Arachnoid cyst with GnRH-dependent sexual precocity and growth hormone deficiency

Abstract

The coexistence of gonadotropin-releasing hormone (GnRH)–dependent sexual precocity and growth hormone deficiency in patients with arachnoid cysts is rarely reported, and its pathogenesis is not well recognized. This report describes an 11-year-old female who had a huge intracranial arachnoid cyst with initial symptoms and signs of sexual precocity. Her brain magnetic resonance imaging revealed distorted hypothalamus with a thin and stretched pituitary stalk. After treatment with cysto-peritoneal shunting and gonadotropin-releasing hormone analogue, her puberty was arrested and subnormal growth rate was observed. Catch-up growth was detected after growth hormone therapy. Hence, coexistence of gonadotropin-releasing hormone–dependent sexual precocity and growth hormone deficiency in this patient was confirmed.

Introduction

Intracranial arachnoid cysts, which comprise 1% of intracranial mass lesions [1], are developmental anomalies of the arachnoid membrane [2]. Many arachnoid cysts are clinically silent and are identified only incidentally [3]. Nevertheless, suprasellar arachnoid cysts, which constitute 9% of all arachnoid cysts [4], are frequently symptomatic [4], [5] and related to gonadotropin-releasing hormone (GnRH)-dependent sexual precocity [6], [7]. Not until recently has it been clarified that suprasellar arachnoid cysts may actually affect many aspects of the hypothalamic-pituitary function [8], [9], [10], [11]. However, so far only few studies have reported combined sexual precocity and growth hormone deficiency associated with arachnoid cysts [9], [10], [11]. Thus we report here an 11-year-old Taiwanese female with combined GnRH-dependent sexual precocity and growth hormone deficiency associated with an extensive intracranial arachnoid cyst. She was relieved from the progression of sexual precocity and achieved a normal growth rate after treatment with both gonadotropin-releasing hormone analogue and recombinant human growth hormone.