Congenital anterior abdominal wall defects. Gastroschisis has a good prognosis.
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Biomedical subjects
Publications and source records attributed to M D Stringer.
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A 14-year-old girl presenting with acute massive gastric dilatation secondary to duodenal obstruction by the superior mesenteric artery is described. The diagnosis was facilitated by contrast-enhanced abdominal computerized tomography. She was successfully treated by gastrostomy and subsequent duodenal derotation. This unique presentation of the superior mesenteric artery syndrome is discussed.
An 8-year-old boy presented with acute pancreatitis and was found to have a fenestrated duodenal web (windsock) and associated anomalies. After partial excision of the web and duodenoplasty, he has remained well with no further symptoms.
Isolated gastroschisis in two children of unrelated mothers but with the same father is described. There was no evidence of consanguinity, abnormal karyotype or a specific teratogen in either case. Genetic factors may be involved in the aetiology of some cases of gastroschisis.
AIMS: To assess the long term morbidity and quality of life in survivors of gastroschisis. DESIGN: All babies born with gastroschisis between 1972 and 1984 and who survived more than one year were identified. Those who could be traced were questioned about their general health, growth, abdominal symptoms, cosmetic concerns, education, employment, and fertility. RESULTS: Of the 35 patients, two have died, seven could not be traced, and three declined to be interviewed. Twenty three subjects (70% of survivors) with a median age of 16 years (range 12-23 years) responded. Twenty two (96%) were in good health and overall growth was within normal limits. Eight subjects (35%) have had further surgery related to gastroschisis, including two for adhesive small bowel obstruction and three for scar complications. In 13 (57%), absence of an umbilicus caused distress during childhood. CONCLUSION: Most gastroschisis survivors can eventually expect normal growth and good health. Adhesive bowel obstruction is an uncommon, but potentially late, complication. The umbilicus should be conserved during gastroschisis repair.
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A six year old boy underwent extensive investigation for recurrent abdominal pain and was found to have a persistently raised serum amylase. Endoscopic retrograde cholangiopancreatography was normal and macroamylasaemia was excluded. Serum amylase concentrations were found to be raised in other family members spanning three generations, all of whom were asymptomatic. Clearance studies suggested no evidence of a renal tubular defect and serum lipase concentrations were normal. This is the first report of apparently familial hyperamylasaemia and the mode of inheritance is consistent with an autosomal dominant pattern.
BACKGROUND: Femoral hernias are rare in children, accounting for fewer than 1 per cent of all paediatric groin hernias. Misdiagnosis is common and a source of complications. There is no consensus on the age and sex distribution or the optimum method of repair. METHODS: A personal experience of four children with femoral hernia is reported together with an institutional review of a further ten hernias encountered during the past 11 years. RESULTS: Peak incidence was between 5 and 10 years of age. Misdiagnosis was common, partly because of the variability in presenting symptoms and signs. In this series, boys were more commonly affected but a literature review indicated a similar sex incidence. CONCLUSION: A femoral hernia should be positively excluded if the operative findings at inguinal exploration are inconsistent with the preoperative signs and in any child with a suspected recurrent inguinal hernia. Excision of the sac and repair of the femoral canal is curative.
BACKGROUND: Perianal sepsis is a relatively common surgical problem in children and yet the contribution of rare aetiological factors, the frequency of fistula formation complicating perianal abscess, the significance of bacteriological findings and the optimum treatment of fistula in ano are poorly understood. METHODS: A consecutive series of 58 children (51 boys, seven girls) with a perianal abscess or fistula attending a tertiary referral centre over a 4-year period was reviewed. RESULTS: Thirty-eight children (34 boys, four girls), ranging in age from 1 month to 12 years, presented with an abscess. After treatment, three developed a further abscess but only four developed a fistula. Fistula formation only occurred in those with enteric bacterial infection. Of 24 children with a perianal fistula five required further surgery for recurrence, four of whom were initially treated by fistulotomy. In most patients, the fistula was simple, low and direct, and treatment by fistulectomy resulted in significantly fewer recurrences. Apart from Crohn's disease, identifiable aetiological factors were rare. CONCLUSION: Perianal sepsis is most common in otherwise healthy boys under 2 years of age. Abscess is best treated by incision and drainage, and fistulectomy is successful for the small proportion who develop a fistula.
The accuracy of the modified Alvarado score was assessed prospectively in the preoperative diagnosis of acute appendicitis in children. A consecutive series of 118 patients (54 boys, 64 girls) with acute abdominal pain was studied prospectively over a 6 month period. Appendicitis was confirmed in 38 of 43 children undergoing appendicectomy, giving a false-positive appendicectomy rate of 11.6%. No child under active observation was subsequently found to have a perforated appendix. The overall sensitivity of a modified Alvarado score of > or = 7 was 76.3% and its specificity was 78.8%. Current clinical practice is more accurate than the modified Alvarado score in the diagnosis of acute appendicitis in children.
