Aurel S Mironescu, Liviu Muntean, Ecaterina Popa, Elena Georgescu
Department of Pediatric Surgery, Brasov, Romania
|Address for Correspondence|
|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 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.|
|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
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
Fig 2b. Aspect after adhesiolysis
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.
Last Updated : Tuesday, May 01, 2007 Vol 4 Issue 5 Art #17
- Alexander F, Babak D and Goske M. Use of intraluminal stents in multiple intestinal atresia. J Pediatr Surg. 37, E34: 1-3, 2002
- Ashcraft KW, ed, Murphy JP, ass.ed, Sharp RJ, ass.ed, Sigalet DL, ass.ed, Snyder CL, ass. ed. Pediatric Surgery 3rd ed. W.B.Saunders Company: 2000
- Basu R, Burge DM. The effect of antenatal diagnostic on the management of small bowel atresia. Pediatr.Surg.Int. 20: 177-179, 2004
- Christensen AE, Qvist N, Husby S. Prolonged parenteral nutrition after neonatal gastrointestinal surgery. A Danish experience. Dan Med Bull. 49:244-247, 2002
- Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA: Intestinal atresia and stenosis. A 25-years experience with 277 cases. Arch.Surg. 133:490-497, 1998
- Federici S, Domenichelli V, Antonellini C and Domini. Multiple intestinal atresia with apple peel syndrome:successful treatment by five end-to-end anastomoses, jejunostomy and transanastomotic silicone stent. J Pediatr Surg. 38:1250-1252, 2003
- Fenster S, Brevoord JCD, Goldhoorn GA et al. Excellent long-term outcome for survivors of apple-peel atresia. J Pediatr Surg. 37: 61-65, 2002
- Fleet MS, de la Hunt MN. Intestinal atresia with gastroschisis: selective approach to management. J Pediatr Surg. 38:1323-1325, 2000
- Komuro H, Hori T, Amagai T et al. The etiologic role of intrauterine volvulus and intussusception in jejunoileal atresia. J Pediatr Surg. 39: 1812-1814, 2004
- Kumaran N, Shankar KR, Lloyd DA, Losty PD. Trends in the management and outcome of jejuno-ileal atresia. Eur J Pediatr Surg. 12: 163-167, 2002
- Louw JH, Barnard CN. Congenital intestinal atresia: observations on its origin. Lancet 2:1065-1067, 1955
- Mathai J, Sudipta S, Zachariah N, Chacko J, Thomas G. Proximal malecot vent in neonatal small-bowel anastomosis. Pediatr.Surg.Int. 19: 2452-46, 2003
- Namasivayam S et al. 180o rotated intestinal anastomosis for jejunoileal atresia in neonates-a preliminary study. Pediatr.Surg.Int. 18: 751-752, 2002
- Nishida S, Levi D, Kato T, Nery JR, et al. Ninety-five cases of intestinal transplantation at the University of Miami. J Gastrointest Surg. 6:233-239, 2002
- Patil VK, Kulkarni BK, Jiwane A, Kothari P, Poul S. Intestinal atresia: an end-to-end linear anastomosis technique. Pediatr.Surg.Int. 17;661-663, 2001
- Puri P, Fujimoto T. New observations on the pathogenesis of multiple intestinal atresias. J Pediatr Surg. 23:221-225, 1988
- Rowe MI, ed, O'Neill JA, ed, Grosfeld JL, ed, Fonkalsrud EW,ed, Coran AG, ed. Essentials of Pediatric Surgery. 1st ed. St.Louis, Mo: Mosby-Year Book Inc.: 1995
- Tandler J.Zur entwicklungsgeschichte des menschlichen duodenum in fruhen embryonalstadien. Morphol Jahrb. 29:187-216, 1900
- Todani T, Tabuchi K, Tanaka S. Intestinal atresia due to intrauterine intussusception: analysis of 24 cases in Japan. J Pediatr Surg.10:445-451, 1975
- Welch KW ed, Randolph JG ed, Ravich MM, ed, O'Neill JA Jr, ed, Rowe MI, ed. Pediatric Surgery, 4th ed. St Louis, Mo: Mosby -Year Book Inc: 1986.
|How to Cite URL :|
|S.Mironescu A, Muntean L, Popa E, Georgescu E. TOTAL JEJUNO-ILEAL ATRESIA-AN UNCOMMON CONDITION. Pediatric Oncall [serial online] 2007[cited 2007 May 1];4. Art #17. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=156&type=J&tid=&imgid=&reportid=147&tbltype=|