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Is specialist centre delivery of gastroschisis beneficial?
INTERNATIONAL
ABSTRACTS
1509
inflammation of the rectosigmoid can be present in untreated children with ulcerative colitis at the onset of the disease. In fact, these patients may be classified as having Crohn’s disease. Physicians managing children with colitis must be aware of these diagnostic problems if the potential therapeutic pitfalls that might follow from an inaccurate diagnosis are to be avoided.--Richard R. Ricketts
Chronic Constipation in Children. V. LoeningBaucke. terology 105: I557- 1564. (November). 1993.
Gastroen-
This is an excellent review of the evaluation, differential diagnosis, and management of chronic constipation and encopresis in children. Encopresis is usually a complication of long-standing constipation. It is reported to affect 2.8% of 4 year olds, 1.9% of 6 year olds, and 1.6%, of 10 to 11 year olds. The male:female ratio ranges from 2.5: 1 to 6: I. The differential diagnosis of constipation with or without fecal soiling includes idiopathic constipation of childhood, which accounts for 90% to 95% of the cases. The remaining 5% to 10% of cases result from anal lesions (fissures, anterior location, stenosis), neurogenic abnormalities (meningocele or tumor, hypotonia, Hirschsprung’s disease, or chronic intestinal pseudoobstruction), endocrine and metabolic disorders (hypothyroidism, diabetes insipidus, hypercalcemia), or constipation induced by drugs. A nice algorithm for the workup of constipation in childhood is presented. Once Hirschsprung’s disease is ruled out by anorectal manometry. barium enema, and/or rectal biopsy, a constipation treatment program is instituted. This program consists of patient and parent education, fecal disimpaction, and a maintenance program comprising a regular schedule of toilet sitting, increased dietary fiber, and laxatives. If defecation dynamics are abnormal. biofeedback treatment may be instituted as well. The maintenance program is continued for at least 6 months. Follow-up studies at the encopresis clinic of the University of Iowa showed that among patients over 5 years of age, approximately 50% will be off laxatives and not be constipated or have soiling by 12 months. Another 20%* may be weaned from the laxative program within 2 years. The remainder will require laxatives for daily bowel movements or may have continued soiling for many years, occasionally into adulthood. This article is a good review of a very common prohlem seen by pediatric surgeons.-Richard R. Ricketts
ABDOMEN Antenatal Diagnosis of Choledochal Cyst. J.D. Bancroft. J.C. Bucu\,alas. R.C. Rvckman, et al. J Pediatr Gastroenterol Nutr 18:142-145. (February). 1994. The authors report on five infants who were diagnosed as having choledochal cyst by prenatal ultrasonography, and on seven previously published cases. All but one infant had type I (Alonso-Lej) cysts. The diagnoses were made during the second or third trimester. Eight of the 12 infants became jaundiced in the first few days of life: three patients had palpable abdominal masses. There was complete obstruction of the distal common bile duct in seven of the nine infants who underwent operative cholangiography. An interesting finding was fibrosis on the liver biopsy specimens of patients who were operated on as early as 1 week after delivery, indicating that the human fetal liver is capable of fibrotic change. Despite the evidence of fibrosis, five patients demonstrated clinical and biochemical improvement after surgical excision and ileoenteric anastomoses. It is emphasized that choledochal cyst is a surgically correctable disorder that may present on antenatal ultrasonography. Prompt evaluation and treatment of neonates with antenatal detection of a sonolucent intraabdominal mass is needed.Thomas F. Tratv, h
Emergency Liver Transplantation After Kasai Pottoenterostomy. MT. Corbal&, N. Heaton. h4. Rela, et al. Arch Dis Child 70:147-148. (February), 1994. The authors report on three patients who had had stable liver function after Kasai portoenterostomy but who had acute onset of a febrile illness initially believed to be cholangitis. All the children were in their first year of life. The condition of all three deteriorated because of progressive liver necrosis. One child died and two survived after emergency liver transplantation. The authors highlight the seriousness of this condition of acute liver necrosis. Of the few previous cases reported. all had died. Emergency liver transplantation is the only successful treatment. Acute liver necrosis may be distinguished from cholangitis by very high transaminase activities, prolongation of the international normalized ratio, and progressive hepatic coma.