• Title/Summary/Keyword: Congenital intestinal atresia

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Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization

  • Kylat, Ranjit I;Witte, Marlys H;Barber, Brent J;Dori, Yoav;Ghishan, Fayez K
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.22 no.6
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    • pp.594-600
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    • 2019
  • With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, postdouble switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ${\leq}0.9g/dL$) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Generalized Meconium Peritonitis Diagnosed with Antenatal Ultrasonography (산전 초음파검사로 진단된 범발성 태변성 복막염 2예보고)

  • Kim, Soong-Chul;Hwang, Shin;Yoo, Shi-Joon;Kim, In-Koo
    • Advances in pediatric surgery
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    • v.1 no.1
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    • pp.59-62
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    • 1995
  • Meconium peritonitis is defined as an aseptic, chemical or foreign-body peritonitis caused by spill of meconium in the abdominal cavity related to the prenatal perforation of the intestine. Perforation is usually caused by obstruction from meconium ileus, intestinal atresia, stenosis, volvulus, internal hernia, congenital peritoneal bands, intussusception, or gastroschisis. Less commonly, no evidence of distal obstruction exists. Here, we present two cases of generalized meconium peritonitis of antenatal diagnosis. The first case, detected at 8 months of gestational age, had a perforation of the proximal blind pouch of jejunal atresia, associated with respiratory distress due to severe abdominal distension. This case was successfully treated with resection and anastomosis and brief period of postoperative ventilatory support. The second case had a distal ileal perforation with thick meconium in the terminal ileum. In this case, there was no dilatation of ileum proximal to the perforation site. Resection and anastomosis was performed and postoperative course was uneventful.

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The Impact of Prenatal Diagnosis on the Outcome of Neonatal Surgical Emergencies Evaluated by Mortality Rate (소아외과 영역의 선천성 응급 질환에서 산전 진단이 술후 사망률에 미친 영향)

  • Kim, Tae-Hoon;Lee, Seong-Cheol;Kim, Hyun-Young;Jung, Sung-Eun;Park, Kwi-Won;Kim, Woo-Ki
    • Advances in pediatric surgery
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    • v.10 no.2
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    • pp.87-91
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    • 2004
  • As prenatal ultrasonography becomes popular, the number of prenatal diagnosis of congenital surgical diseases is also increasing. To evaluate the impact of antenatal ultrasonography on outcome the mortality rate in neonatal surgical emergencies was studied. The authors retrospectively reviewed 281 patients (congenital diaphragmatic hernia: 44, tracheoesophageal fistula: 78, intestinal atresia: 98, omphalocele: 28 and gastroschisis: 33 who had been managed at Seoul National University Childrens Hospital, from January 1991 to December 2000. The patients were divided into two groups; group A (1991 to 1995; 139 patients) and group B (1996 to 2000; 142 patients). These two groups were subdivided into prenatally diagnosed subgroup and postnatally diagnosed subgroup. We analyzed the changes of prenatal diagnosis rate, total mortality rate, and mortality rate of subgroups. Prenatal diagnosis rate was increased significantly in group B (Group A: 24.5 % and Group B: 45.1 %). Total mortality rate of group A was 21.6 %, and that of group B was 10.6 %, showing a significant decrease in group B. However, in both group A and B, when compared antenatally diagnosed subgroup with postnatally diagnosed subgroup, the mortality rate was lower in postnatally diagnosed subgroups but statistically not significant. The authors conclude that although prenatal diagnosis rate has been increased, prenatal diagnosis itself has not resulted in significant improvement in outcome.

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