Traumatic rupture of a splenic epidermoid cyst is a rare but recognised cause of "acute abdomen" in a child. We describe such a case occurring after very minor trauma, show the influence that imaging had in making an accurate diagnosis, and discuss how this influenced clinical management.
During the last 4 years, three infants have presented with finger-tip injuries secondary to entrapment in woollen/synthetic mittens. The accident happened at home in one case but the other two occurred in different neonatal units. Spontaneous amputation of the terminal phalanx of the index finger occurred in two patients but in the other there was complete healing. This problem may be avoided by restricting the use of mittens, by changing their design, and by a greater awareness of this hazard.
Three male fetuses examined sonographically in late gestation to assess growth and well-being were found to have evidence of gallbladder cholelithiasis. Postnatally, all three were asymptomatic but they had variable sonographic findings. In two, the abnormalities noted sonographically had resolved spontaneously by 6 weeks of age, but the third patient had a persistent gallbladder "calculus" at 6 months. All three boys, now aged 9 months to 4 years, remain well, with no evidence of biliary tract disease. Sonographic evidence of fetal gallbladder cholelithiasis may be found in late gestation, is more common among boys, and frequently is associated with benign spontaneous resolution.
The incidence, presentation, significance, and outcome of infants with internal enteric fistula formation secondary to necrotizing enterocolitis (NEC) were examined. Of 130 infants with NEC treated during a 7-year period, an enteric fistula developed in five (4%). The gestational age of these patients (3 boys, 2 girls) ranged from 25 to 40 weeks and their birth weight ranged from 800 to 3,460 g. Two had Down's syndrome. Plain abdominal radiographs showed widespread intramural gas in all, and portal vein gas in two. Four patients required early laparotomy, which confirmed extensive intestinal necrosis; a diverting jejunostomy or ileostomy was constructed in three, and the abdomen was closed with drainage in one. Fistulas were diagnosed by contrast radiology between 16 and 51 days after the onset of NEC, and were jejunocolic (2), ileocolic (2), and colocolic (1). They were associated with enteric stricture(s), an inflammatory mass, and clinical signs of intermittent sepsis. One infant with an ileocolic fistula died of sepsis before definitive surgical treatment. Of the four who underwent surgery, two survived after limited intestinal resection, but one of the two with short bowel syndrome died. Enteric fistula formation is a rare complication of NEC. Typically it occurs with colonic stricture(s) and is associated with signs of incomplete bowel obstruction and intermittent sepsis. Resectional surgery is successful, but there appears to be a significant risk of short bowel syndrome.
The authors studied the predictive value of detailed fetal sonographic parameters on outcome for 55 patients with prenatally diagnosed congenital diaphragmatic hernia managed at an ECMO center. Their sonographic assessment included gestational age at time of diagnosis, polyhydramnios (largest amniotic fluid pocket diameter), presence of liver and/or stomach herniation, and abdominal circumference at the level of the umbilical cord. They measured the right lung two-dimensional area at the level of the atria as an estimate of lung size and mediastinal shift. The ratio of right lung area to head circumference (LHR) was calculated to minimize lung size differences owing to gestational age. The principal outcome variable was survival. The overall survival rate was 65%. If the diagnosis was made after 25 weeks' gestation, the survival rate was 100% (12 of 12); the rate was 56% if the diagnosis was made at or before 25 weeks (P < .005). All five neonates with an LHR of less than 0.6 died; the survival rate was 100% for those whose LHR was greater than 1.35; and those with an LHR between 0.6 and 1.35 had a 61% survival rate (P < .001). The survival rate for those whose liver was not herniated was 100% (10 of 10); herniation of the liver decreased the survival rate to 56% (P < .05). Stomach position, polyhydramos, and abdominal circumference were not found to be useful survival predictors. No prenatal sonographic parameter was absolutely predictive of postnatal death except very small right lung size, which was present in only 5 of the 55 patients. Survival is highly likely if the liver is not herniated into the thorax and/or the right lung is large.
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This study analyses the morbidity and mortality of colostomy formation and closure over a 17-year period during which 138 consecutive infants and children had a colostomy formed as the initial management of Hirschsprung's disease or anorectal malformation. Complications after colostomy formation were encountered in 38 (27.5%) patients and included colostomy prolapse, stenosis, retraction, dysfunction, skin excoriation and parastomal hernia. The complication rate with transverse colostomies was higher than with other types. Colostomy closure was associated with complications in nine patients (6.5%), the most serious of which was adhesive small bowel obstruction (5). The mortality was less than 1%, but significant morbidity still exists. Refinements in surgical technique may help reduce the incidence of complications, but stoma prolapse, particularly with transverse colostomies, remains a major challenge.