. When this subsided he was discharged to the care of his parents on ISt April I964, until he could be readmitted for further surgical therapy. However, the parents moved away and attempts to contact them to arrange for follow-up visits and treatment were unsuccessful. The patient has not been seen since.
340
B R I T I S H JOURNAL
OF PLASTIC SURGERY
FIG. 2
FIG. 3
Fig. 2.--Schematic representation of findings at operation. Fig. 3.--Post-operative appearance.
SUMMARY A newborn white male had multiple congenital anomalies involving the genitourinary system (exstrophic bladder, bifid and ectopic bladder, bifid and ectopic scrotum) ; musculoskeletal system (calcaneal valgus deformity of the left foot, diastasis, symphysis pubis, deformity of sacrum and fourth and fifth lumbar vertebra:, deformed left innominate bone and acetabulum with congenital subluxation of femur) ; and musculocutaneous system (congenital flexion contracture of fascial band extending from the ischial tuberosity to the calcaneus, communicating hydrocele with right inguinal hernia secondary to lax hypoplastic abdominal musculature). The various surgical approaches included orthopa:dic, pa:diatric, plastic and urological surgery.
Submitted for publication, September I965.