Decision making in Pediatric Colorectal Surgery - A webinar of case discussions

Cystoscopy is the first diagnostic step to determine whether the anterior orifice is a urethra (vaginal atresia) or a urogenital sinus.

If the anus is normal, you have to be worried about a urogenital sinus with a potential endocrine, particularly adrenal problem.

If the anus is abnormal or there is no anus at all, you need to think about a cloaca.

Patients with two perineal orifices have been determined to be DSD cases in the newborn period and left for some time without a gender assignment.

Prepubertal MRI is not useful for evaluating gynecologic anatomy because the structures are very small and difficult to see.

In distal vaginal atresia, the most common setup is normal ovaries, fallopian tubes, and nothing else—no uterus and no vagina.

The second most common scenario in distal vaginal atresia is normal ovaries, fallopian tubes, and an upper vagina that would not possibly reach the perineum but might fill with menstrual blood.

The least common scenario in distal vaginal atresia is truly a distal vaginal atresia, and in some cases you can make that reach with a laparoscopic approach.

If there is an upper vagina in distal vaginal atresia, it has to have a way to get out, and mobilizing the distal vagina laparoscopically and sewing it to the perineum gives a very nice functional and cosmetic result.

If a normal anus is present, you need to suspect urogenital sinus and investigate adrenal problems by checking electrolytes.

If the anus is abnormal, you need to suspect most likely the presence of a cloaca.

In distal vaginal atresia, the most frequent internal anatomy is normal ovaries and fallopian tubes but absent uterus and absent vagina.

The second anatomical option in distal vaginal atresia is normal ovaries and fallopian tubes with uterus and only the upper third of the vagina.

The third and most uncommon option in distal vaginal atresia is a true distal vaginal atresia.

If there is no hydrocolpos and no risk to the urinary tract, you can wait until the patient is pre-pubertal for surgery.

If there is no uterus in distal vaginal atresia, there is no rush for surgery because there will most likely be no menstrual blood and no risk for the patient.

If there is a uterus and a vagina that is atretic, you need to think of a vaginostomy initially, and then consider a pull-through or interposition sooner rather than later.

Very often you don't need to do a vaginostomy; not infrequently you can do intermittent catheterization of the common channel.

If doing intermittent catheterization of the common channel, you need to prove by ultrasound that the catheter is going into the hydrocolpos and decompressing it, and that hydronephrosis is improving.

In many patients with hydrocolpos, the urethra takes a very sharp turn anteriorly, so the catheter preferentially goes into the hydrocolpos.

If there is a true distal vaginal atresia, intermittent catheterization will not work; you have to relieve hydronephrosis by draining the hydrocolpos via interventional radiology or laparoscopy.

A pigtail catheter is recommended for hydrocolpos drainage because as the hydrocolpos recedes it pulls away from the abdominal wall and a regular tube can fall out, but a pigtail won't.

Before removing a pigtail catheter used for hydrocolpos drainage, it is a great opportunity to do a contrast study and see exactly what anatomy you are dealing with.