Growth, puberty and hypothalamic-pituitary function in children with suprasellar arachnoid cyst

Abstract

A suprasellar arachnoid cyst may cause disorders of growth, puberty and hypothalamic-pituitary function, due to the proximity of the cyst to the hypothalamic-pituitary area. A total of 30 patients (17 boys) with cyst diagnosed at 4.3 1 years were routinely evaluated at 5.4 1 years; 24 of them had one or multiple cyst derivations. Some 23 cases had an abnormal height, weight or puberty: short (<)2SD, 5 cases) or tall (>2SD, 10 cases) stature, overweight (body mass index, BMI, >2SD, 6 cases), central precocious puberty (10 cases) and/or no progression of pubertal development (3 cases). The growth hormone (GH) peaks after pharmacological stimulation test were low (<10 lg/l) in 16 patients, con®rmed by a second evaluation in 8/11 of them. The plasma free thyroxine was low in ®ve patients, prolactin was high in two and the cortisol and concomitant plasma and urinary osmolalities were normal. BMI was correlated negatively with the GH peaks (r )0.37, P < 0.01) and positively with the plasma leptin concentrations (r 0.55, P < 0.01). The plasma fasting insulin concentrations were also correlated negatively with the GH peaks (r )0.55, P < 0.02) and positively with the plasma insulin-like growth factor I concentrations (r 0.64, P < 0.002). The adult height (12 cases) was at 4SD in 1 and <)2SD in 4 patients, two of whom had precocious puberty untreated with gonadotropin releasing hormone (GnRH) analogue, and two had untreated GH de®ciency. The adult height of those treated was normal. One girl had primary amenorrhoea and two boys had low plasma testosterone, despite a normal gonadotropin response to a GnRH test. Conclusion Suprasellar arachnoid cysts may cause de®ciencies of growth hormone and thyrotropin, stimulation of the hypothalamic-pituitary-gonadal axis, tall stature and/or overweight. These last two disorders may be due to hyperinsulinism, itself due to suprasellar arachnoid cyst.

GH deficiency with central precocious puberty: a new rare disorder associated with a developmental defect of the hypothalamic–pituitary area

“…In those patients, the possibility that the hGH treatment could have influenced the early onset of puberty is unknown (27). This rare combination of GHD and CPP has been reported essentially in patients with septo-optic dysplasia (6-10) and arachnoid cyst (2)(3)(4)(5). It has also exceptionally been described after a head trauma (7,12).…”

“…However, GHD has been reported to be associated with CPP in cases of arachnoid cyst (2)(3)(4)(5), septo-optic dysplasia (6-10), brain tumors in the pituitary region, or after cerebral radiation therapy, particularly if administered as prophylaxis against acute lymphoblastic leukemia (11)(12)(13). This association has also been reported in very rare cases after a head injury (7,13).…”

Long-term endocrine outcome of suprasellar arachnoid cysts

“…Abnormality in various aspects of this axis has been documented in relation to suprasellar arachnoid cysts. 2,18 Although endocrine abnormalities are commonly known to present as symptoms of suprasellar arachnoid cysts, other modes of detection have been mentioned in the literature, e.g., the possibilities of "preexistence" and "posttreatment presentation." 18 Nevertheless, this is the first study to describe in detail the long-term endocrine outcomes of patients with suprasellar arachnoid cysts.…”

“…2,7 The most controversial topic on suprasellar cysts has been the optimal choice of surgical modality. First, operations using only fenestration precluded shunt procedures including ventriculoperitoneal shunting (VPS) and cystoperitoneal shunting (CPS) as the surgical modality of treatment.…”

Arachnoid Cysts - Common and Uncommon Clinical Presentations and Radiological Features

“…Suprasellar arachnoid cysts may cause obstruction of the third ventricle at the level of the foramen of Monroe leading to hydrocephalus. Endocrine dysfunction with deficiencies in growth hormone and thyrotropin or disturbance to the hypothalamic-pituitarygonadal axis may occur in the case of suprasellar arachnoid cyst [26]. A rare "bobble-head-doll" syndrome has been described due to a suprasellar arachnoid cyst causing compression on the third ventricle and dorsal thalamic nuclei [27].…”