Hemispherectomy in a Premature Neonate with Linear Sebaceous Nevus Syndrome

Abstract

A male neonate with seizures, linear sebaceous nevus syndrome and hemimegalencephaly underwent hemispherectomy at 36 weeks’ gestational age. He has had no clinical seizures in the 13 months since surgery but continues to have sharp wave activity over some parts of the intact hemisphere. He has moderate developmental delay and a mild hemiparesis but is making developmental progress.

Hemispherectomy for Hemimegalencephaly Due to Tuberous Sclerosis and a Review of the Literature

“…12 A two-staged hemispherectomy was performed in a premature neonate of 36 weeks gestational age with hemimegalencephaly and linear sebaceous nevus syndrome, M A N U S C R I P T A C C E P T E D ACCEPTED MANUSCRIPT another neurocutaneous disorder. 25 As in our patient, hemispherectomy led to seizure cessation and developmental progress.…”

The group of epidermal nevus syndromes

“…23 It is the best known ENS, and some authors still prefer to categorize it as ''the ENS.'' [24][25][26][27][28][29][30] The terminology of this birth defect is rather confusing and includes, in addition to Schimmelpenning syndrome, 5,21,31 many other names, such as Feurstein, FeuersteinFeurstein, Feuerstein Mims syndrome, [32][33][34] Schimmelpenning-Feuerstein-Mims syndrome, 35-37 Solomon syndrome, [38][39][40] ENS, 12,38,[41][42][43][44][45][46] Jadassohn nevus phacomatosis, [47][48][49] Jadassohn-Schimmelpenning-Feuerstein-Mims syndrome, 50 organoid nevus phacomatosis, 35,51 organoid nevus syndrome, 52,53 sebaceous nevus syndrome, [54][55][56][57][58] linear sebaceous nevus syndrome, 50,[59][60][61][62][63][64] and Jadassohn sebaceous nevus syndrome. 65 Consequently, Schimmelpenning syndrome temporarily had three different Online Mendelian Inheritance in Man numbers; these have resently been reduced to two entries (163200 and 601359).…”

Epilepsy in Neurological Phenotypes of Epidermal Nevus Syndrome

“…182,183 Surgery is often performed after 3 months of age and has satisfactory outcome regarding the long-term control of seizures and progress in development. 119,143,184 Surgical resection of highly epileptogenic focal cortical dysplasias including HME is often needed, but if it can be postponed until after 6 months of age, the complications are fewer. If a younger infant is in intractable status epilepticus, surgery may be the only option after medical treatments, including ketogenic diet, have failed.…”

“…Hemispherectomy for HME in the neonatal period is rare, but was performed in a premature neonate with LSNS with good results. 184 Ipsilateral colpocephaly (selective dilatation of the occipital horn) is a characteristic finding in HME. In many cases, unnecessary shunting procedures have been performed when it is confused with obstructive hydrocephalus; this unnecessary risk to the patient should be avoided.…”