Multiple atresia of the small intestine: a 20-year review.

Eur J Pediatr Surg. 2008; 18(1):13-8 (ISSN: 0939-7248)

Baglaj M; Carachi R; Lawther S

BACKGROUND: Multiple intestinal atresia (MIA) presents with a wide spectrum of bowel pathologies. Its
treatment is a challenging task since restoration of anatomical continuity of the affected intestine must be
balanced against preservation of the intestine's maximal length.

MATERIAL AND METHODS: A retrospective analysis of the medical notes of 26 patients with MIA treated
over a 20-year period between 1986 - 2006 was undertaken with a special emphasis on the clinical and
surgical perspectives.

RESULTS: All 26 cases of MIA were sporadic with no familial history. The mean gestational age and birth
weight were 36.1 weeks and 2781 g, respectively. Twenty-three of the infants underwent operative repair
within the first days of life. Three patients with gastroschisis had a delayed diagnosis of bowel atresia. The
number of atresias per patient ranged from 2 to 10. In 24 newborns atresias were confined to the small
bowel, with 2 other patients having additional obstruction of the ascending colon. Various combinations of
anatomical types of atresias were found, with type I and type III occurring in 19 patients each. Type II was
diagnosed in 7 newborns. Surgical management of MIA consisted of one-stage restoration of bowel
continuity with multiple anastomoses and/or enteroplasties in 22 patients. Four patients had an
enterostomy performed at initial operation. Early and late postoperative complications requiring operative
treatment occurred in 8 patients. The duration of parenteral nutrition ranged from 6 days to 20 months,
exceeding 100 days in 6 children. The follow-up ranges from 3 months to 16 years. All the patients are
alive and are on a full oral diet.

CONCLUSION: Clinical observations of sporadic cases of MIA confined to the small bowel lend support to
the hypothesis of a vascular incident etiology. One-stage restoration of intestinal continuity with
preservation of maximal intestinal length should be the basic principle of any operative management of
MIA. Despite a relatively high morbidity related to the primary damage of the fetal intestine, excellent
results with 100 % survival rates can be obtained. After taking the differences in pathogenesis, anatomical
and histological features, and the prognosis for sporadic and hereditary forms of MIA into account, these
two entities should be classified separately in a modified classification of intestinal atresia.
Neonatal outcomes associated with intestinal abnormalities
diagnosed by fetal ultrasound.
J Pediatr Surg. 2009; 44(1):71-4; discussion 74-5 (ISSN: 1531-5037)
Ruiz MJ; Thatch KA; Fisher JC; Simpson LL; Cowles RA

PURPOSE: Intestinal abnormalities are sometimes seen during antenatal testing; however, the postnatal
importance of these findings has not been well established. We evaluated whether abnormal intestinal
appearance on fetal ultrasound (US) was ultimately related to neonatal outcome.

METHODS: Fetal US examinations from 2003 to 2006 were evaluated. Hyperechogenic bowel was defined
as having the echogenicity comparable to bone, and dilated bowel was identified based on the
sonographer's assessment. Persistence or resolution of US findings on subsequent US examinations and
eventual outcomes were assessed. Cases were categorized as hyperechogenic or dilated and then
subgrouped based on whether the US finding resolved.

RESULTS: Sixty-eight fetuses had either hyperechogenic (n = 48) or dilated bowel (n = 20) on antenatal
US. In 56 cases, complete data were available for analysis. Of 44 liveborn infants, 11 (25.0%) had an
abdominal abnormality, and 33 (75.0%) were normal at birth. Compared to those with dilated bowel,
fetuses with hyperechogenic bowel had a higher rate of prenatal demise (20.8% vs 10%) but a lower rate of
abnormality at birth (10.3% vs 53.3%). Hyperechogenic bowel resolved on subsequent US more frequently
than dilated bowel (65.5% vs 20.0%). In both groups, all fetuses with sonographic resolution were normal
at birth. Of 9 fetuses that had persistently hyperechogenic bowel, 3 (33.3%) were born with an abnormality,
and all were found to have meconium peritonitis or meconium ileus. In the 12 cases where dilated bowel
did not resolve, 8 (66.7%) were eventually born with an abnormality, most commonly intestinal atresia.

