A Multidisciplinary Approach to a Complex Anorectal Malformation and Urologic Anomaly

Abstract

Background:

Anorectal malformation (ARM) is a common congenital anomaly. The combination of a utricle cyst and an ARM is rare. The purpose of this video is to describe a multidisciplinary approach to the diagnosis and management of a complex anorectal and urologic anomaly.

Materials and Methods:

A 38-week gestation male infant had multiple genitourinary anomalies. These included distal hypospadias with the urethral opening situated just below the coronal sulcus, a bifid scrotum, and left renal agenesis. The testes had descended bilaterally and were palpable in the scrotum, which ruled out a disorder of sexual differentiation. He had a high imperforate anus and underwent a divided colostomy on day of life two. At 1 month, he developed a scrotal abscess; MRI demonstrated a pelvic cyst. A mucous fistulogram revealed the rectum terminated 4 cm cranial to an anal marker with a possible fistulous connection between the rectum, seminal vesicles, bladder, and the cystic structure. Retrograde urethrogram demonstrated that the cyst was connected posteriorly to the urethra, consistent with a utricle cyst. The rectum connected to the urethra but filled separately. At 3 months, the infant underwent combined endoscopy and cystoscopy to delineate the connections and attempt transurethral drainage of the utricle cyst. Endoscopy of the mucous fistula with a GIF-XP190N pediatric gastroscope (Olympus America Medical) allowed placement of a 0.035 × 150 cm guidewire (Cook Medical). The wire passed transurethral and allowed isolation of the mucous fistula and the utricle cyst. Because a wire was already in the mucous fistula, during cystoscopy with a 9.5F offset pediatric cystoscope (Richard Wolf Medical), the opening of the utricle cyst could be isolated. Each had a separate opening into the urethra. A 0.035 × 145 cm guidewire (Cook Medical) was placed into the utricle cyst and an 8F feeding tube (Bard Medical) was slid over the wire, to allow for transurethral drainage. The mother was trained to flush the cyst, which promoted resolution of the recurrent infections. Before the anal reconstruction, the transurethral drain dislodged but was not replaced. Instead the mucus fistula was flushed. The infant remained infection free before surgery.

Results:

After transurethral drainage and flushing, the utricle cyst appeared resolved. The infant underwent repair of his ARM at 7 months of life, 3 months after the initial drainage procedure. There was no evidence of the utricle cyst or previous infections during the pull through procedure.

Follow-up:

The infant is managed by a multidisciplinary team that includes pediatric urology. At present he is undergoing clinical examination and an ultrasonography once or twice annually and remains asymptomatic. His colostomy was reversed. Owing to his many genitourinary anomalies he is followed by pediatrics urology and will be followed into adulthood. Less than 40% of cysts become symptomatic. ¹ If intervention is required, most are cured by endoscopic procedures.

Conclusion:

A multidisciplinary approach allows for delineation and management of complex anorectal and urogenital anomalies with preservation of reconstructive planes. This video was presented at the 28th Annual Congress for Endosurgery in Children, March 22, 2019 in Santiago, Chile.

No competing financial interests exist.

Runtime of video: 4 mins 43 secs