The Prenatal screening for neurological defects includes the measurement of maternal serum alpha-fetoprotein (MSAFP) and high-quality second-trimester ultrasound to detect fetal anomalies. Meningocele presents as a swelling over the back covered with skin, present at birth. Defects can present at thoracolumbar, lumbosacral, lumbar, thoracic, sacral, and cervical regions. Neurological involvement and deficits are rare in meningocele. On clinical exam, a fluctuant midline mass that transilluminates is present.

The initial assessment of the newborn is essential at birth. A detailed examination of the head and neck, including the head, skull bones, and anterior fontanelle's size and shape, is done. A complete neurological exam shall be done at birth to exclude any neurological abnormalities. The clinical manifestation of the thoracic meningocele is approximatelyrelated to its size and proximity with the surrounding structures. They can present with back pain, shortness of breath, coughing, and palpitations when accompanied by the lung and mediastinal structure compression.[2][9]

All patients must be assessed radiologically after birth, depending on their clinical status. Plain X-rays films show any associated skull defects, bone abnormalities, and deformities of the spine. Magnetic resonance imaging (MRI) is the best diagnostic test to study the neural tissue abnormalities and assess any associated hydrocephalus. Ultrasonography to have a quick assessment of hydrocephalus can be helpful. Surgery should be on board to evaluate the need for emergent surgical repair.[2]

Management is mainly a multidisciplinary approach. It is vital to counsel family regarding the immediate and long-term effects of treatment. Surgery is preferred as soon as it is practical on large meningoceles. In suspected central nervous system infection or meningitis, treatment is started with prophylactic antibiotics and anticonvulsants immediately. Patients require monitoring in the neonatal intensive care unit with an assessment of electrolytes and blood counts. Consider blood grouping and cross-match for possible transfusion.

The association of meningocele with tethered cord syndrome is high. MRI is essential for the diagnosis of meningocele. Active surgical treatment is suggested immediately after definite diagnosis. During surgery, the surgeon should explore the spinal canal and release the tethered cord after repairing the protruded meninges.[2][3]

Encephalocele attributes to the developmental abnormalities delineated by the herniation of neural tissue through a skull defect. In children with tissue masses in the nasal cavity, presenting with breathing difficulties, menigocephaloceles or meningoceles should be suspected. Clinicians often misdiagnose them as polyps or tumors. Inappropriate treatment of undiagnosed hernias may lead to secondary meningitis or fluid leak due to their incision or puncture.

MRI is a non-invasive, safe imaging method that allows a definite diagnosis of menigocephaloceles and meningoceles. Noonan syndrome shares some similar characteristic facial features as lateral meningocele syndrome. Facial features of lateral meningocele syndrome comprise of arched eyebrows, ptosis, flat midface, thin upper lip, low-set, and posteriorly angulated ears, and low posterior hairline, hands with wide and short distal second and third fingers(pseudo-clubbing).[13][12]

Meningocele usually has no neural involvement and without any acute neurological complications, hence a favorable prognosis. Spina bifida aperta usually presents a skin defect with the looming risk of CSF leak, whereas the occult forms have no visible skin opening. The prevalence of spina bifida at birth has decreased by 23% over ten years between 1995 and 2004 after the Food and Drug Administration mandated fortification of all cereal and grain products with folic acid. In clinical practice, the prognosis of patients with meningocele is excellent, and simple surgical repair is adequate.[5][3][2]

The compression of meningocele can cause symptoms of increased intracranial pressure due to the displacement of extra spinal cerebrospinal fluid into the intracranial cavity. Raised intracranial tension may cause erosion of the skull base and may result in a cerebrospinal fluid leak. Recent reports indicate that meningocele is often associated with tethered cord syndrome, and those symptoms of tethered cord syndrome occur in a considerable proportion of children who had meningocele repair. Myelomeningocele is commonly associated with hydrocephalus.[1][14][3]

The prevalence of neural tube defects has dipped significantly during the past thirty years due to advances in ultrasonography's refined resolution for in utero fetal examination, the clinical availability of laboratory markers like serum alpha-fetoprotein measurements, termination of affected pregnancies, and consumption of folic acid supplements by women in the reproductive age group.

Mothers with diabetes, obesity, hyperthermia, drugs that interfere with folate metabolisms, excessive vitamin A, cigarette smoking, alcohol consumption during pregnancy, and antiepileptic drugs like valproate and carbamazepine have increased risk of children with Neural tube defects. Folic acid supplementation of 4 mg to mothers with risk factors. Prenatal screening for neurological abnormalities is based on an ultrasound performed routinely or oriented by maternal alpha-fetoprotein (AFP) screening around 12, 22, and 32 weeks.[6][2]

A number of institutions have organized spina bifida clinics with a multi-disciplinary care team consiting of neurologists, neurosurgeons, rehabilitation specialists, physical and occupational therapists to take care of children with spinal dysraphism. Such organized treatment is highly recommended to ensure the best care of such patients.