Tuberous Sclerosis Complex Co-Existing with Bilateral Cryptorchidism: A Case Report and Review of Literature.
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Abstract
Background: Tuberous sclerosis is a rare multisystemic, genetic neurocutaneous syndrome that causes benign tumours to grow in the brain and vital organs. It demonstrates high clinical and radiological manifestations.
AIM: To report a case of tuberous sclerosis complex co-existing with bilateral cryptorchidism in the University of Port-Harcourt Teaching hospital, Port Harcourt.
CASE REPORT: A case of 11year old male, a primary three pupil, who developed seizure disorder at the age of 7 months and subsequently developed facial angiofibroma and periungual fibroma at the age of 9years. Neuroimaging revealed multiple calcified subependymal harmatomas on the walls of the lateral ventricles and multiple subcortical and cortical tubers. The patient is currently on anticonvulsant and his seizures are controlled. He has moderate degree of mental retardation. The angiofibroma are not receding.
CONCLUSION: Tuberous sclerosis complex is an uncommon disease entity. Treatment is symptomatic. Prognosis varies in accordance with the severity of the specific symptoms. A regular imaging follow up is advised for patients with this condition to avoid development of obstructive hydrocephalus from the tumours.
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