weekly_question 30/4/2023

[Admin]

A newborn female, diagnosed at birth with cloaca without hydrocolpos, hydronephrosis or other associated malformation undergoes a proximal sigmoid colostomy and mucous fistula. At 6 months of age, she undergoes cystovaginoscopy and 3D fluoroscopic assessment of the cloaca which reveals a 1.5 cm common channel and a 2 cm urethra. Based on current recommendations, the most appropriate reconstruction for this patient would be:

A Vaginoplasty, introitoplasty and posterior sagittal anorectoplasty (PSARP)

B Total urogenital mobilization (TUM) and PSARP

C Laparoscopic TUM and PSARP

D Urogenital separation (UGS) and PSARP

E Anterior sagittal anorectoplasty

[Admin]

Correct answer
B Total urogenital mobilization (TUM) and PSARP

Cloacal malformations are rare, complex female anorectal malformations defined by the presence of a single perineal orifice with confluence of the urologic, genital and gastrointestinal tracts. Diagnosis is suspected at birth when there is no anal opening and introital assessment reveals a single perineal orifice. Pelvic and renal ultrasound are critical to the initial evaluation to exclude hydrocolpos, and secondary hydronephrosis resulting from reflux of urine into the vagina, which can be managed with intermittent catheterization, but may require vaginostomy. A completely diverting colostomy should be performed proximal enough to ensure distal length for both the rectal pullthrough as well as the possibility of using a short length of the sigmoid colon for a vaginal replacement if needed. Reconstruction should be delayed until at least 6 months and consideration should be given to referral to a center with pediatric colorectal expertise, especially those with a common channel > 2 cm. Patients with common channels < 1cm are amenable to PSARP with vaginoplasty, with the urethra left untouched.

Advances in cloacal reconstruction have evolved over the past 25 years, beginning with total urogenital mobilization (TUM). Until recently, expert guidance for treatment has been based on the cloacal anatomy defined by endoscopy and lateral or 3D reconstructed cloacagram assigning patients into treatment groups based on common channel length: 1-3 cm (short) and >3cm (long). For both groups, urogenital mobilization has been recommended, however with long common channels, modifications may be required when the mobilized urogenital tract does not reach the perineum.

The observation of suboptimal urinary continence (due either to leakage of urine or urethral loss) following cloacal reconstruction in a series of referred patients who had undergone TUM for longer common channels, led to the realization that urethral length was a critical predictor of urinary continence. This has led to a shift in emphasis in preoperative planning to consider urethral length independent of common channel length.

Therefore, current expert guidance recommends the preservation of a urethra of at least 1.5 cm based on the preoperative cloacagram (Figure 1). If the cloacagram demonstrates a urethral length of at least 1.5 cm, then TUM is a good choice. However, if the urethra is < 1.5 cm, the reconstruction should include urogenital separation (UGS), with the common channel becoming the neourethra following vaginal separation. Common channels > 3cm may require other procedural modifications, including transabdominal mobilization or vaginal replacement.

[Radwan suleiman abukarsh]

B

[Admin]

Correct answer
B Total urogenital mobilization (TUM) and PSARP

Cloacal malformations are rare, complex female anorectal malformations defined by the presence of a single perineal orifice with confluence of the urologic, genital and gastrointestinal tracts. Diagnosis is suspected at birth when there is no anal opening and introital assessment reveals a single perineal orifice. Pelvic and renal ultrasound are critical to the initial evaluation to exclude hydrocolpos, and secondary hydronephrosis resulting from reflux of urine into the vagina, which can be managed with intermittent catheterization, but may require vaginostomy. A completely diverting colostomy should be performed proximal enough to ensure distal length for both the rectal pullthrough as well as the possibility of using a short length of the sigmoid colon for a vaginal replacement if needed. Reconstruction should be delayed until at least 6 months and consideration should be given to referral to a center with pediatric colorectal expertise, especially those with a common channel > 2 cm. Patients with common channels < 1cm are amenable to PSARP with vaginoplasty, with the urethra left untouched.

Advances in cloacal reconstruction have evolved over the past 25 years, beginning with total urogenital mobilization (TUM). Until recently, expert guidance for treatment has been based on the cloacal anatomy defined by endoscopy and lateral or 3D reconstructed cloacagram assigning patients into treatment groups based on common channel length: 1-3 cm (short) and >3cm (long). For both groups, urogenital mobilization has been recommended, however with long common channels, modifications may be required when the mobilized urogenital tract does not reach the perineum.

The observation of suboptimal urinary continence (due either to leakage of urine or urethral loss) following cloacal reconstruction in a series of referred patients who had undergone TUM for longer common channels, led to the realization that urethral length was a critical predictor of urinary continence. This has led to a shift in emphasis in preoperative planning to consider urethral length independent of common channel length.

Therefore, current expert guidance recommends the preservation of a urethra of at least 1.5 cm based on the preoperative cloacagram (Figure 1). If the cloacagram demonstrates a urethral length of at least 1.5 cm, then TUM is a good choice. However, if the urethra is < 1.5 cm, the reconstruction should include urogenital separation (UGS), with the common channel becoming the neourethra following vaginal separation. Common channels > 3cm may require other procedural modifications, including transabdominal mobilization or vaginal replacement.

[Admin]

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[Admin]

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