Familial Apple Peel Jejunal Atresia with Helical Umbilical Cord Ulcerations in Three Consecutive Pregnancies

2016 ◽  
Vol 19 (1) ◽  
pp. 51-55 ◽  
Author(s):  
Sunil Jaiman ◽  
Sirisha Rao Gundabattula ◽  
Chinmayee Ratha
2019 ◽  
Vol 12 (8) ◽  
pp. e230160
Author(s):  
Jyotsna M Kirtane ◽  
Snehal A Bhange ◽  
Fazal Nabi ◽  
Varshil Shah

This is a case report of a neonate who was antenatally diagnosed with jejunal atresia which turned out to be duodenal atresia with apple peel syndrome. A previous sibling, who also had apple peel but with jejunal atresia, succumbed to sepsis after surgery. The first sibling had jejunal stenosis and had died of sepsis following surgery. Combination of duodenal atresia with apple peel is extremely rare. This coupled with a familial condition is rarer still. This case was challenging due to the short length of the gut and prolonged need for total parenteral nutrition and sepsis in postoperative period.


2000 ◽  
Vol 124 (6) ◽  
pp. 880-882
Author(s):  
Shoji Yamanaka ◽  
Yukichi Tanaka ◽  
Motoyoshi Kawataki ◽  
Rieko Ijiri ◽  
Kiyoshi Imaizumi ◽  
...  

Abstract We present an autopsy case of a 46-day-old male infant with chromosome 22q11 deletion, which is considered the primary cause of several diseases, including DiGeorge syndrome and velocardiofacial syndrome. The patient had 2 notable congenital abnormalities: multiple dissecting pulmonary arterial aneurysms distributed in both lungs and multiple jejunal atresia with apple-peel deformity. The former, a very rare pathologic condition especially in infancy, was found incidentally at autopsy and was the primary cause of death. To our knowledge, neither of these lesions has been reported previously in a patient with chromosome 22q11 deletion.


Author(s):  
Mahmoud Marei Marei ◽  
Mohamed Hamed Abouelfadl ◽  
Ahmed Arafa Elsayed Rawwash ◽  
Hamed Mahmoud Seleim ◽  
Wesam Mohamed Mahmoud ◽  
...  

Abstract Background High jejunal atresia is associated with significant dilatation of the proximal segment. This poses two problems: (a) calibre discrepancy with the distal unused segment and (b) hypomotility causing stasis. Tapering jejunoplasty/enteroplasty could offer a practical solution in selected cases, leading to early establishment of feeds. This work aims to evaluate the outcome of tapering jejunoplasty including its effect on establishing enteral feeding in neonates with proximal jejunal atresia. Results Twenty-two neonates with jejunal atresia (types I, II and IIIa) were reviewed. Cases with multiple atresia, apple-peel variant and meconium ileus were excluded. The included cases fell retrospectively into two groups: group A (13 cases)—very proximal atresia and significant dilatation and group B (9 cases)—mid/distal jejunal atresia. For group A, we excised only the distal tip of the dilated bowel and stripped a seromuscular triangle up to the duodenojejunal flexure and inverted the mucosa along the antimesenteric border, followed by an end-to-oblique anastomosis. For group B, we performed a standard excision of a short proximal segment and an end-to-oblique anastomosis. There was no significant difference in the gestation age or birth weight between both groups. The mean operative time was 90 min for group A and 60 min for group B. The duration until full enteral feeds became tolerated, and parenteral nutrition was weaned accordingly was shorter in group A (mean 10.8 days) as compared to group B (mean 14.5 days), p = 0.045. Conclusion Tapering jejunoplasty by seromuscular stripping and mucosal inversion facilitates early establishment of feeds in proximal jejunal atresia.


1987 ◽  
Vol 22 (5) ◽  
pp. 451-453 ◽  
Author(s):  
Laurie S. Ahlgren
Keyword(s):  

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
William M. Curtin ◽  
Jaimie L. Maines ◽  
Christina T. DeAngelis ◽  
Niamh A. Condon ◽  
Serdar H. Ural ◽  
...  

Umbilical cord ulceration has been associated with congenital upper intestinal (duodenal or jejunal) atresia and can lead to fatal fetal intrauterine hemorrhage. We report a case of spontaneous hemorrhage from the umbilical cord, incidentally noted at the time of ultrasound in a 33-week fetus with suspected duodenal atresia, in which immediate delivery resulted in a good outcome. Despite many reports in the literature of congenital upper intestinal atresia and its association with umbilical cord ulceration, the propensity for this lesion for fetal hemorrhage, and the resulting perinatal morbidity and mortality, there appears to be a gap in the dissemination of this knowledge. In fetuses with suspected congenital upper intestinal atresia, recognition of the entity of umbilical cord ulceration may be improved by ultrasound with special attention to the amount of Wharton’s jelly within the cord. Routine antepartum fetal surveillance may reduce perinatal morbidity and mortality from this condition. A high index of suspicion is needed to make the diagnosis of umbilical cord ulceration in association with congenital upper intestinal atresia. The role of amniotic fluid bile acids in the genesis of this disorder needs further study.


1991 ◽  
Vol 6 (6) ◽  
Author(s):  
MarkB. Smith ◽  
Leon Smith ◽  
William Wells ◽  
Emmanuel Shapira ◽  
Mark Hendrickson ◽  
...  
Keyword(s):  

Placenta ◽  
2010 ◽  
Vol 31 (11) ◽  
pp. 1015-1018 ◽  
Author(s):  
M. Ichinose ◽  
T. Takemura ◽  
K. Andoh ◽  
M. Sugimoto

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