bubble_chart Overview

Cranioschisis refers to a congenital defect of the skull; it most commonly occurs in the occipital region of the head, while the nasal root and the base of the anterior cranial fossa are less frequently affected. Cranioschisis can be further divided into occult and overt types. The former involves only a simple absence of the skull without a protruding mass, whereas the latter presents with a protruding cystic mass, hence also termed cystic cranioschisis. Based on the contents of the mass, it can be classified into:

1. meningocele;
2. encephalocele;
3. meningoencephalocele;
4. encephalocystocele;
5. meningoencephalocystocele.

The incidence of this condition shows no regional or gender differences, but it is less common than spina bifida. The primary treatment is surgical intervention. The prognosis is relatively favorable for occult cranioschisis and overt cranioschisis with meningocele or encephalocele, while the prognosis for other types is poor.

bubble_chart Etiology

This disease is a common malformation resulting from incomplete neural tube closure during the fetal period. Its etiology may be related to the susceptibility of the central nervous system to various traumas, infections, radiation exposure, or chemical damage during development. However, it remains unclear which specific factor leads to which particular malformation.

bubble_chart Clinical Manifestations

1. Occult Cranioschisis: No local or neurological symptoms, often discovered incidentally through skull X-rays taken for other reasons, revealing a cranial defect.
2. Manifest Cranioschisis (also known as Cystic Cranioschisis): A cystic protrusion is noticeable at birth along the midline of the occiput or at the root of the nose, covered by skin or a thin membrane-like structure. If the protrusion pulsates or increases in tension when the infant cries, it indicates communication with the intracranial space. A positive transillumination test suggests simple meningocele. In cases of meningoencephalocele, shadows of brain tissue may be visible, accompanied by intellectual disability.
3. Occipital meningoceles may cause visual disturbances, while those at the nasal root can lead to facial deformities: a widened and raised nasal root with eyes displaced laterally, increasing the interorbital distance.

bubble_chart Diagnosis

1. The protruding mass at the root of the nose or occipital region exhibits pulsation, with increased tension or enlargement during crying.
2. Skull X-ray reveals a round or oval-shaped skull defect along the midline.
3. Cranial computed tomography shows a localized skull defect along the midline; cerebrospinal fluid or brain tissue connected to the intracranial space can be observed within the protruding mass.
4. Diagnosis can be confirmed by extracting cerebrospinal fluid through puncture of the protruding mass.

bubble_chart Treatment Measures

1. Occult cranioschisis does not require surgical treatment.
2. Cystic cranioschisis is mainly treated by surgical repair. If the surface of the protrusion is a thin membrane-like structure and tends to rupture, emergency surgery should be performed as soon as possible to prevent meningitis; if the skin is intact, the repair surgery can be performed between six months and one year after birth.
3. If hydrocephalus is present, it can be treated first.
4. Prevent infection and provide symptomatic treatment.

Pregnant women should avoid exposure to the aforementioned factors in early pregnancy; if a baby is found to have a head protrusion after birth, do not use folk plaster for external application to reduce swelling, as it may cause the mass to rupture and lead to meningitis. Instead, seek medical guidance and treatment at a hospital. After surgery, the child should avoid excessive crying and coughing to prevent the repaired meninges from rupturing and the cranial contents from protruding again.