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Choledochocele: Case report, literature review and a proposed classification
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more frequently in patients with cystic fibrosis. They suggest that the dogma that appendicitis and cystic fibrosis is rare may be dangerous. The evidence for this is not conclusive and may lead to late diagnosis with increased morbidity in these patients.--J.D. Orr Segmental Aganglionosis of the Small Bowel--Myth or Reality? R.
Gonzalez-Vasquez and W.H. Heiss. Z Kinderchir 43:424-426, (December), 1985. Reports on zonal aganglionosis of the bowel are rare and the few cases reported concern colon and rectum. A case of segmental aganglionosis of the jejunum in a 2-month-old boy is reported. A narrow jejunal segment measuring 10 cm had to be resected. Histological examination revealed segmental aganglionosis of the myenteric plexus. The suggested causes of this rare zonal aganglionosis of the small bowel include (1) local damage of the plexus by hypoxemia, (2) primary abnormality in the gastrointestinal anlage, (3) intrauterine inflammation, and (4) double migration and development gradient of the neuroblast precursors. A defect in the microenvironment of the enteric neurons would result in failure of differentiation. This possible explanation would challenge the accepted theory of pathogenesis of colonic aganglionosis.--Thomas A. Angerpointner Reliability of Anorectal Manometry in the Diagnosis of Hirschsprung's Disease. N. Iwai, J. Yanagihara, K. Tokiwa, et al. Z
Kinderchir 43:405-407, (December), 1988. Anorectal manometry was performed in 79 patients, aged two days to 19 years, who were referred for diagnosis of constipation or symptoms of intestinal obstruction. Definitive diagnosis was obtained in 75 of the 79 patients (95%). Thus, anorectal manometry is a reliable tool in the diagnosis of Hirschsprung's disease. In the neonatal period, however, a definitive diagnosis was possible in only 17 of 21 neonates (81%). If intestinal obstruction can be managed conservatively, manometry should be repeated in neonates after 2 weeks if diagnosis cannot be definitely established by the first investigation. Additionally, barium enema and rectal suction biopsy should be performed if Hirschsprung's disease is suspected manometrically.--Thomas A. Angerpointner Posterior Sagittal Anorectoplasty for Reoperation in Children With Anoractal Malformations. A.J.L. Brain and E.M. Kiely. Br J
Surg 76:57-59, (January), 1989. The authors studied a consecutive series of 12 children who had previously undergone surgery for anorectal anomalies but whose fecal incontinence rendered them socially unacceptable. The majority had been born with a supralevator anoreetal anomaly with a fistula or a cloacal abnormality. Eight of the 12 children had already undergone unsuccessful attempts at secondary surgery. A further clinical and neurophysiological assessment was carried out preoperatively. This revealed anatomical problems, such as recurrent fistula, massive urethral diverticulum, absent vagina, and a missed urogenital sinus. The external sphincter was noted to be displaced from the anus in nine patients. Posterior sagittal anorectoplasty, with correction of the anatomical defects and precise reconstruction of the bowel within the sphincters, was carried out after the formation of a loop colostomy. No postoperative complications were observed. Contrast studies were performed to confirm healing of the suture lines before the stomas were closed. Follow up, from 4 to 46 months, revealed good fecal control in only two patients but with a significant improvement in two others. Although the rest remain incontinent, sensation was improved. The authors note that these results are disappointing and at variance with previously published reports. They suggest that the
INTERNATIONAL ABSTRACTS
posterior sagittal anorectoplasty used as a secondary procedure can correct the anatomical defects but may not improve fecal incontinence.--J.D. Orr Anal Bleeding Caused by Tubular Rectal Duplication With Ectopic Gastric Mucosa. C. Janneck. Z Kinderchir 43:353-354, (October),
1988. The author reports an extremely rare rectoreetal duplication, containing gastric mucosa with a fistula to the rectum in a 10year-old boy with myelodysplasia syndrome, anal stenosis, and a sacral defect. Persistent tarry stools prompted diagnosis and extirpation of the duplication. The combination of anorectal malformations, presacral mass, and sacrococcygeal defect may indicate a Currarino triad, an autosomal dominant hereditary syndrome that can be simply detected and identified by x-ray examination of the sacrum.-- Thomas A. Angerpointner ABDOMEN Choledochocele: Case report, Literature Review and a Proposed Classification. G.E. Sarris and D. Tsang. Surgery 105:408-414,
(March), 1989. A case of type III (Alonso-Lej) choledochal cyst known as a choledochocele is presented. In this patient, epigastric pain with hyperamylasemia 3 years after cholecystectomy prompted an upper gastrointestinal series showing a duodenal filling defect. Subsequent HIDA and CT scan findings are presented, and this patient as well as 48 other cases (ages 3 to 38 years) from the literature are reviewed. An anatomic classification is proposed whereby in type A choledochocele (67% of patients), the ampulla opens into the choledochocele, which communicates with the duodenum. Three anatomic type A subtypes are depicted, as well as type B (21% of patients) in which the cyst communicates only with the most distal common duct that empties through the "normal" ampulla. This classification provides a framework for operative or endoscopic management. Papillotomy or incision should be limited to only smaller lesions with the awareness of the potential development of carcinoma within unexcised lesions. Excision with or without sphincteroplasty is recommended.--Tom Tracy, Jr Radiological Treatment of Common Bile Duct Lithiasis in Infancy.