-D.M. Bor,e Pancreatitis in Patients With Organic Acidemias. S.G. Kahler, W G. Sherwood, D. Woo(t; et al. J Pediatr 124:239-243, (February), 1994. The records of all patients with organic acidemias who had follow-up at five institutions during the past 10 years were reviewed. Pancreatitis was recognized by symptoms and laboratory findings and confirmed by imaging studies. surgery, or autopsy. At three institutions, all cases of pancreatitis in children under age 10 years were reviewed. There were nine children with pancreatitis (7 with acute, two with chronic) among 108 children with branchedchain organic acidemias. They ranged in age from 13 months to 9 years. Five had methylmalonic acidemia, three had isovaleric acidemia, and one had maple syrup urine disease. There were three deaths; acute hemorrhagic pancreatitis occurred in two, and chronic pancreatitis (found at autopsy) in the third. All three patients with isovaleric acidemia and pancreatitis were identified after the occurrence of pancreatitis. Through a survey of pancreatitis at three institutions. 57 other patients were found (none with an inborn error) in addition to three with inborn errors included in this study. It is concluded that acute or chronic pancreatitis may complicate branched-chain organic acidemias and must be considered in the assessment of patients with these disorders who have acute clinical deterioration and vomiting, abdominal pain, encephalopathy or shock, or milder symptoms. Conversely. an inborn error of organic acid metabolism should be considered in children with pancreatitis of unknown origin.-George W. Holcomb. Jr Is Specialist Centre Delivery of Gastroschisis Beneficial? G. Nicholl.7. r/: Upadhyaya, P. Gomall, et al. Arch Dis Child 69 (Fetal Neonatal ed):71-73. (July), 1993. The authors report on 43 consecutive infant cases of gastroschisis treated at Birmingham Children’s Hospital. This hospital does not have an obstetric unit, and the babies were admitted from either the regional obstetric unit in the city (9 infants) or outlying obstetric units (34 infants). The authors found no significant difference in gestational age, birth weight, cesarean section rate, time until surgery. or mortality rate between the two groups. There was also no significant difference with respect to the mean time to establish enteral feeding or of the number of successful primary closures. It is concluded that there are no significant advantages to in utero transfer in these pregnancies.-D.M. Rune Laparoscopy for Right Lower Quadrant Pain in Children. F. Schier and J. Waldschmidt. Pediatr Surg Int 9:258-260, (April), 1994. The authors report their experience with 170 laparoscopic appendicectomies performed since 1988. They observe that the visualiza-
ABSTRACTS
1509
inflammation of the rectosigmoid can be present in untreated children with ulcerative colitis at the onset of the disease. In fact, these patients may be classified as having Crohn’s disease. Physicians managing children with colitis must be aware of these diagnostic problems if the potential therapeutic pitfalls that might follow from an inaccurate diagnosis are to be avoided.--Richard R. Ricketts
Chronic Constipation in Children. V. LoeningBaucke. terology 105: I557- 1564. (November). 1993.
Gastroen-
This is an excellent review of the evaluation, differential diagnosis, and management of chronic constipation and encopresis in children. Encopresis is usually a complication of long-standing constipation. It is reported to affect 2.8% of 4 year olds, 1.9% of 6 year olds, and 1.6%, of 10 to 11 year olds. The male:female ratio ranges from 2.5: 1 to 6: I. The differential diagnosis of constipation with or without fecal soiling includes idiopathic constipation of childhood, which accounts for 90% to 95% of the cases. The remaining 5% to 10% of cases result from anal lesions (fissures, anterior location, stenosis), neurogenic abnormalities (meningocele or tumor, hypotonia, Hirschsprung’s disease, or chronic intestinal pseudoobstruction), endocrine and metabolic disorders (hypothyroidism, diabetes insipidus, hypercalcemia), or constipation induced by drugs. A nice algorithm for the workup of constipation in childhood is presented. Once Hirschsprung’s disease is ruled out by anorectal manometry. barium enema, and/or rectal biopsy, a constipation treatment program is instituted. This program consists of patient and parent education, fecal disimpaction, and a maintenance program comprising a regular schedule of toilet sitting, increased dietary fiber, and laxatives. If defecation dynamics are abnormal. biofeedback treatment may be instituted as well. The maintenance program is continued for at least 6 months. Follow-up studies at the encopresis clinic of the University of Iowa showed that among patients over 5 years of age, approximately 50% will be off laxatives and not be constipated or have soiling by 12 months. Another 20%* may be weaned from the laxative program within 2 years. The remainder will require laxatives for daily bowel movements or may have continued soiling for many years, occasionally into adulthood. This article is a good review of a very common prohlem seen by pediatric surgeons.-Richard R. Ricketts
