**3. Clinical features**

This depends on the age at presentation and the time after the onset of symptoms or previous surgical intervention.

#### **3.1 Neonates and infants**

At birth, hydrocephalus is apparent in about 15% of neonates with myelomeningocele, with features such as macrocephaly with craniofacial disproportion, increasing head size, bulging anterior fontanelle, calvaria sutural diastasis, distended scalp veins, poor feeding, vomiting, sunsetting eyes, bradycardia, and recurrent apnea [1].

Patients who have had myelomeningocele repair may present with pseudomeningocele at the site of repair; CSF leak from the repair and lower brainstem compromise from the Chiari II malformation may cause stridor from vocal cord weakness, a weak high-pitched cry, swallowing difficulties, poor feeding, nasal regurgitation of feeds, weakness, and hypotonia [10].

Occipito-frontal circumference measurement is important because the patent fontanelles and calvarial sutures may mask overt signs of raised intracranial pressure due to increasing head size, though there may be significant pathology in the brain. When OFC crosses centiles or increases rapidly, surgical intervention is mostly indicated [11].

#### **3.2 Post infancy**

Beyond infancy, hydrocephalus typically presents with features of raised intracranial pressure such as headache, vomiting, and altered consciousness. They may also

*Hydrocephalus Associated with Myelomeningocele DOI: http://dx.doi.org/10.5772/intechopen.110535*

**Figure 1.**

*Clinical picture of an infant with macrocephaly, bulging anterior Fontanelle, craniofacial disproportion, and distended scalp veins.*

present with loss of developmental milestones, diplopia, unsteady gait, and impaired cognitive functions (**Figure 1**) [1].
