Accelerated growth of the cranial perimeter with suture diastasis

Specialty: Pediatrics.

• Progressive macrocephaly
• suspected infantile hydrocephalus
• rapid increase in head size

Why it occurs

• Congenital or acquired obstructive or non-obstructive hydrocephalus (abnormal accumulation of cerebrospinal fluid in the ventricular system due to stenosis of the sylvian aqueduct, Chiari type II malformation, or secondary to intraventricular hemorrhage or previous neonatal bacterial meningitis with impaired resorption in the arachnoid villi)
• Benign familial megalencephaly or macrocephaly (normal constitutional variant of brain growth characterized by completely normal psychomotor development and a history of macrocephaly in one or both parents)
• Subdural fluid collections or hygromas (accumulation of cerebrospinal fluid or blood in the subdural space, frequently of accidental traumatic origin or secondary to physical abuse/shaken infant syndrome)
• Childhood central nervous system tumors (posterior fossa tumors, giant tuberous sclerosis astrocytomas or ependymomas that compress the CSF ducts)
• Metabolic lysosomal storage diseases (Canavan disease, Alexander disease, mucopolysaccharidosis with progressive cerebral infiltration and edema).

Initial workup

Precise and serial measurement of the cranial perimeter and recording of the growth curve of the child and his parents. Transfontanellar ultrasound (rapid, safe and initial test of choice in infants with a patent anterior fontanel to immediately distinguish hydrocephalus from subdural collections). High-resolution Brain Magnetic Resonance (MRI) (the gold standard to assess the anatomy of the ventricular system, the brain parenchyma and rule out masses or fluid collections). Ophthalmological examination with fundus to look for papilledema or retinal hemorrhages (highly suggestive of physical abuse or intracranial hypertension).

Standard management

• In case of obstructive hydrocephalus or tumor, the definitive treatment is surgical — ventriculo-peritoneal shunt placement or endoscopic ventriculostomy of the third ventricle). Acetazolamide (carbonic anhydrase inhibitor that reduces CSF production, used temporarily in slowly progressive or presurgical hydrocephalus; dose of 20 to 100 mg/kg/day orally divided every 8 hours
• Furosemide — adjuvant loop diuretic; dose of 1 mg/kg/day orally or IV
• Corticosteroids such as Dexamethasone (indicated to reduce vasogenic edema associated with brain tumors; doses of 0.15 to 0.5 mg/kg/day IV).