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Total Jejuno-Ileal Atresia-An Uncommon Condition 01/09/2014 00:00:00

Total Jejuno-Ileal Atresia-An Uncommon Condition

Aurel S. Mironescu, Liviu Muntean, Ecaterina Popa, Elena Georgescu.
Department of Pediatric Surgery, Brasov, Romania.

Aurel Mironescu, Spitalul Clinic de Copii Str.Nicopole Nr.45 500063 Brasov, Romania.
Intestinal atresia is commonly seen as one of the four types described in any of the specialty textbooks. None of those four types ever included a total jejuno-ileal atresia. We present the case of a one of the twin newborn infant, 34 weeks of gestation, 1300 g birth weight admitted in our department, for symptoms of high intestinal obstruction that proved to be an intestinal atresia of an unusual type, a total jejuno-ileal atresia. Inspite of this very serious malformation, the infant lived survived for over three months with just traditional methods of feeding.
intestinal atresia
Intestinal atresia is commonly seen as one of the four types described in any of the specialty literature. None of those four types ever included a total jejuno-ileal atresia. The surgical management for long gap atresias include a variety of methods, the results depending on several factors, among which one of the most important is the existence of a certain length of short bowel to start from.
Case Report
A premature baby boy, one of the twin newborns of a healthy, young mother, was admitted in our department, directly from the maternity hall in November 2005. On admission he weighed 1300 g and presented bilious vomiting since first 24 hours of life. A plain x-ray showed few distended loops in the upper abdomen and contrast (Gastrographin, Shering Co.) administered via a nasogastric tube failed to pass beyond a hugely dilated duodenum.

Figure 1a and b
<b>Figure 1a and b </b>

His general condition prevented an immediate operation. After about 48 hours of intensive therapy, including TPN and antibiotics, the infant was surgically explored. At surgery we found a hugely dilated duodenum, ending blindly at the level of the fourth segment. Some very small loops of colon were placed in contact with this blind duodenum, but no short gut, nor appendix. (Figure 2a and b). We performed a lateral anastomosis of the resected end caecum, on the dilated duodenum.

Figure 2a: Initial aspect at surgery
<b>Figure 2a: Initial aspect at surgery </b>

Fig 2b. Aspect after adhesiolysis
<b>Fig 2b. Aspect after adhesiolysis</b>

After surgery, the baby had a slow recovery, he was on TPN for three weeks, then continuous enteral feeding, progressively until up to 140 ml/24 h of formula, with very little progress in putting weight, eventually dying at 14 weeks, from an intractable septic complication, not related to his intestinal condition.
The international literature describes four types of intestinal atresia: type I or mucosal web, type II or fibrous cord, type IIIa or mesenteric gap defect, type IIIb or "apple peel" atresia and type IV or multiple atresias. (2,5,17,20) Intestinal atresia is a well-recognized cause of intestinal obstruction in the newborn, the management of the neonates born with this condition having been improved in recent years due to many new acquisitions in neonatal intensive care and anesthesia, operative procedures and use of total parenteral nutrition (TPN). With an incidence of 1 in 4000 to 5000 live births, intestinal atresia was initially considered to be produced by a lack of recanalization of the solid cord stage of the bowel (Tandler 1900) (18), this theory being confirmed only for the atresia of the duodenum. (2,5) Recent works come to confirm the suggested theory of Spriggs in 1912 (17, 20), that the origin of intestinal atresias might be mechanical accidents, including vascular occlusions, theory confirmed initially by the experiments of Louw and Barnard in 1955 who obtained intestinal atresias by inducing mesenteric vascular accidents in puppies. (11) Thus, it was confirmed that intrauterine clinical instances of intestinal volvulus, intussusception, internal hernia, or even constriction of the intestines in a tight gastroschisis could lead to intestinal atresia. (8,9,16,19) None of those reports mentioned the possibility of getting a total jejuno-ileal atresia, but it is obvious that a volvulus of the entire small bowel during the early intrauterine phases could be the explanation for such a type of atresia. The antenatal diagnosis of small bowel atresia, suspected by visualizing multiple distended loops of bowel, is considered as a sign for a more pronounced in utero intestinal distention and, as a result, a longer period of postnatal intestinal dysfunction and a longer need for TPN. (3,4) There have been described different new techniques to get better results with intestinal atresia and diminish as much as possible the consequences of the short bowel syndrome. (1,6,7,8,10,12,13,15) All those new procedures started from some available small gut, but for a total jejun-ileal atresia, as the one we are describing, the only surgical and logical solution would be an intestinal transplantation, a method available in only very few institutions in the world. (14)

By presenting this intriguing case, we want to see if anyone else has come to see such an uncommon condition and share experiences but also discuss about the opportunity of including it in one of the types of intestinal atresia, in the future.
Compliance with Ethical Standards
Funding None
Conflict of Interest None
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Cite this article as:
Mironescu A S, Muntean L, Popa E, Georgescu E. Total Jejuno-Ileal Atresia-An Uncommon Condition. Pediatr Oncall J. 2007;4: 35.
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