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2013-06-23-10 Answer to the case of the week #351 © Al-Asali www.TheFetus.net

Answer to the case of the week #351
 
August 15, 2013 - August 29, 2013 
 
Othman A. R. M. Al-Asali, MD; Fayez Yassen, MD.

Department of Obstetrics and Gynecology, Al-Hammadi Hospital, Riyadh, Saudi Arabia.
 
Case report
This is a 29-year-old woman (G3P2) from a non-consanguineous marriage, with non-contributive history. She had attacks of hypoglycemia over the last year, associated with low fasting glucose and high insulin levels, managed by diet. 

The patient underwent amniocentesis at 18 weeks revealing normal karyotype - 46XY.

At 23 weeks the patient was hospitalized due to diarrhea related to Entamoeba histolytica
 infection and was placed on Flagyl (metronidazole).

Her ultrasound examinations performed at 24 and 28 weeks of gestation did not show any fetal anomaly. 

At 34 weeks ultrasound examination revealed dilated bowel loops measured 24.4 mm in the largest diameter. The finding raised suspicion of ileal atresia.

At 37 weeks the dilated bowel loops measured 19.0 mm in the largest diameter and a small ascites with distended abdomen (abdominal circumference corresponded to 39 weeks) were also noticed.

Cesarean section was performed at 37 weeks due to fetopelvic disproportion (fetal biometry corresponded to 39 weeks) and a male newborn weighing 3230 grams (Apgar score 9, 10) was delivered. Amniotic fluid was stained by old and fresh meconium.

Postnatal abdominal ultrasound and X-ray were done, but revealed no anomaly. No vomiting or abdominal distension were present and normal meconium discharge was noticed. 

The newborn was placed at neonatal unit with usual care and feeding. At 6th day after delivery the neonate was discharged home.

At 9th day the neonate had to be admitted at NICU (Neonatal Intensive Care Unit) because of vomiting, abdominal distension, and poor feeding. In our differential diagnostic contemplation we were thinking of:

1 - sepsis, 
2 - Intestinal obstruction (saccular ileal dilatation, congenital segmental dilatation of the ileum), 
3 - Hirschsprung disease.
 
Abdominal X-ray was done revealing:
 
Distended bowel loops at the upper abdomen with multiple fluid levels within the small bowel loops;
No free air under the diaphragm;
No intraabdominal calcifications.
 
At 10th day contrast X-ray with Gastrografin applied via nasogastric tube and by enema was performed and found:

Free passage of Gastrografin into the stomach with no evidence of gastric filling defect, however a mild degree of gastric malrotation was noted;
Normal filling of the duodenum and proximal jejunal loops;
In the late films, dilatation of the ileum, as well as the distal segment of the jejunum were seen (this was noted to be present till the last film taken 5 hours post contrast injection).

At 11th day the neonate is operated and excision biopsy of the dilated loop of intestine followed by ileostomy was done.
 
  • Gross appearance:  Specimen consists of a loop of small intestine measuring 21 x 2.5 cm. It shows one area of dilatation measuring 5 cm in longitudinal axis of the small intestine, and the area is 6 cm from the upper resection margin and 10 cm from the lower resection margin. The mucosa shows green tinged surface. Representative sections is taken for histological processing
    • Dilatation area;
    • Upper resection margin;
    • Lower resection margin.
  • Histology:  The examined slides revealed a loop of small intestine showing, more than 12 ganglion cells (per field X40) in the muscle layer, and congested hemorrhagic blood vessels in the wall. A mild inflammatory infiltrate is also seen, in the lamina propria. There is no evidence of malignancy.
  • Diagnosis:  Resection of loop of small intestine shows saccular dilatation and congestion related to Congenital Segmental Intestinal Dilatation
Images 1, 2, 3, and 4: 34-37 weeks - the images show dilated bowel loops of the fetus.

 

 

Images 5, 6, and 7: 37 weeks - the image 5 shows small amount of ascites among bowels. The image 6 shows enlarged abdominal circumference of the fetus corresponding to 39 weeks. Image 7 shows normal amount of the amniotic fluid.

 



Images 8, 9: Contrast X-ray with Gastrografin applied via nasogastric tube at 10th day after delivery showing saccular dilatation of the ileum.

 

Images 10, 11: Image 10 - perioperative image showing dilated part of the ileum. Image 11 - histological specimen of the dilated segment of the ileum.

 

Discussion
 
Neonatal bowel obstruction as a result of segmental dilatation of the gut is a very rare disorder with limited published cases [1–3]. Segmental dilatation of the ileum is a rare condition, with only 126 published cases [3]. The anomaly may present as an isolated entity (as it was in our case) or may be associated with other congenital malformations, gastrointestinal bleeding, anemia, abdominal pain, malabsorption, and growth failure in older children [4,5].

