| Literature DB >> 33552794 |
Klenam Dzefi-Tettey1, Emmanuel K Edzie2, Philip Gorleku2, Albert D Piersson3, Obed Cudjoe4.
Abstract
Tuberous sclerosis (TS) is a rare genetic disorder of autosomal-dominant inheritance. Mutations on either of the two genes Tuberous Sclerosis Complex 1 (TSC1) or Tuberous Sclerosis Complex 2 (TSC2) play a role and result in hamartomas involving many organs, like the brain, heart, kidneys, skin, lungs, and liver. This case report is about a four-year-old boy with facial angiofibromas, hypo-pigmented skin lesions on the lower back and dorsum of the right wrist, and previous history of seizures who was referred to the radiology department of the Korle Bu Teaching Hospital for Magnetic Resonance Imaging (MRI) of the brain. The MRI of the brain revealed subependymal giant cell astrocytomas, subependymal nodules, and cortical tubers. Ultrasonography of the abdomen also showed multiple angiomyolipomas and multiple simple cysts in both kidneys. The aim of this case report is to present the imaging findings and create awareness that this rare genetic disorder does exist in Ghana and advocate for formation of support groups for parents with children with tuberous sclerosis.Entities:
Keywords: angiofibromas; renal angiomyolipomas; seizures; subependymal giant cell astrocytoma; tuberous sclerosis
Year: 2021 PMID: 33552794 PMCID: PMC7854338 DOI: 10.7759/cureus.12481
Source DB: PubMed Journal: Cureus ISSN: 2168-8184