Fourth Ventricular Hamartoma Presenting with Status Epilepticus Treated with Emergency Surgery in an Infant

Abstract

A 4-week-old infant presented with hemifacial spasms noticed from the 4th day after delivery. These progressed in severity, with generalization every 3–4 h. On admission, the infant went into refractory status epilepticus and had to be electively ventilated and taken for surgery on a semi-emergency basis. MRI showed a fourth ventricular hamartoma and video EEG showed spikes that were synchronous with the facial ‘twitches’ with generalization. Following the first surgery, the infant had an initial complete recovery, but developed recurrence of facial twitches after 2 weeks. Repeat MRI showed a small residual tumor which was re-operated and completely excised (at 8 weeks). Following this, the patient had complete recovery from seizures (5-year follow-up). This is the youngest patient reported presenting with status epilepticus with a fourth ventricular hamartoma operated successfully.

Epilepsy surgery in infants up to 3 months of age: Safety, feasibility, and outcomes: A multicenter, multinational study

“…For 63 of 64 patients, preoperative MRI was reported and included: a focal lesion in 15 (24%), hemispheric abnormality in 42 (67%), and multilobar in 6 (9%). One lesion was located in the fourth ventricle (a hamartoma) 46 …”

Surgical treatment of status epilepticus: A palliative approach

“…Different modalities were proposed in the literature over the years as an alternative and as an ultimate approach in patients with catastrophic epilepsy and status epilepticus (Gorman et al., ; Rossi et al., ; Krsek et al., ; Ng et al., ; Khoury et al., ; Zumsteg et al., ; Costello et al., ; Ng et al., ; Mohamed et al., ; Ng et al., ; Nahab et al., ; Zamponi et al., ; Lega et al., ; Loddenkemper et al., ; Tellez‐Zenteno et al., ; Chandra et al., ; Prasad et al., ; Wehner et al., ; Chassoux et al., ; Weimer et al., ). Single case reports were described in the literature applying resections of epileptogenic focus, multilobar resection, tumor resection, and callosotomy (Gorman et al., ; Rossi et al., ; Krsek et al., ; Ng et al., ; Khoury et al., ; Zumsteg et al., ; Costello et al., ; Ng et al., ; Mohamed et al., ; Ng et al., ; Nahab et al., ; Lega et al., ; Loddenkemper et al., ; Tellez‐Zenteno et al., ; Chandra et al., ; Wehner et al., ; Chassoux et al., ; Weimer et al., ). In a total and cumulative number of 44 patients, a resection of the epileptogenic foci was achieved (Gorman et al., ; Rossi et al., ; Ng et al., ; Khoury et al., ; Zumsteg et al., ; Costello et al., ; Ng et al., ; Mohamed et al., ; Ng et al., ; Nahab et al., ; Lega et al., ; Loddenkemper et al., ; Tellez‐Zenteno et al., ; Wehner et al., ; Chassoux et al., ; Weimer et al., ).…”

“…Different modalities were proposed in the literature over the years as an alternative and as an ultimate approach in patients with catastrophic epilepsy and status epilepticus (Gorman et al, 1992;Rossi et al, 1999;Krsek et al, 2002;Ng et al, 2003;Khoury et al, 2005;Zumsteg et al, 2005;Costello et al, 2006;Ng et al, 2006;Mohamed et al, 2007;Ng et al, 2007;Nahab et al, 2008;Zamponi et al, 2008;Lega et al, 2009;Loddenkemper et al, 2009;Tellez-Zenteno et al, 2009;Chandra et al, 2011;Prasad et al, 2011;Wehner et al, 2011;Chassoux et al, 2012;Weimer et al, 2012). Single case reports were described in the literature applying resections of epileptogenic focus, multilobar resection, tumor resection, and callosotomy (Gorman et al, 1992;Rossi et al, 1999;Krsek et al, 2002;Ng et al, 2003;Khoury et al, 2005;Zumsteg et al, 2005;Costello et al, 2006;Ng et al, 2006;Mohamed et al, 2007;Ng et al, 2007;Nahab et al, 2008;Lega et al, 2009;Loddenkemper et al, 2009;Tellez-Zenteno et al, 2009;Chandra et al, 2011;Wehner et al, 2011;Chassoux et al, 2012;Weimer et al, 2012). ...…”

Status epilepticus: Having treatment paradigms in place in Neurosurgical settings

“…If medical management fails, emergency resective neurosurgery has been tried and shown to be successful for control of seizures. [27] Various surgical procedures including focal resection, multiple subpial resection for partial seizures and corpus callosotomy, hemispherotomy, vagal nerve stimulation can be done and are found to be effective on a case to case basis. [28] An important and potentially reversible cause of SE, noticed recently, is autoimmune encephalitis, with antibodies against neural tissue including NMDA receptor, voltage-gated potassium channel, antiglutamic acid decarboxylase antibodies, where steroids and immunotherapy like immunoglobulin and plasma exchange provides a good response.…”