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JIA: Jejunoileal Atresia

Def: an obstruction of the middle region


(jejunum) or distal region (ileum) of the small
intestine.
The small intestine immediately proximal to
the obstruction become enlarged or dialated.
Impairing ability to absorb nutrients
Impairing peristaltic movement of the small bowel
1) Definition of JIA and anatomic site that it
occurs, noting subtypes of this malformation if
applicable
JIA Type I:
Mesenteric development and
intestinal length are normal
Mucosa and submucosa forms a web or
membrane within the intestinal lumen
Windsock Effect
Increased pressure proximal to the obstruction causes
the web to prolapse, pushing the web through the bowel
distally
1) Definition of JIA and anatomic site that it
occurs, noting subtypes of this malformation if
applicable
JIA Type II:
Mesenteric development and
overall intestinal length is normal
Small bowel is not a continuous lumen
Proximal small bowel ends in bulbous blind pouch
Distal small bowel is flattened
Proximal and distal small bowel connected by
fibrous cord.


1) Definition of JIA and anatomic site that it
occurs, noting subtypes of this malformation if
applicable
Type IIIa:
Similar to Type II
Small V-shaped mesenteric defect
is present, bowel length is shortened
Proximal blind end is markedly dilated and
aperistaltic
No fibrous cord connects the proximal and distal
small bowel
1) Definition of JIA and anatomic site that it
occurs, noting subtypes of this malformation if
applicable
Type IIIb:
Similar to Type IIIa
Significant mesenteric defect is present
Superior mesenteric artery is largely absent
Small bowel supplied by a single ileocolic or right colic
artery
Known as Christmas tree or apple peel deformity
Bowel wraps around a single perfusing artery
1) Definition of JIA and anatomic site that it
occurs, noting subtypes of this malformation if
applicable
Type IV:
Multiple JIAs of any
combination Types I III
Appears as a string of sausages due to multiple lesions

Likely result of multiple ischemic insults or
inflammatory process
1) Definition of JIA and anatomic site that it
occurs, noting subtypes of this malformation if
applicable
1) Definition of JIA and anatomic site that it
occurs, noting subtypes of this malformation if
applicable
Intrauterine vascular accident Necrosis of
bowel segment Resorption of tissue
IVAs caused by malrotation, volvulus (abnormal
twisting of SMA), gastroschisis, omphalocele
All result in blood supply / gut segment separation

Duodenal Atresia is usually a failure to
recanalize versus vascular insult of JIA
2) Embryologic cause of this malformation; how
does the mechanism differ from duodenal atresia?
When does this malformation occur?
Within the first day of life
Vomiting green bile
Bowel sounds absent in distal small intestine
No meconium
Abdominal distention
3) Describe the typical clinical patient presentation
in JIA: symptoms, onset, age, physical exam
findings.
Prenatally
Ultrasonography will likely show excess amniotic fluid
(polyhydromnios)
Intestines arent properly absorbing ingested amniotic fluid

First day of life
Abdominal X-ray, with and w/o contrast enema
Stomach and proximal small bowel will appear dilated
Distal small bowel will appear flattened
4) Describe the recommended method for
diagnosing suspected JIA
5) Typical X-Ray findings in JIA
5) Typical X-Ray findings in JIA
Triple Bubble Presentation
Immediately a tube is placed orally into the
stomach to evacuate excess fluid and gasses
Prevents vomiting and aspiration
Relieves GI discomfort
IV Fluids and Nutrients are provided until
surgery is available
Surgical intervention is necessary to repair
the bowel obstruction and blood supply
6) Initial stabilizing treatment versus definitive
surgical treatment and prognosis in JIA.
After surgery, patients will have a feeding
tube until bowel movements begin
Typical hospital stay post-surgery is a few
weeks or until patient is nutritionally stable

Regular follow-up is required to check bowel
development and function
6) Initial stabilizing treatment versus definitive
surgical treatment and prognosis in JIA.
Types I, II, and IIIa have good prognosis
Fairly normal small bowel length results in almost
normal bowel function

Types IIIb, IV is associated with
complications
Shortened small bowel is associated with short
gut syndrome and malabsorption
6) Initial stabilizing treatment versus definitive
surgical treatment and prognosis in JIA.
References
Sadler, TW. Langmans Medical Embryology, 8
th
ed. 2000.

Intestinal Atresia, Stenosis, and Webs. May 1, 2006.
http://www.emedicine.com/ped/topic3043.htm. Accessed Nov. 26, 2007.

Bowel Obstruction in the Newborn. June 15, 2004.
http://www.emedicine.com/ped/topic2857.htm. Accessed Nov. 26, 2007.

Intestinal Atresia and Stenosis in Children. Nov. 27, 2007.
http://www.chp.edu/centers/03x_lit_disease_15.php. Accessed Nov. 27, 2007

Intestinal Atresia University of Michigan Pediatric Surgery. Apr. 23, 2007.
http://surgery.med.umich.edu/pediatric/clinical/physician_content/a-
m/intestinal_atresia.shtml. Accessed Nov. 26, 2007.

Intestinal Atresia and Stenosis, Cincinnati Childrens Hospital Medical
Center. Apr. 2007.
http://www.cincinnatichildrens.org/health/info/abdomen/diagnose/obstruction
s.htm. Accessed Nov 26, 2007.