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Updated: Mar 18, 2026

Right Hemihepatectomy by Suprahilar Intrahepatic Transection of the Right Hemipedicle using a Vascular Stapler

Right Hemihepatectomy by Suprahilar Intrahepatic Transection of the Right Hemipedicle using a Vascular Stapler

Published on: January 25, 2010

Hemispherectomy Procedure in Proteus Syndrome.

PrastiyaIndra Gunawan¹, Lusiana Lusiana¹, Darto Saharso¹

¹Airlangga University College of Medicine, Jl. Prof DrMoestopo, 6-8 Surabaya, Indonesia.

Iranian Journal of Child Neurology

|July 5, 2016

Summary

Proteus syndrome, a rare overgrowth disorder, can cause severe neurological issues like hemimegalencephaly and intractable seizures. Hemispherectomy successfully resolved seizures in a 1-month-old infant with this condition.

Keywords:

Hemimegalencephaly Hemispherectomy Intractable epilepsy Linear nevi Proteus syndrome

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Area of Science:

Neurology
Genetics
Pediatrics

Background:

Proteus syndrome is a rare congenital overgrowth disorder affecting bone, soft tissue, and skin.
Central nervous system involvement occurs in approximately 40% of cases, including hemimegalencephaly, seizures, and cognitive deficits.

Observation:

A 1-month-old male infant presented with recurrent seizures, facial asymmetry, subcutaneous mass, and linear nevi.
Cranio-cervical MRI confirmed right-sided hemimegalencephaly.
Despite triple antiepileptic drug therapy and a ketogenic diet, seizures persisted.

Findings:

The infant's seizures were refractory to medical management, including antiepileptic drugs and ketogenic diet.
Surgical intervention via hemispherectomy led to complete seizure resolution.

Implications:

Hemispherectomy can be an effective treatment for intractable epilepsy secondary to hemimegalencephaly in Proteus syndrome.
Early surgical consideration may improve neurodevelopmental outcomes in affected children.
This case highlights the importance of recognizing diverse CNS manifestations of Proteus syndrome.