JIA: Jejunoileal Atresia

An Intestinal Complication of Embryology

Interdisciplinary Clinical Cases November 29, 2007
Sub-Group Three

1) Definition of JIA and anatomic site that it occurs, noting subtypes of this malformation if applicable

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 Bowel wraps around a single perfusing artery

Known as Christmas tree or apple peel deformity

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

2) Embryologic cause of this malformation; how does the mechanism differ from duodenal atresia? When does this malformation occur?

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

3) Describe the typical clinical patient presentation in JIA: symptoms, onset, age, physical exam findings.

Within the first day of life

Vomiting green bile Bowel sounds absent in distal small intestine No meconium Abdominal distention

4) Describe the recommended method for diagnosing suspected JIA

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

5) Typical X-Ray findings in JIA

5) Typical X-Ray findings in JIA

Triple Bubble Presentation

6) Initial stabilizing treatment versus definitive surgical treatment and prognosis in JIA.

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

References

Sadler, TW. Langmans Medical Embryology, 8th 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/am/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.