D. Pariente, O. Bernard, [7. Gauthier, et al. Pediatr Radiol 19:104107, (January), 1989. The authors report ten infants, aged 20 days to 11 months, who presented with common bile duct lithiasis and who were explored and cured by radiological procedures. Ultrasound examination showed bile duct dilatation in nine cases with sludge in the gallbladder in five and in the common bile duct in two. Histological findings of cholangitis were present in four infants. Percutaneous transhepatic cholangiograms were performed by gallbladder puncture in six infants and bile duct puncture in four. There was evidence of filling defects in the common bile ducts but no anatomical anomaly. Blackish concretions were removed through a side-holes catheter or pushed into the duodenum by washing with saline. An external drainage tube was left in place for a few days to allow for a control cholangiogram. Three infants underwent subsequent surgery but no residual lithiasis was found. No recurrence has occurred with a follow-up ranging from 10 months to 7 years. Mechanisms of this entity are discussed.--Prem Puri. Biliary Atresia. D. Schroeder and P.W.B. Pease. Pediatr Surg
Internat 4:101-104, (February), 1989. A retrospective study was performed on all babies presenting to Princess Mary Hospital, Auckland, with conjugated hyperbilirubi-
more frequently in patients with cystic fibrosis. They suggest that the dogma that appendicitis and cystic fibrosis is rare may be dangerous. The evidence for this is not conclusive and may lead to late diagnosis with increased morbidity in these patients.--J.D. Orr Segmental Aganglionosis of the Small Bowel--Myth or Reality? R.
Gonzalez-Vasquez and W.H. Heiss. Z Kinderchir 43:424-426, (December), 1985. Reports on zonal aganglionosis of the bowel are rare and the few cases reported concern colon and rectum. A case of segmental aganglionosis of the jejunum in a 2-month-old boy is reported. A narrow jejunal segment measuring 10 cm had to be resected. Histological examination revealed segmental aganglionosis of the myenteric plexus. The suggested causes of this rare zonal aganglionosis of the small bowel include (1) local damage of the plexus by hypoxemia, (2) primary abnormality in the gastrointestinal anlage, (3) intrauterine inflammation, and (4) double migration and development gradient of the neuroblast precursors. A defect in the microenvironment of the enteric neurons would result in failure of differentiation. This possible explanation would challenge the accepted theory of pathogenesis of colonic aganglionosis.--Thomas A. Angerpointner Reliability of Anorectal Manometry in the Diagnosis of Hirschsprung's Disease. N. Iwai, J. Yanagihara, K. Tokiwa, et al. Z
Kinderchir 43:405-407, (December), 1988. Anorectal manometry was performed in 79 patients, aged two days to 19 years, who were referred for diagnosis of constipation or symptoms of intestinal obstruction. Definitive diagnosis was obtained in 75 of the 79 patients (95%). Thus, anorectal manometry is a reliable tool in the diagnosis of Hirschsprung's disease. In the neonatal period, however, a definitive diagnosis was possible in only 17 of 21 neonates (81%). If intestinal obstruction can be managed conservatively, manometry should be repeated in neonates after 2 weeks if diagnosis cannot be definitely established by the first investigation. Additionally, barium enema and rectal suction biopsy should be performed if Hirschsprung's disease is suspected manometrically.--Thomas A. Angerpointner Posterior Sagittal Anorectoplasty for Reoperation in Children With Anoractal Malformations. A.J.L. Brain and E.M. Kiely. Br J
