Hereditary multiple intestinal atresia: thirty years later

Bilodeau, Audrey; Prasil, Pascale; Galvez‐Cloutier, Rosa; Laframboise, Rachel; Meguerditchian, Ari-Nareg; Roy, G.; Leclerc, Suzanne; Péloquin, Jean

doi:10.1016/j.jpedsurg.2004.01.031

Cited by 45 publications

(45 citation statements)

References 25 publications

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“…It has a frequent occurrence in northern Quebec and a dismal prognosis with virtually no survivors beyond several months of age. 9 Duodenal atresias, which are slightly more frequent than jejunal and ileal atresias together, 8 are very rarely multiple, usually located in the second duodenum and have a well-known association with other congenital anomalies including Down's syndrome (24% of cases), VACTERL association, Fanconi's anemia, CHARGE syndrome and other less common causes including microcephaly-oculo-digito-esophageal duodenal (MODED) or Feingold's syndrome and alveolar capillary dysplasia. MODED or Feingold's syndrome is an autosomal dominant condition associated with brachydactyly, variable microcephaly and duodenal and esophageal atresias.…”

Section: Denouement and Discussionmentioning

confidence: 99%

Apple peel small bowel

Herman

Siegel

2008

J Perinatol

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Case presentation A 2600 g infant was born at 36-week gestation to an 18-year-old gravida 2, para 2 mother after an uncomplicated pregnancy. The amniotic fluid was meconium stained. Apgars were 8 at 1 min and 9 at 5 min. The child had vomiting with initial feeds, which worsened over the first 18 h and the abdomen became progressively distended. An upper gastrointestinal series ( Figure 1) and contrast enema ( Figure 2) were performed. Denouement and discussionThe upper gastrointestinal series demonstrated atresia of the proximal bowel at the duodenojejunal junction. The enema demonstrated a malrotated microcolon in the anterior, lower mid abdomen. The presence of a microcolon with a proximal duodenal or jejunal atresia suggested at least a second more distal atresia or a more complex malformation. Attempts to reflux contrast into the terminal ileum beyond the ileocecal valve were unsuccessful. The patient was then taken to the operating room where a laparotomy disclosed a jejunal atresia just beyond the duodenojejunal junction and the absence of the dorsal mesentery except at the terminal ileum as it inserted into the cecum. The short segment of distal bowel was 'twisted' or spiraled just proximal to the cecum. Only 6 cm of distal small bowel were present. The terminal ileum was obstructed by a web-preventing passage of a catheter. This web was incised at enterotomy and the bowel was reanastomosed.Apple peel small bowel (APSB) is a disorder characterized by proximal jejunal or distal duodenal atresia, agenesis of the dorsal mesentery and variable parts of the distal small bowel, agenesis of the distal superior mesenteric artery and a malrotated microcolon. 1 The syndrome was named, however, after the appearance of the small bowel distal to the obstruction, which spirals in the abdomen like a continuous segment of cut apple peel. This spiral or helical appearance of the distal bowel can be seen on preoperative contrast

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Section: Denouement and Discussionmentioning

confidence: 99%

Apple peel small bowel

Herman

Siegel

2008

J Perinatol

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“…Bu mul tipl at re zi ti pin de kla sik tip 4 at re zi den fark lı ola rak me zen ter de fek ti gö rül mez. [2][3][4][5][6][7][8][9][10] Li te ratür de yak la şık ola rak 43 ol gu bil di ril miş tir. 10 Ula-şı la bi len li te ra tür ler de ka lıt sal bar sak at re zi li be bek le rin ai le geç mi şin de ak ra ba ev li li ği ol ma sı -na rağ men bi rin ci de re ce den ak ra ba ev li li ğin den do ğan kar deş ler de bar sak at re zi si ne bir li te ra tür de rast lan mış tır.…”

Section: Tar Tiş Maunclassified

“…Bu inf la ma tu var sü re cin ne de ni bi lin me mek te olup, ken di ol gu muz da yap tı ğı mız kis tik fib ro zi se yö nelik kro mo zom ana li zi ta yi nin de po zi tif bir so nuç elde edi le me miş tir. 2,10 Re ka na li zas yon de fek ti ve epi tel yu mun aşı rı pro li fe ras yo nu et yo pa to ge nez de ile ri sü rü len di ğer fak tör ler dir.…”

Section: Tar Tiş Maunclassified

“…Kardeşlerde ve akrabalarda nadir de olsa birkaç olgu rapor edilmiştir. [5][6][7][8][9][10] Hastalığın otozomal resesif geçiş-li olduğu düşünülmektedir. 11 Hastalar genellikle postoperatif dönemde kaybedilmektedirler.…”

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“…11 Hastalar genellikle postoperatif dönemde kaybedilmektedirler. 10 Cerrahi tedavinin amacı barsak devamlılığını sağla-mak ve maksimum barsak uzunluğunu korumaya çalışmaktır.…”

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