CONCLUSIONS: Hyperechogenic and dilated bowel are associated with a significant rate of fetal demise.
Hyperechogenicity is more common than dilation and is more likely to be transient. Dilated bowel is more
often associated with neonatal abnormality than hyperechogenic bowel. Persistence of fetal US findings
predicts a higher likelihood of abnormality in the neonate.
Multiple intestinal atresias. Report of 2 cases].
Chir Pediatr. 1989; 30(2):61-4 (ISSN: 0180-5738)
Aigrain Y; Enezian G; Sonsino E; Boige N; Kazandjian V; Boureau M

We report two new cases of multiple gastrointestinal atresias as described by Guttman and al in 1973. The
clinical presentation is characteristic: the newborn presents himself with a neonatal occlusion. Plain
abdominal film shows gas in a distended stomach with intraluminal calcifications. At laparotomy in both
cases a diaphragm was present at the pyloric region followed by innumerable diaphragms only a few
centimeters apart until the rectum in the first case, and the right colon in the second one. Reconstruction of
the intestinal lumen was impossible and the babies died due to sepsis. Pathologic finding in both cases
were those of numerous intraluminal diaphragm associated in the first cases with types II atresias. There
was no normal epithelium from the stomach to the rectum. This strongly supports the hypothesis of a
malformation of the gastrointestinal tract occurring during early fetal life. The possibility of an autosomic
recessive transmission of the process was outlined by Guttman
Intestinal atresias: factors affecting clinical outcomes.
J Pediatr Surg. 2008; 43(7):1244-8 (ISSN: 1531-5037)
Piper HG; Alesbury J; Waterford SD; Zurakowski D; Jaksic T

BACKGROUND: Despite improvements in care, intestinal atresias are associated with prolonged
hospitalization and occasionally mortality. Although each type of atresia is distinct, it is unclear which
factors impact clinical course. This study seeks to identify predictors of untoward outcome.

METHODS: Neonates with duodenal, jejunal/ileal, and colonic atresia, treated at 1 institution from 1982 to
2005 were reviewed. Data were evaluated using nonparametric analysis of variance and logistic
regression. Nonparametric data were expressed as medians with interquartile range (IQR).

RESULTS: A total of 132 infants were evaluated including 63 with duodenal, 60 with jejunal/ileal, and 9 with
colonic atresias. Overall mortality was 7% with associated congenital anomalies identified as an
independent risk factor (P = .01). Infants with associated anomalies were more likely to have low birth
weight (2.3 +/- 0.8 vs 3.0 +/- 0.8 kg, P = .01), which further increased mortality risk. Atresia location did not
affect mortality or length of stay; however it did impact the time to full enteral nutrition with jejunal atresia
requiring longer than duodenal (17 [IQR, 9-40 days] vs 10 [IQR, 7-70 days]; P = .01).

CONCLUSION: Overall mortality from intestinal atresia is low and is not dependent on the location of
obstruction. Infants with birth weight less than 2 kg and associated anomalies are at an increased risk for
prolonged hospital stay and mortality.
A proposed classification system for familial intestinal atresia and
its relevance to the understanding of the etiology of jejunoileal
atresia.
J Pediatr Surg. 2006; 41(11):1822-5 (ISSN: 1531-5037)
Shorter NA; Georges A; Perenyi A; Garrow E

Familial cases of the various types of intestinal atresia are well described, and we now report an additional
family. Based on a review of the literature, a classification system for the different types of familial atresia is
presented. Current teaching attributes most jejunoileal atresias to in utero vascular accidents occurring
relatively late in gestation (after the 11th or 12th week). Although some cases clearly occur this way, as a
result of processes such as volvulus and intussusception, knowledge of the familial form of the disease
indicates that most cases of jejunoileal atresia actually result from disruption of a normal embryologic
pathway, most likely the development of the superior mesenteric artery and its branches. They should be
considered to be true embryologic malformations rather than acquired lesions.
Duodenal atresia and stenosis: reassessment of treatment and
outcome based on antenatal diagnosis, pathologic variance, and
long-term follow-up.
World J Surg. 1993; 17(3):301-9 (ISSN: 0364-2313)
Grosfeld JL; Rescorla FJ

Duodenal atresia and stenosis was observed in 103 infants and children from 1972 to 1991. There were 59
girls and 44 boys. Atresia was noted in 79 instances and stenosis in 24. Maternal hydramnios was detected
in 33 cases, 46 babies were premature, and 31 had Down's syndrome. Fifty-four infants had significant
associated anomalies including 35 with cardiac defects. Diagnosis was achieved by prenatal ultrasound
examination in 14 cases, observation of a double-bubble sign on abdominal radiograph in 73, and contrast
studies in 30 infants including 24 with stenosis. At operation annular pancreas was noted in 37 cases,
malrotation in 37 cases, anterior portal vein in 4, and a second web in 3. Surgical treatment included
duodenoduodenostomy in 85, duodenotomy and web excision in 8, and duodenojejunostomy in 10.
Operative survival was 95%. Deaths were related to complex cardiac defects. Despite antenatal diagnosis,
prompt intervention, and apparent early surgical success (95% survival), late deaths (5%) and late
complications including motility disorders, megaduodenum, gastroesophageal reflux, duodenal-gastric
reflux, gastritis, peptic ulcer disease, blind loop syndrome, and biliary-pancreatic conditions may be
observed months to years after management during the neonatal period. Modifications in surgical
technique including early tapering duodenoplasty may be useful, and close long-term follow-up is an
essential component of patient care.
Etiology and treatment of small intestinal atresia:
Analysis of a series of 127 jejunoileal atresias and
comparison with 62 duodenal atresias
H.H. Nixon
,
R. Tawes
Abstract
Pathological findings and rarity of anomalies in other systems support an origin of jejunoileal atresia in a late fetal

accident to the bowel; incarceration of the physiological umbilical hernia may be a common cause. This contrasts
with duodenal atresias which probably arise earlier and have many serious associated anomalies.

