8-Month-Old Male Patient Born with Anorectal Malformation

Khalid Shreef, M.D.
Professor, Pediatric Surgery, Zagazig University College Hospitals, Egypt

  • 8-mo-old male presents for evaluation. He was born with anorectal malformation s/p colostomy at birth. Baby presented with fever for 6 d, abdominal distension, bilious emesis, and no colostomy output for 3 d.
  • On examination, the patient was malnourished, pale, and dehydrated. Baby had significant abdominal distension with tenderness of the left lower abdomen.
  • A general surgeon performed a loop colostomy.
  • Baby had perineal hypospadias and bilateral cryptorchidism. He had a history of recurrent fevers for 6 mo before this case presentation with multiple hospital admission, but no definitive diagnosis of the source of fever.
  • Urinalysis was obtained that was normal.
  • Leukocyte count was high at 11. CRP 79.7.
  • BUN and serum creatinine were normal. Abdominal ultrasound revealed a cystic lesion (12 cm x 6 cm x 7 cm) extending from the pelvis to the upper abdomen.
  • Both kidneys had increased echogenicity with moderate hydronephrosis.
  • Distal loopogram showed the colon displaced by a large cystic lesion.
  • CT showed large cystic lesion with the bowel shifted to the right side of the abdomen.
  • Patient was taken to the operating room. A large cystic lesion was identified occupying the whole abdomen. It was attached to a bowel loop. The bowel loop was gently dissected to the cyst wall. A band was identified, which was later discovered to be an ectopic ureter connected to a small kidney surrounded by adhesions with hypoplastic renal vessels. This was a ureteric cyst connected to an atrophic kidney.
  • A left nephro-ureterectomy was performed. The histopathology showed dysplastic and disorganized muscle coat, which was lined with columnar epithelial mucosa, rather than with transitional epithelium.

Takeaways

  • Congenital segmental dilation of the ureter is rare in children. Only 12 cases are reported in the literature.
  • Congenital segmental dilation of the ureter is characterized by a giant, focal segmental dilation of the mid ureter and ureteric cyst formation. The histopathology typically shows the ureter is lined with columnar epithelial mucosa rather than the usual transitional epithelium.
  • With congenital segmental dilation of the ureter, the distal ureter may be normal, stenotic or atretic. The kidney sometimes shows duplicated collection system, hypoplastic, dysplastic or nonfunctioning kidney.
  • Treatment options for congenital segmental dilation of the ureter include anastomosis of the upper normal ureter to the contralateral ureter. This is anastomosed in an end-to-side fashion. Other options include excision of the diseased segment with an end-to-end uretero-ureterostomy. Nephro-ureterectomy may be indicated.
  • All newborns with anorectal malformation should have renal ultrasound to evaluate for renal anomalies.

Patient Case Discussion

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