Suprasellar arachnoidal cyst as a cause of precocious puberty--report of three patients and literature overview.
The authors present three boys--3 years old, 5.8 years old and 10.4 years old--who were diagnosed with isosexual precocious puberty (IPP) triggered by a rare developmental disorder of suprasellar arachnoid cyst (SAC) accompanied by corpus callosum and fornix dysgenesis as well as anterior commissura magna agenesis (patient 1) and empty sella (patients 2, 3). The reason for diagnostic management recommendation was a rapid progression of IPP signs over one year (patients 1, 2) or 6 months (patient 3) prior to hospitalization, these signs having been present but less intense since infancy (patient 1), 4th year of life (patient 2) and approximately 8 years of age (patient 3). Neurological signs (spastic paresis in patient 1, postural tremor in patient 2 and head bobbing and behavioral changes in patient 3), as well as slowly progressing increased head circumference were observed since neonatal period (patient 1), 1 year old (patient 2) and approximately 4 years old (patient 3). None of the patients manifested hypophyseal-hypothalamic axis dysfunction other than IPP prior to and after surgical management. Shunt implantation resulted in gradual resolution of neurological signs in all patients and in patient 3 also in partial normalization of serum testosterone levels and growth rate. Regression of IPP in patients 1 and 2 was achieved by administration of a long-acting GnRH analogue. Our observations are in accord with data reported by other investigators and confirm the often slow, insidious development of subsequent SAC signs, the type and intensity of which differ from patient to patient. We suggest that some of the neuroanatomical anomalies coexisting with SAC may have a common genesis, or they could under certain conditions be an additional trigger for IPP and possibly other hypothalamopituitary dysfunction.
Starzyk
Jerzy
J
Department of Pediatric and Adolescent Endocrinology, Polish-American Children 's Hospital, Faculty of Medicine, Jagiellonian University, Cracow, Poland. mistarzy@cyf-kr.edu.pl
Kwiatkowski
Stanisław
S
Urbanowicz
Wiesław
W
Starzyk
Bozena
B
Harasiewicz
Marek
M
Kalicka-Kasperczyk
Anna
A
Tylek-Lemańska
Dorota
D
Dziatkowiak
Hanna
H
D016080:Arachnoid Cysts / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D020863:Central Nervous System Cysts / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003337:Corpus Callosum / Q000473:pathology; D004652:Empty Sella Syndrome / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D020712:Fornix, Brain / Q000473:pathology; D006801:Humans; D008297:Male; D011629:Puberty, Precocious / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012686:Septal Nuclei / Q000473:pathology; D016896:Treatment Outcome