Large Skull with Ventricular asymmetry 

Dr Anakha V Ajay, Dr Jayasree (Dept of Neonatology) Dr Suhas( Dept of Neurosurgery) Dr C Jayakumar(Dept of Pediatrics), AIMS, Kochi
A female neonate born to thirty two year old Gravida 2 Abortion 1 mother at 36+6 weeks of gestation elective LSCS due to large head. Antenatally mother had history of GDM on insulin. Anomaly scan showed severe triventriculomegaly secondary to suspected aqueductal stenosis with both lateral and third ventricle dilated. 
Right lateral ventricle – 34 mm and left lateral ventricle – 43mm. 
HC falls at + 4 SD, brachycephaly.
Ba cried immediately after birth. APGAR score 8/9 at 1/5 min. 
Clinical examination revealed large head with widely open anterior and posterior fontanelle. Vaginal tag present.
Auxology showed weight of 2.88 kg Head circumference of 40.5 cm( above 97th percentile) Length of 45 cm. Systemic examination were within normal limits. Moro complete.
Ba was received in NICU in view of hydrocephalus. Ba was hemodynamically stable without any inotropic support and on room air since admission. Ba was on full feeds. In view of jitteriness, serum calcium and magnesium levels were checked and were found to be low, hence ba was given IV calcium and magnesium boluses followed oral supplementation. Since there was no significant clinical or biochemical improvement endocrinology consultation was sought and was advised to start IV calcium along with oral calcium and vitamin D supplementation. Serial calcium and magnesium levels were within normal limits and oral supplements were stopped. Ba was active, with normal tone. 
In view of antenatal scans showing supratentorialhydrocephalus with suspected aqueductal stenosis, a Neurosonogram was done which showed similar findings. Neurosurgery consultation was sought and was advised venticulo subgaleal shunt (VSG)surgery. VSG surgery was done on day 7 of life. CSF analysis done was normal. Ba has been enrolled into the early intervention program. USG KUB and abdomen done to rule out other anomalies were reported to be normal. Thyroid profile was found to be normal. In view of persistent hypocalcemia, Serum PTH levels and vitamin D levels were done and were reported to be within normal limits. In view of presumed sepsis, blood culture was taken and ba was started on IV Ampicillin and amikacin. Sepsis screen was negative and IV Antibiotics were stopped after 48 hours after blood cultures were reported to be normal. Prophylactic anti-fungal was given in view of central line. BERA done was normal. Daily HC monitoring was done and was maintaining at 40 cm. At present, ba is on direct breast feeds along with top up feeds and multivitamin supplementsBa was vaccinated with BCG/ OPV & Hep B prior to discharge. Condition on discharge: Ba is active, feeding well. Ba is advised to be under regular neurosurgery and neurodevelopmental follow up.
 
Hydrocephalus is a common but complex condition caused physical or functional obstruction of CSF flow that leads to progressive ventricular dilatation. Prevalence estimates for infantile hydrocephalus vary between one and 32 per 10,000 birth. It can be congenital or acquired hydrocephalus, non communicating and communicating hydrocephalus. Most common causes of congenital hydrocephalus are aqueductstenosis, brain malformations, spina bifida etc. and of acquired hydrocephalus are brain tumors, intraventricular haemorrhages, meningitis etc. In an infant the most obvious signs is an unusual enlargement of the bas head, bulging fontanelle, scalp vein appear to have unnatural fullness(prominence). Diagnosis is via imaging. Antenatal scans are also useful in the diagnosis. Treatment is primarily shunt which drains excess CSF. The second surgical option is the endoscopic third ventriculostomy.
Prognosis is good if it is diagnosed and managed early. Most children undergo surgery and survive to age one can lead healthy lives with normal intelligence and physical development. Some may be slower to develop skills like hand eye coordination or walking.
Take home message: Close monitoring and multidisciplinary follow up is crucial for assessing developmrnt of complications.