1. Neonatal Management of Jejunoileal Atresia
Andrew L Mesher MD
Resident, Dept of General Surgery
University of Washington School of Medicine
September 9, 2010

2. CASE 36+0 week gestation, 1.9kg SGA neonate, DOL#1
Notable abdominal distention at birth
150cc of meconium/bile aspirated from stomach after birth
No passage of meconium
Ambiguous genitalia, systolic murmur
Birth/Prenatal History: NSVD, Prenatal U/S - IUGR, polyhydramnios

3. Physical Exam Gen: Intubated, NG draining bilious thin liquid, SGA,
Abdomen: No significant distension, soft, no abdominal wall defects, no masses,
GU: Non-fused labioscrotal folds, hypospadias at base of microphallus
Rectal: Sacral dimple, patent anus

5. Neonatal Obstruction Functional ie Hirshsprungs
Meconium ileus, plug, perforation
Internal hernia
Malrotation with midgut volvulus
Intestinal atresia/stenosis
Duodenal
Jejunal
Ileal
Colonic

6. Evaluation Plain Radiographs Upper GI, w/wo SBFT Contrast Enema
CF genetic tests
Suction rectal biopsy
Sweat chloride test

7. Evaluation Plain Radiographs Contrast Enema Upper GI, w/wo SBFT
Suction rectal biopsy
CF genetic tests
Sweat chloride test

8. Evaluation Plain Radiographs Upper GI, w/wo SBFT Contrast Enema
JIA: dilated loops of proximal bowel with air and fluid, absence of distal gas, “string of pearls” of calcified meconium with multiple atresias
DA: double bubble sign
Inspissated meconium: ground glass
Upper GI, w/wo SBFT
Malrotation: location of DJ junction,
Intestinal atresia/stenosis: stricture
Contrast Enema
Malrotation: Location of cecum
Colonic atresia: Does contrast reflux past IC valve
Total colonic aganglianosis: calibur of colon, reflux in SB

11. Key Features Third Trimester Polyhydramnios 150cc gastric volume
Failure to pass meconium <24h
No significant abd. distension after 12h of NG decompression
Dilated loops of proximal SB with complete absence of distal gas
BE with reflux past IC valve and cecum in RUQ

12. Type IIIb Jejunal Atresia
Courtesy of Dr. Jacob Stephenson, Dr. Patrick Healey SCH 2010

13. Jejunal Atresia Incidence 1 in 5000 live births Presentation:
Bilious emesis on first day of life, but may be delayed until 2nd or 3rd day in 20%
Abdominal distension, more pronounced with distal lesions
Failure to pass meconium within 24h
Prenatal U/S may show 3rd trimester polyhydramnios, less common with distal lesions

14. Stollman et al J. Pediatr Surg 2009

15. Pathogenesis In utero ischemic accident Hereditary Component
Louw and Bernard in utero ligation of SMA in dog recapitulates atretic segments
Internal hernias, malrotation with volvulus, intussusception, atresia with gastroschisis, thrombosis
de Lorimier 1969: evidence of bowel infarction in 42% of JIA
Hereditary Component
Autosomal recessive pattern of inheritance of multiple intestinal atresia syndrome
Multiple case reports of siblings with IIIb jejunal atresia

16. Perioperative Management
Decompression with NG/OG tube
Fluid resuscitation
Correction of metabolic disturbances
Preoperative antibiotics
Contrast enema preoperatively to identify concomitant colonic atresia ~4%

17. Operative Approach Small transverse supraumbilical laparotomy
Banieghbal et al 2007: minimal access approach via circumbilical incision - successful 15/16
Evisceration
Gram stain and culture of intraperitoneal fluid
Laparoscopic approach has not been described in literature

18. Operative Considerations
Primary goal - preservation of intestinal length >80cm with IC valve
Resection of bulbous, hypertrophied proximal bowel with primary end-to-end single layer reanastamosis
Primary enterostomy is avoided (increased sepsis, length of HA, rates of reoperation) but may be unavoidable in cases of bowel necrosis, peritonitis.
Waldhausen and Sawin 1997: IIIb - Mean time to full enteric feeds 46+/-30d, suggests routine gastrostomy tube placement

19. Type IIIb Considerations
High jejunal atresia may make complete resection of bulbous bowel impossible
Resection into second portion of duodenum with derotation has been proposed
Division of mesenteric bands along apple- peel is important to ensure maximal perfusion
Careful return of narrow mesentary and vascular pedicle to prevent kinking

20. Anastomotic Considerations
Proximal dilated bowel - dysfunctional peristalsis, ganglion cell dysfunction, high wall tension - creates functional obstruction
Size disparity between proximal and distal segments
Techniques:
Tapering enteroplasty with GIA stapler
Antimesenteric plication w/wo seromuscular strip
End to oblique anastamosis

21. Anastamotic techniques

22. To taper or not to taper
Technique remains individualized and surgeon dependent
No new data, certainly no randomized data regarding functional outcomes
Weber 1982: 162 infants, 35% tapering or plication - successful w/o anastamotic leaks in all 65. 4/7 infants with plication suffered breakdown requiring reoperation. None with seromuscular strip.

23. Prognosis Prior to 1950 - Mortality 50-90%
Currently - Survival 85-90%, due to improved operative technique, perioperative management, advent of hyperalimentation
Morbidity is associated with short-bowel syndrome and TPN-associated complications

24. Conclusions JIA is a common cause of neonatal obstruction
Prompt recognition with early operative management is key
Resection and primary reanastamosis should be attempted
Avoidance of primary enterostomy
Prognosis is excellent, but morbidity associated with short bowel syndrome/TPN
Large animal model with in utero ligation of SMA may be used to evaluate tapering enteroplasty prospectively