A planned ultrasound examination of a woman pregnant with twins at 25 weeks showed that one of the fetuses had a malformation – occipital meningoencephalocele of a large size, with the hernial sac measuring 10 × 12 cm.
To clarify the diagnosis and for further management of pregnancy, the woman from Kaliningrad was sent to the Perinatal Centre of Almazov National Medical Research Centre. Doctors of the specialized Perinatal Centre confirmed the presence of a giant occipital meningoencephalocele in the fetus.
This malformation is a combined anomaly of the brain and skull. Hernia of this size occurs in 1 in 5,000 births. Depending on the contents, there are several forms of cranial hernias. In the case of meningoencephalocele, it is a protrusion or herniation of brain (neural) tissue along with meninges through a defect in the cranium into a swelling filled with cerebrospinal fluid.
At 35 weeks, the woman was admitted to the antenatal department of the Perinatal Centre. The specialists could perform all the necessary examinations in advance and prepare the pregnant woman for successful delivery at 39 weeks by C-section.
Since the newborn had a giant cranial hernia, urgent surgery was necessary. It was dangerous to wait, not only because the brain tissue was outside the skull, the case was complicated by the fact that the substance was compressed in the area of the hernia orifice.
On the seventh day after birth, after a thorough examination and consultation with the leading specialists of Almazov Centre, A. Kim, head of Pediatric Neurosurgery Department No. 7 and neurosurgeon M. Nikolaenko performed a microsurgical repair of the malformation.
Two weeks after the surgery, the baby is progressing and gaining weight. Check-up examinations showed that the surgery was successful, there are no signs of concern and threat to the infant’s life. The patient with her mother will soon be discharged for further follow-up.
In 6 months, the baby will be admitted again to Almazov Centre for a comprehensive examination and further management.