Meningohydroencephalocoele

Meningohydroencephalocoele is a rare neural tube defect that involves herniation of the meninges, cerebrospinal fluid, and parts of the brain through a defect in the skull. This condition is often associated with significant morbidity and mortality.

Etiology

The exact cause of Meningohydroencephalocoele is unknown. However, it is believed to be multifactorial, involving both genetic and environmental factors. Some studies suggest a possible association with maternal use of certain medications during pregnancy, such as antiepileptic drugs.

Clinical Presentation

Patients with Meningohydroencephalocoele often present at birth with a visible swelling on the head. Other symptoms may include seizures, developmental delay, and hydrocephalus. The severity of symptoms can vary widely, depending on the size and location of the defect.

Diagnosis

Diagnosis of Meningohydroencephalocoele is usually made prenatally through ultrasound or magnetic resonance imaging (MRI). Postnatal diagnosis can be confirmed through computed tomography (CT) scan or MRI.

Treatment

Treatment of Meningohydroencephalocoele involves surgical repair of the defect. The goal of surgery is to close the defect and restore the normal shape of the skull. In some cases, additional surgeries may be needed to treat associated conditions, such as hydrocephalus.

Prognosis

The prognosis for patients with Meningohydroencephalocoele is variable. Factors that can affect prognosis include the size and location of the defect, the presence of associated conditions, and the patient's overall health.

See Also

• Neural tube defects
• Hydrocephalus
• Seizures

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