A Proposed Classification for the Spectrum of Vanishing Gastroschisis
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Abstract:
Infants born with gastroschisis in association with intestinal atresia are well described. We are the proposing the classification of vanishing gastroschisis. In this series of six cases, at one end of the spectrum is an infant having gastroschisis with a much narrower defect on the right side of umbilicus. The ischemic bowel loops were connected to bowel inside the abdomen by a fibrous band compressing the exposed bowel mesentery. On the other end of spectrum, an infant having extensive bowel atresia and complete closure of abdominal wall defect (gastroschisis) detected on antenatal ultrasound. These cases should raise awareness of this devastating complication in prenatal management of gastroschisis.Keywords:
Gastroschisis
Intestinal atresia
Abdominal wall defect
Short Bowel Syndrome
<b><i>Background:</i></b> Gastroschisis can be associated with short bowel syndrome (SBS). We present 4 cases with significant bowel shortening with very different ante- and postnatal presentations. <b><i>Cases:</i></b> Three of the cases demonstrated ‘vanishing' gastroschisis with progressively worsening antenatal intra-abdominal bowel dilatation with no defect present at birth. The fourth case followed an uneventful course antenatally but developed SBS considered secondary to gastroschisis-related atresia and necrosis. All 4 cases were classified as having SBS and were enrolled within the paediatric Intestinal Rehabilitation Programme. This involves autologous gastrointestinal reconstruction following a period of bowel expansion with concomitant parenteral and enteral nutrition. <b><i>Discussion:</i></b> These cases demonstrate that the serious complication of significant bowel loss cannot always be anticipated. Early multidisciplinary discussion with the parents enables a shared understanding of potential outcomes. It can aid the recognition of ‘vanishing' gastroschisis enabling expedient investigations and early intervention, minimising morbidity and maximising bowel length.
Gastroschisis
Short Bowel Syndrome
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Gastroschisis
Short Bowel Syndrome
Intestinal atresia
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Neonates with a prenatal diagnosis of gastroschisis but born without any abdominal wall defect have midgut atresia. The condition is rare, and most reported cases died as a result of short bowel syndrome [1]. We describe a case with gastroschisis diagnosed at 19 weeks’ gestation that resolved spontaneously in utero. The boy was born without an abdominal wall defect, but had midgut atresia and presented after 2 days with ileus. After short small bowel resection and primary anastomosis he is thriving without parenteral nutrition.
Gastroschisis
Intestinal atresia
Abdominal wall defect
Short Bowel Syndrome
Ileus
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Gastroschisis is a rare congenital defect of the ventral abdominal wall through which abdominal contents herniate outside of the body without a peritoneal sac. The most common location of the defect is to the right of the umbilicus, however, there have been case reports of defects in alternate locations along the abdominal wall, particularly on the left side. Here we describe a case of a baby born with a gastroschisis-like defect in the midline supraumbilical abdominal wall, the only such case described in the literature, to our knowledge. The defect was small with herniated bowel and loss of domain in the abdomen requiring delayed closure with a silo. Cases of gastroschisis in unusual locations bring into question the pathogenesis of such defects, a topic which remains controversial.
Gastroschisis
Abdominal wall defect
Omphalocele
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Gastroschisis
Closure (psychology)
Abdominal wall defect
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Key Clinical Message Gastroschisis is a full‐thickness congenital abdominal wall defect usually occurring to the right of the umbilicus. About twenty cases of left‐sided gastroschisis have been reported, without reference to the laterality of the umbilical vein. This first case highlights the importance of considering and reporting this association by the perinatal team.