Surg 76:57-59, (January), 1989. The authors studied a consecutive series of 12 children who had previously undergone surgery for anorectal anomalies but whose fecal incontinence rendered them socially unacceptable. The majority had been born with a supralevator anoreetal anomaly with a fistula or a cloacal abnormality. Eight of the 12 children had already undergone unsuccessful attempts at secondary surgery. A further clinical and neurophysiological assessment was carried out preoperatively. This revealed anatomical problems, such as recurrent fistula, massive urethral diverticulum, absent vagina, and a missed urogenital sinus. The external sphincter was noted to be displaced from the anus in nine patients. Posterior sagittal anorectoplasty, with correction of the anatomical defects and precise reconstruction of the bowel within the sphincters, was carried out after the formation of a loop colostomy. No postoperative complications were observed. Contrast studies were performed to confirm healing of the suture lines before the stomas were closed. Follow up, from 4 to 46 months, revealed good fecal control in only two patients but with a significant improvement in two others. Although the rest remain incontinent, sensation was improved. The authors note that these results are disappointing and at variance with previously published reports. They suggest that the
INTERNATIONAL ABSTRACTS
posterior sagittal anorectoplasty used as a secondary procedure can correct the anatomical defects but may not improve fecal incontinence.--J.D. Orr Anal Bleeding Caused by Tubular Rectal Duplication With Ectopic Gastric Mucosa. C. Janneck. Z Kinderchir 43:353-354, (October),
1988. The author reports an extremely rare rectoreetal duplication, containing gastric mucosa with a fistula to the rectum in a 10year-old boy with myelodysplasia syndrome, anal stenosis, and a sacral defect. Persistent tarry stools prompted diagnosis and extirpation of the duplication. The combination of anorectal malformations, presacral mass, and sacrococcygeal defect may indicate a Currarino triad, an autosomal dominant hereditary syndrome that can be simply detected and identified by x-ray examination of the sacrum.-- Thomas A. Angerpointner ABDOMEN Choledochocele: Case report, Literature Review and a Proposed Classification. G.E. Sarris and D. Tsang. Surgery 105:408-414,
(March), 1989. A case of type III (Alonso-Lej) choledochal cyst known as a choledochocele is presented. In this patient, epigastric pain with hyperamylasemia 3 years after cholecystectomy prompted an upper gastrointestinal series showing a duodenal filling defect. Subsequent HIDA and CT scan findings are presented, and this patient as well as 48 other cases (ages 3 to 38 years) from the literature are reviewed. An anatomic classification is proposed whereby in type A choledochocele (67% of patients), the ampulla opens into the choledochocele, which communicates with the duodenum. Three anatomic type A subtypes are depicted, as well as type B (21% of patients) in which the cyst communicates only with the most distal common duct that empties through the "normal" ampulla. This classification provides a framework for operative or endoscopic management. Papillotomy or incision should be limited to only smaller lesions with the awareness of the potential development of carcinoma within unexcised lesions. Excision with or without sphincteroplasty is recommended.--Tom Tracy, Jr Radiological Treatment of Common Bile Duct Lithiasis in Infancy.
D. Pariente, O. Bernard, [7. Gauthier, et al. Pediatr Radiol 19:104107, (January), 1989. The authors report ten infants, aged 20 days to 11 months, who presented with common bile duct lithiasis and who were explored and cured by radiological procedures. Ultrasound examination showed bile duct dilatation in nine cases with sludge in the gallbladder in five and in the common bile duct in two. Histological findings of cholangitis were present in four infants. Percutaneous transhepatic cholangiograms were performed by gallbladder puncture in six infants and bile duct puncture in four. There was evidence of filling defects in the common bile ducts but no anatomical anomaly. Blackish concretions were removed through a side-holes catheter or pushed into the duodenum by washing with saline. An external drainage tube was left in place for a few days to allow for a control cholangiogram. Three infants underwent subsequent surgery but no residual lithiasis was found. No recurrence has occurred with a follow-up ranging from 10 months to 7 years. Mechanisms of this entity are discussed.--Prem Puri. Biliary Atresia. D. Schroeder and P.W.B. Pease. Pediatr Surg
Internat 4:101-104, (February), 1989. A retrospective study was performed on all babies presenting to Princess Mary Hospital, Auckland, with conjugated hyperbilirubi-