ABDOMEN Antenatal Diagnosis of Choledochal Cyst. J.D. Bancroft. J.C. Bucu\,alas. R.C. Rvckman, et al. J Pediatr Gastroenterol Nutr 18:142-145. (February). 1994. The authors report on five infants who were diagnosed as having choledochal cyst by prenatal ultrasonography, and on seven previously published cases. All but one infant had type I (Alonso-Lej) cysts. The diagnoses were made during the second or third trimester. Eight of the 12 infants became jaundiced in the first few days of life: three patients had palpable abdominal masses. There was complete obstruction of the distal common bile duct in seven of the nine infants who underwent operative cholangiography. An interesting finding was fibrosis on the liver biopsy specimens of patients who were operated on as early as 1 week after delivery, indicating that the human fetal liver is capable of fibrotic change. Despite the evidence of fibrosis, five patients demonstrated clinical and biochemical improvement after surgical excision and ileoenteric anastomoses. It is emphasized that choledochal cyst is a surgically correctable disorder that may present on antenatal ultrasonography. Prompt evaluation and treatment of neonates with antenatal detection of a sonolucent intraabdominal mass is needed.Thomas F. Tratv, h
Emergency Liver Transplantation After Kasai Pottoenterostomy. MT. Corbal&, N. Heaton. h4. Rela, et al. Arch Dis Child 70:147-148. (February), 1994. The authors report on three patients who had had stable liver function after Kasai portoenterostomy but who had acute onset of a febrile illness initially believed to be cholangitis. All the children were in their first year of life. The condition of all three deteriorated because of progressive liver necrosis. One child died and two survived after emergency liver transplantation. The authors highlight the seriousness of this condition of acute liver necrosis. Of the few previous cases reported. all had died. Emergency liver transplantation is the only successful treatment. Acute liver necrosis may be distinguished from cholangitis by very high transaminase activities, prolongation of the international normalized ratio, and progressive hepatic coma.-D.M. Bor,e Pancreatitis in Patients With Organic Acidemias. S.G. Kahler, W G. Sherwood, D. Woo(t; et al. J Pediatr 124:239-243, (February), 1994. The records of all patients with organic acidemias who had follow-up at five institutions during the past 10 years were reviewed. Pancreatitis was recognized by symptoms and laboratory findings and confirmed by imaging studies. surgery, or autopsy. At three institutions, all cases of pancreatitis in children under age 10 years were reviewed. There were nine children with pancreatitis (7 with acute, two with chronic) among 108 children with branchedchain organic acidemias. They ranged in age from 13 months to 9 years. Five had methylmalonic acidemia, three had isovaleric acidemia, and one had maple syrup urine disease. There were three deaths; acute hemorrhagic pancreatitis occurred in two, and chronic pancreatitis (found at autopsy) in the third. All three patients with isovaleric acidemia and pancreatitis were identified after the occurrence of pancreatitis. Through a survey of pancreatitis at three institutions. 57 other patients were found (none with an inborn error) in addition to three with inborn errors included in this study. It is concluded that acute or chronic pancreatitis may complicate branched-chain organic acidemias and must be considered in the assessment of patients with these disorders who have acute clinical deterioration and vomiting, abdominal pain, encephalopathy or shock, or milder symptoms. Conversely. an inborn error of organic acid metabolism should be considered in children with pancreatitis of unknown origin.-George W. Holcomb. Jr Is Specialist Centre Delivery of Gastroschisis Beneficial? G. Nicholl.7. r/: Upadhyaya, P. Gomall, et al. Arch Dis Child 69 (Fetal Neonatal ed):71-73. (July), 1993. The authors report on 43 consecutive infant cases of gastroschisis treated at Birmingham Children’s Hospital. This hospital does not have an obstetric unit, and the babies were admitted from either the regional obstetric unit in the city (9 infants) or outlying obstetric units (34 infants). The authors found no significant difference in gestational age, birth weight, cesarean section rate, time until surgery. or mortality rate between the two groups. There was also no significant difference with respect to the mean time to establish enteral feeding or of the number of successful primary closures. It is concluded that there are no significant advantages to in utero transfer in these pregnancies.-D.M. Rune Laparoscopy for Right Lower Quadrant Pain in Children. F. Schier and J. Waldschmidt. Pediatr Surg Int 9:258-260, (April), 1994. The authors report their experience with 170 laparoscopic appendicectomies performed since 1988. They observe that the visualiza-