Spontaneous external rupture of hydrocephalus after fontanelle closure: a case report and review of literature

Child's Nervous System https://doi.org/10.1007/s00381-020-04915-w CASE REPORT Spontaneous external rupture of hydrocephalus after fontanelle closure: a case report and review of literature Varidh Katiyar 1 & Kanwaljeet Garg 1 P. Sarat Chandra 1 1 1 1 & Ramesh Doddamani & Pankaj Kumar Singh & Manmohan Singh & Received: 15 April 2020 / Accepted: 1 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020 Abstract We report a 2-year-old child with congenital hydrocephalus who presented with a frontal meningocoele due to ventriculosubgaleal fistula secondary to hydrocephalus (internal rupture), which gradually ruptured spontaneously through the skin. This case is unique given its association with occipital encephalocele and is only the second case to present with external rupture beyond the age of 1 year. Further, the ventricles have ruptured through the skull, after the closure of anterior fontanelle, into the subgaleal space. It challenges the present understanding that external rupture occurs only through an open anterior fontanelle. Keywords Spontaneous rupture . External rupture . Hydrocephalus . Fontanelle closure . Subgaleal space Background Hydrocephalus is one of the most common causes of neurosurgical consultation in the pediatric age group. The spectrum of cases of hydrocephalus differs widely among the populations with different socio-cultural and economic backgrounds. In developing countries with more influence of alternative medical practices and poverty resulting in unaffordability, adequate intervention is usually delayed. This results in some bizarre complications, unheard in the developed countries in present times. Few cases with spontaneous external rupture through the open * Kanwaljeet Garg Varidh Katiyar Ramesh Doddamani Pankaj Kumar Singh Manmohan Singh P. Sarat Chandra 1 Department of Neurosurgery, All India Institute of Medical Sciences, 720, CNC, AIIMS, New Delhi 110029, India anterior fontanelle have been reported in the literature [1–4]. We report a child with congenital hydrocephalus who presented with a frontal meningocele due to ventriculo-subgaleal fistula secondary to hydrocephalus (internal rupture), which gradually ruptured spontaneously through the skin. Case A 2-year-old boy born at full term by normal vaginal delivery presented to our outdoor clinic with complaints of a cystic swelling in the right frontal region and occipital region since birth. Though he had no focal neurological deficits, but the developmental milestones were delayed for age in all domains. At presentation, he was able to speak only monosyllables and was able to walk with support. There was bony deformity in the right frontal region as well, with scalloping of the skull and everted bony edges around the skull defect. He was diagnosed with multiple cranial meningocoeles including one in the occipital region and frontal region. He also had a swelling in the occipital region that was soft and around 4 × 4 cm in size. This was diagnosed as an occipital encephalocele. He also had skin erosions over the right frontal swelling and had complaints of occasional watery discharge from them (Fig. 1). While in the hospital, there was rupture at the ulcer site with CSF leak through the ulcer. There was a resultant decrease in the size of the swelling of the right frontal region. The computed tomography scan before the leak Childs Nerv Syst Fig. 1 Clinical picture of the boy showing the subgaleal collection of CSF with overlying skin ulceration and bony deformity revealed enlarged lateral ventricles (right side more than the left side). The right lateral ventricle was continuous with the subgaleal swelling communicating through a defect in the right frontal bone. The outer table of the skull around the defect was also deficient over a large area with everted bony edges at the margins of the area of resorption (Fig. 2). The magnetic resonance imaging (MRI) revealed significant thinning of the cerebral cortex over the enlarged ventricles and wide area of communication between the ventricles and subgaleal collection. It also showed the presence of occipital encephalocele (Fig. 3). The patient was planned for ventriculoperitoneal shunt followed by repair of dural defect and cranioplasty. Right occipital medium pressure Chhabra shunt, using a slit and spring valve mechanism, was placed. His postoperative course was uneventful and he was discharged after 5 days. CT scan was done on postoperative day 1 which shows adequate placement of shunt in the frontal horn (Fig. 4). The skin erosions gradually healed over 1 month. In addition, the encephalocele also reduced in size gradually. At the time of last follow-up, the patient has progressed in the developmental milestones across all domains and has shown good catch-up development; however, he is still significantly behind his chronological age. He is now able to climb stairs one at a time, speak 3 word sentences, and knows his name. Definitive surgery is planned for encephalocele but it has been delayed as elective procedures are suspended because of the COVID 19 pandemic. Discussion Hydrocephalus requires timely intervention to prevent the development of complications like thinning of cortical mantle Fig. 2 Computed tomography scan showing the bony deformity and the defect through which ventricles are communicating with the subgaleal collection. a Axial section. b Coronal section Childs Nerv Syst Fig. 3 MR T2 weighted images showing the dilated ventricles and their communication with the subgaleal collection (red arrow). Occipital encephalocele can also be seen (yellow arrow). a Coronal sections. b Sagittal sections and internal and external rupture of hydrocephalus. The procedures for hydrocephalus are fairly simple and thus widely available these days. However, due to socio-cultural and economic factors as well as illiteracy, many cases present with neglected hydrocephalus in developing countries. In prenatal causes like aqueductal stenosis, the increasing hydrocephalus may result in loss of white matter and corpus callosal volume and lead to loss of septal leaflets or even ventricular rupture in intrauterine life itself [5]. With the delay in treatment, there is gradual thinning of the cerebral cortex and enlargement of the head especially in infants before sutural closure. If the pathologic process goes without intervention for long, it may lead to some odd complications like ventricular diverticulum [6] and even ventricular rupture. A few cases with internal rupture of enlarging ventricles into cisternal spaces or subdural space [7–9] and external rupture through the patent anterior fontanelle presenting with CSF leak with or without meningitis have already been reported in the literature (Table 1) [1–4, 10, 11]. Out of the 6 other cases of external rupture described in the literature, 2 were associated with lumbosacral myelomeningocele [2, 3] and in other 3, congenital aqueductal stenosi (...truncated)