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Gastroschisis is a congenital defect of the anterior abdominal wall . The baby is born with a full thickness tear to the right of the umbilicus with evisceration of segments of small bowel
Epidemiology : 1/300 births
Aetiology : Vascular accident of the right omphalomesenteric artery and abuse of vasoactive drugs . This occurs during the 10th week of intrauterine life following obliteration of right umbilical vein
Presentation :
Herniated loops of small and large bowel is to the right of a normally placed umbilicus , seperated from it by a small bridge of skin. Bowel is not covered by sac . Bowel loops appear shortened, dilated and thickened. Bowel may appear oedematous in cases presenting late to tertiary centres. Such is the finding in images D and E. Bowel is covered by fibrinoid material pointing to the reaction of the bowel to amniotic fluid in utero. The babies may present with ileus and vomiting due to poor bowel motility or intestinal obstruction
Investigations :
Antenatal abdominal ultrasound can be used to make diagnosis before 20 weeks in 70 -72 percent of cases . 50 percent of cases diagnosed antenatally are small for gestational age.This helps to plan delivery which in most caseswould be by Caesarean section though this mode of delivery does not confer any advantage over vaginal delivery
FBC, elctrolytes are done prior to any surgical intervention
Treatment :
Reduction of bowel with surgical closure of the defect is the primary goal. The bowel must be protected from dessication, further injury and hypothermia. This is done by suturing silastic bag to the edges of the defect. The bowel is gradually reduced manually over 4-5 days to avoid putting undue pressure on the inferior vena cava. Bowel motility may take about 4-6 weeks to return and so parenteral nutrition must be commenced as soon as possible. Most of the babies born with this congenital defect do not have other congenital disorders and so have a better prognosis at survival. Mortality is about 17 percent.
Challenges : There has been doccumented higher mortalities in most centers in Ghana for the following reasons
1. Lack of parenteral feeds for neonates
2. Lack of specialist care in peripheral health facilities
3. Lack of ICU services in peripheral health facilities
4. Lack of skilled antenatal USG services to pick these these anomalies and refer to tertiary facilities
5.Failed diagnosis leading to delayed presentation –
( Image D) is a 6 day old baby who was misdiagnosed as a case of ompalocele and referred. Baby was ill looking had cord sepsis and was dehydrated . Bowel was offensive with a segment of necrotic tissue at the through the defect. A diagnosis of gastroschisis , cord sepsis and strangulated bowel . Surgical exploration of the abdomen revealed underdeveloped terminal ileum, and colon which were resected. Jejunum was anastomosed to the distal bowel which was about 7cm to the anus. The resected segment on opening revealed failed tuburisation in most areas. Note the thin strip of mucosa shown by the needle
Credits : The Paediatric Surgical Team, MBU , KATH Kumasi
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