Preduodenal portal vein, jejunal atresia, and short bowel syndrome—A new combination of intestinal and vascular malformations

The serial transverse enteroplasty procedure (STEP) was introduced as a bowel-lengthening procedure to reduce complications related to short bowel syndrome (SBS). Although some have described it as a useful adjunct to the Bianci procedure, others have acknowledged it as a primary procedure. We present a case of jejunal atresia in which two STEP procedures were performed 7 months apart to increase small bowel length. A 1-day-old, term girl presented with a known bowel obstruction diagnosed in utero. A laparotomy revealed a Type IIIb jejunal atresia with no remaining small bowel or cecum. A STEP procedure with an end jejunostomy and ascending colon mucous fistula lengthened the small bowel from 35 to 50 cm. A repeat procedure 7 months later lengthened it to 89 cm. The STEP procedure results in slower intestinal transit time and increases enterocytes contact with oral intake. We performed it during our initial exploration to increase small bowel size by 30 per cent. A repeat procedure 7 months later increased length to 89 cm. The use of multiple, staged STEP procedures avoided the need for bowel transplantation and long-term total parenteral nutrition dependence, demonstrating its effectiveness as a primary procedure for the surgical management of SBS.

Download Full-text

Preduodenal portal vein, malrotation, and high jejunal atresia: a case report

Journal of Pediatric Surgery ◽

10.1016/j.jpedsurg.2011.09.059 ◽

2012 ◽

Vol 47 (1) ◽

pp. e27-e30 ◽

Cited By ~ 6

Author(s):

Maciej Baglaj ◽

Sylwester Gerus

Keyword(s):

Case Report ◽

Portal Vein ◽

Jejunal Atresia ◽

Preduodenal Portal Vein

Download Full-text

Vanishing gastroschisis with jejunal atresia and extreme short bowel syndrome: A case series

Journal of Neonatal Surgery ◽

10.47338/jns.v10.932 ◽

2021 ◽

Vol 10 ◽

pp. 7

Author(s):

Naresh Pawar ◽

Pramila Sharma ◽

Punit Singh Parihar ◽

Manika Boipai

Keyword(s):

Abdominal Wall ◽

Short Bowel Syndrome ◽

Anterior Abdominal Wall ◽

Abdominal Wall Defect ◽

Case Series ◽

Rare Condition ◽

Wall Defect ◽

Short Bowel ◽

Jejunal Atresia ◽

Tailored Approach

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.

Download Full-text