Congenital segmental intestinal dilatation was first described by Sjolin and Thoren from the Departments of Pediatrics and Surgery, University Hospital, Uppsala, Sweden, 1962 [7]. The condition is characterized by a sharply defined and markedly dilated segment of the gut flanked by normal caliber of the afferent and efferent bowel. During neonatal period the anomaly presents with acute intestinal obstruction or simulates Hirschsprung disease (vomiting, abdominal distention, constipation), in older infants may manifest with anemia, malabsorption, intestinal bleeding caused by ulceration of heterotopic gastric mucosa, chronic constipation, or features of intermittent intestinal obstruction.

Although the same anomaly may affect small bowel or colon, clinical sings and time of presentation may differ according to the site of involvement. Clinical picture of cases involving the colon is very similar to that of Hirschsprung disease, usually appearing in the infancy or later on. 

Segmental intestinal dilatation is an exceptional pathology with an unknown etiology and a misleading clinical presentation. Several theories were proposed to explain this malformation; however, most authors are rather inclined to an embryological theory incriminating an extrinsic intrauterine intestinal compression. Pathological examination shows a bowel wall with all layers as well as a normal ganglion cells. Surgical resection of dilated intestinal segment is curative.

Segmental dilatation of the intestine should be considered as a cause of intestinal obstruction in a neonate with antenatal discovery of intraabdominal cystic lesion. Lack of specific of radiologic findings explains difficulties to postulate an exact preoperative diagnosis, however, favorable evolution after surgical resection of the dilated segment, points out to the diagnosis of the congenital segmental intestinal dilatation.

In our case the emergency surgical abdominal exploration leaded to final solution of the diagnostic dilemma. Routine prenatal sonographic examination at 34 weeks of gestation revealed a thin-walled dilatation of bowel loop which initially raised suspicion of ileal atresia. At birth the newborn passed copious amount of meconium and no clinical signs of intestinal obstruction were observed. Initial postnatal X-ray did not find any signs of intestinal obstruction either. Oral feeding was started and the neonate was discharged home. Poor feeding and abdominal distension had developed later and the neonate had to be admitted to the hospital nine days after delivery. Subsequent contrast X-ray found dilated segment of jejunum and surgical exploration was recommended. During the surgery a dilated, sharply demarcated, thin-walled segment of the small bowel was found and resected. Histological examination found normal mucous membrane and ganglion cells within the dilated segment.

The case represents a rare neonatal functional intestinal obstruction of unknown etiology. The anomaly often presents itself as an isolated dilated small bowel segment, without evidence of intrinsic or extrinsic cause of obstruction or abnormal neurologic innervations.

Mathé JC et al [8] in their study of the myenteric plexus with a silver staining preparation, explains the anomaly as a consequence of reduction in the number of argyrophil neurons and irregular and altered neuron cell bodies
 
References
 
1. Saha S, Konar H, Chatterjee P, Basu KS, Chatterjee N, Thakur SB, Dasgupta A. Segmental ileal obstruction in neonates--a rare entity. J Pediatr Surg. 2009 Sep;44(9):1827-30.
2. Ojha S, Menon P, Rao KL. Meckel's diverticulum with segmental dilatation of the ileum: radiographic diagnosis in a neonate. Pediatr Radiol. 2004 Aug;34(8):649-51.
3. Waters KJ, Levine D, Lee EY, Buonomo C, Buchmiller TL. Segmental dilatation of the ileum: diagnostic clarification by prenatal and postnatal imaging. J Ultrasound Med. 2007 Sep;26(9):1251-6.
4. Shah AD, Kovanlikaya A, Beneck D, Spigland N, Brill PW. Segmental dilatation of the ileum in a healthy adolescent. Pediatr Radiol. 2009 Dec;39(12):1350-3. 
5. Eradi B, Menon P, Rao KL, Thapa BR, Nagi B. Segmental dilatation of ileum: An unusual cause of severe malnutrition. Pediatr Surg Int. 2005;21:405–6.
6. Herlinger H, Maglinte DD. Philadelphia, PA: WB Saunders Co; 1989. Congenital and developmental anomalies in adolescents and adults.Clinical Radiology of the Small Intestine; pp. 249–73.
7. Sjolin S, Thoren L. Segmental dilatation of the small intestine. Arch Dis Child. 1962 Aug;37:422-4.
8. Mathé JC, Khairallah S, Phat Vuoung NP, Boccon-Gibod L, Rey A, Costil J. Segmental dilatation of the ileum in a neonate. Study of the myenteric plexus with a silver staining preparation. Nouv Presse Med. 1982 Jan 30;11(4):265-6. French.
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