Uterovaginal prolapse in a newborn with meningomyelocele: ca
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A baby girl weighing 3170 grams (40th percentile) with a congenital NTD was born to a 29-year-old mother by cesarean section at the 38th gestational week. She was admitted to the neonatal intensive care unit (NICU), and antibiotic treatment was initiated because the meningomyelocele sac had spontaneously perforated during labor. There was parental consanguinity. The mother also had gestational diabetes controlled by diet and had begun folic acid supplementation after the 6th week of gestation. Triple test screening results were high at the 18th week of gestation. The family refused amniocentesis and therefore no genetic counseling could be done during pregnancy.

A four cm meningomyelocele sac along with concomitant scoliosis was noted during the 21st week of gestation, and hydrocephalus was detected at the 33rd week of gestation. Medical termination of the pregnancy was offered but the parents rejected. The first examination of the baby showed general hypotonicity, a 4x5-cm meningomyelocele sac with concomitant severe scoliosis at the lumbosacral level, an occipitofrontal circumference of 36 cm (80th percentile) with a normotensive anterior fontanelle, bilateral rocker-bottom feet, and an absence of anal tone and deep tendon reflexes in the lower limbs. Cardiovascular, respiratory, abdominal, and genitourinary examinations were normal. Chest X-ray revealed multiple coastal anomalies.

Hydrocephalus and Chiari Type 2 malformation were confirmed with cranial ultrasonography. Echocardiography was normal. During the first postnatal 12–24 hours of life, a previously absent pink and fleshy protuberant mass was detected projecting out from the vagina (Figure 1). Abdominal ultrasonography revealed uterovaginal prolapse with concomitant, inferiorly displaced urinary bladder adjacent to the prolapsed tissues, hypoplastic left kidney, and minimal dilatation in the collecting system of the right kidney. Other possible causes of interlabial masses, such as urethral prolapse, ureterocele, para-urethral cyst, vaginal polyp, and rhabdomyosarcoma, were therefore excluded. The prolapse was manually reduced under general anesthesia by a pediatric surgeon with simple digital reduction on the 3rd day of life but then re-occurred on the 4th day of life.

The patient was scheduled to be followed-up without surgical intervention unless any circulatory insufficiency of the prolapsed tissue developed. Between the 4th and 14th days of life, the uterovaginal tissue demonstrated intermittent spontaneous reduction and re-prolapse without any improvement in or recovery of the defect. Routine care was given to the prolapsed tissue as coverage with moistened sponges and intermittent re-evaluation for possible circulatory disorders. On the 13th day of life, the patient underwent surgery performed by a neurosurgeon and a plastic surgery specialist for the NTD. To repair the large-sized dural defect, a flap shift procedure was carried out from the lumbosacral region to the midline.

A ventriculoperitoneal shunt was not placed at this time. The patient was placed in a supine position for the procedure and then followed-up in the NICU postoperatively. Routine care of the prolapsed uterovaginal tissue continued as before. On the 17th day of life, no postoperative complications were seen regarding the repair of the meningomyelocele and the uterovaginal prolapse, which had been intermittently and insistently seen before the repair of meningomyelocele, was no longer present. Because of the neurogenic bladder and vesicourethral reflux risk, amoxicillin prophylaxis was started, and the patient was discharged on the 18th day of life to outpatient follow-up. The patient was re-hospitalized on the 25th day of age due to cerebrospinal fluid (CSF) leakage from the meningomyelocele surgical scar and an accelerated increase in head circumference.

A ventriculoperitoneal shunt was placed on the 31st day of life to decompress the excessive CSF and lower the intracranial pressure. The patient was discharged again on the 34th day of life. During this period, the patient showed no sign of uterovaginal prolapse. Follow-up of the patient is routinely carried out at our outpatient clinics for possible neurological, developmental and urogenital problems.

Measurements in head circumference have shown a regular course after ventriculoperitoneal shunting with no clinical evidence of increased cranial pressure. The patient was guided to physiotherapy in order to preserve the neurological function of the lower extremities. Urine output and renal function tests were normal without a need for urinal catheterization. Voiding cystourethrography was normal at two months of age. Abdominal ultrasonography showed normal pelvic anatomy at three months of age. The patient is now four months of age and remains without any sign of uterovaginal prolapse.

Source:https://www.jpagonline.org/article/S1083-3188(20)30203-5/fulltext?rss=yes
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