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Abdominal wall defect
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P605 Aims: Gastraschisis is the most frequent cause of pediatric intestinal transplant. The outcome for infants with gastroschisis has improved with current advantages in prenatal diagnosis, neonatal care and surgical techniques. However, a subset of these patients requires extensive bowel resections and in addition small portion of them develops long-term dependence on total parental nutrition (TPN) as a status of short-bowel syndrome. This study reviews our experience with intestinal transplantation for children who had sustained small intestinal loss as a result of gastroschisis in order to elucidate those factors affecting the eventual outcome of children with short-bowel syndrome secondary to gastroschisis. Methods: A retrospective chart review was performed for children who underwent small bowel transplantation for gastroschisis at the University of Miami between August 1994 and June 2003. Results: Thirty-two transplants were performed in twenty-eight children with gastroschisis during the study period. There were 14 boys and 14 girls with a median age of 23 months (range, 6 months to 8.5 years) at transplant. 14 patients (50%) were less than one year old at the time of transplant. Most patients were born prematurely and the average estimated gestation age was 34.5 weeks (range; 30 to 38 weeks). Mean birth weight was 2192 g. (range; 1235 g to 3012 g). Gastroschisis was prenatally diagnosed in 14 cases. Associated mid-gut anomalies were present in 22 infants (Bowel atresia was seen in 14 cases, Volvulus 3 cases, and/or Bowel ischemia 16 cases). Spontaneous prenatal closure of gastroschisis, which was considered to be a rare anomaly usually associated with bowel atresia and ischemia due to very small abdominal defect, was seen in 9 patients. In 3 patients, necrotizing enterocolitis (NEC) was developed after gastroschisis repair. Nineteen infants needed bowel resection within a week after birth. Most of the patients had complicated course and required multiple abdominal surgery before transplant. Transplantation types included isolated small intestine (n=8), liver and small intestine (n=8) and multivisceral transplants (n=16). Eighteen patients (64.3 %) currently are alive at a median follow-up of 15.7 months (range, 0.5 to 72 months). Short-term survival rate has significantly improved in recent years. Patient survival at 6 months, one year, and 2 years was: 50.0 %, 40.0 %, and 40.0% in the period between 1994 and 2000; 94.4 %, 77.9 %, and 77.9 % in 2001 through present. Conclusions: The outcome for most of the children with simple gastroschisis is quite good without being short bowel syndrome or intestinal failure. However, patients with complex gastroschisis, especially those with associated intestinal anomalies, likely to need extra surgeries in their early days of life and have a significant risk to become short bowel syndrome. Intestinal transplantation can be a life-saving option and provides a satisfactory outcome for children with gastroschisis associated short bowel syndrome who already developed serious TPN-related complications.
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Short Bowel Syndrome
Intestinal atresia
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Gastroschisis is a periumbilical, abdominal wall defect arising to the right of the umbilicus. We describe the clinical course of a left-sided gastroschisis in a term female born at 39 weeks' gestation. To our knowledge, there have been only 14 left-sided abdominal wall defects reported in the literature. We discuss our case and review the literature to try to determine if there is any clinic difference between right- versus left-sided lesions to make recommendations as to management.
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Gastroschisis is an anterior abdominal wall defect, usually to the right of the umbilicus. The defect results in herniation of fetal bowel into the amniotic cavity. The absence of a protective membrane around the bowel is the main difference with exomphalos, which is the other most common abdominal wall defect. It is usually an isolated abnormality and the prognosis is mainly determined by the condition of the bowel at birth.
Gastroschisis
Abdominal wall defect
Omphalocele
Abdominal cavity
Fetoscopy
Umbilical hernia
Prenatal ultrasound
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Background: Vanishing gastroschisis may occur due to spontaneous partial or complete closure of anterior abdominal wall defect around the viscera, leading to small bowel ischemia and resultant entry/exit level atresia and extremely short length of the remaining bowel. The prognosis is very poor, even after aggressive surgery, and requires prolonged total parenteral nutrition.
Case Series: We report two female neonates, one with closed and another with closing vanishing gastroschisis, associated with jejunal atresia and extreme short bowel syndrome. In both patients, the antenatal scans showed gastroschisis without the evidence of vanishing gastroschisis. In both neonates, palliative surgeries were done. Both patients died after a few days due to short bowel syndrome and sepsis.
Conclusion: When antenatally detected gastroschisis presents with closed or closing anterior abdominal wall defect, (vanishing gastroschisis), the parents/caregivers must be counseled about the poor prognosis of this condition. A tailored approach to either palliation or aggressive therapy is essential in this rare condition.
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Short Bowel Syndrome
Intestinal atresia
Abdominal wall defect
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