In jejunoileal atresia, the prognosis improves with more distal atresias. “Treatment” grouping as well as “risk”
grouping is offered.

Elective resection of proximal bowel has improved results, but anastomotic problems continue in the smaller
babies and an alternative technique should be sought.

Blind pouch formation with a stasis syndrome has occurred after oblique end-to-end anastomosis.

Familial incidence of the distinct “apple peel” group of atresias is noted.

The value of the ileocecal valve is discussed, and a healthy survival with only 22 cm. of jejunum and no ileocecal
valve is reported.

Duodenal atresia results have improved with gastrostomy decompression and trans-anastomotic feeding.

Associated anomalies and extremely low birth weight are the main problems in treatment, not anastomotic
technique.
Intestinal atresia.
Indian J Pediatr. 2000; 67(9):671-8 (ISSN: 0019-5456)
Prasad TR; Bajpai M

Intestinal atresia accounts for about one third of all cases of neonatal intestinal obstruction. The survival
rate has improved to 90% in most of the series with the operative mortality being < 1%. The survival rate
improves with distal atresias. An increased mortality is observed in multiple atresias (57%), apple peel
atresia (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%) and
gastroschisis (66%). Although appearance of echogenic bowel on prenatal ultrasonography is suggestive
of GI, it is confirmed in only 27% cases. Prenatal ultrasonography is more reliable in detection of duodenal
atresia than more distal lesions. Short bowel syndrome is the major impediment in the management of
jejunoileal atresia. Although total parenteral nutrition (TPN) is the main adjunctive treatment, it delays
intestinal adaptation and may cause cholestasia and subsequent liver damage. Graduated enteric
feedings, use of growth hormone, glutamine and modified diets containing low fat, complex carbohydrates
and protein supplements have been used in a adults with short bowel syndrome to successfully diminish
TPN requirements and enhance nutrient absorption in nearly half of the patients. Utilization of growth
factors to facilitate intestinal adaptation and advances in small bowel transplant may improve the long-term
outcomes in future.
Decreased mortality but increased morbidity in neonates with
jejunoileal atresia; a study of 114 cases over a 34-year period.
J Pediatr Surg. 2009; 44(1):217-21 (ISSN: 1531-5037)
Stollman TH; de Blaauw I; Wijnen MH; van der Staak FH; Rieu PN; Draaisma JM; Wijnen RM

PURPOSE: The aim of the study was to evaluate patient demographics, classification and location of the
atresia, operative management, postoperative care, and outcome in 114 infants with jejunoileal atresia
(JIA) over a period of more than 3 decades.

METHODS: This was a retrospective case series in a tertiary care teaching hospital. Records of all patients
with JIA treated at the authors' institution between 1971 and 2004 were examined.

RESULTS: Sixty-two percent of atresia and stenosis was noted in the jejunum, 30% in the ileum, and 8% in
both the jejunum and the ileum. Atresias and stenosis were classified as follows: 7% type 0, 16% type I,
21% type II, 24% type IIIa, 10% type IIIb, 22% type IV. Gastrointestinal anomalies were encountered in
24% of patients, genitourinary malformations in 9%, cystic fibrosis in 9%, neurologic anomalies in 6%, and
congenital heart disease in 4%. Operative management included resection with primary anastomosis in
69% of all patients and temporary enterostomies in 26%. After operative management, 15% of children had
resultant short bowel syndrome. Oral feeding was allowed on median day 7, and full energy expenditure via
the enteric route was reached on median day 20. Forty-seven percent of infants required central venous
line placement for total parenteral nutrition. Early postoperative complications occurred in 28% of patients
with JIA and late postoperative complications in 17%. We observed a mortality rate of 11%.

CONCLUSIONS: This is one of the largest series of neonates with JIA described. Short bowel syndrome
seems to be the biggest problem resulting in longer hospital stay, more feeding problems, and higher
morbidity and mortality rates. Management of children with short bowel syndrome has improved because of
the use of total parenteral nutrition, new operative techniques, and better intensive care. In the last 15
years, survival has increased at the cost of the surviving children as we noted a higher percentage of late
complications.