European Journal of
Pediatrics
Letter to the editors
9 Springer-Verlag1992
Eur J Pediatr (1992) 151 : 76
Suprasellar arachnoid cyst as a cause of precocious puberty and bobble-head doll phenomenon M. Turgut and O. Ekin Ozcan Department of Neurosurgery, Hacettepe University Medical Centre, 06100, Ankara, Turkey
Sir: Suprasellar arachnoid cysts (SSAC) are relatively rare, comprising 1 % - 1 5 % of all arachnoid cysts [2, 3]. Their association with both precocious puberty and curious head nodding has been noted [1-4]. However, we know of no report describing these two clinical signs in the same patient with SSAC. An 8-year-old boy was admitted with a 2-year history of early pubertal development, a curious head-nodding motion and moderate mental retardation. Examination showed a Tanner pubic hair stage III and a testicular volume of 8 ml. Irregular, involuntary head motions were noted. The testosterone level was elevated to 8.3 ng/ml (normal 0.15 + 0.05 ng/ml). CT showed a large oval lucency in the region of the third ventricle. On metrizamide CT cisternography, contrast material failed to enter the lateral ventricles and lesion. Part of the cyst walt was surgically excised allowing wide communication between the cyst and subarachnoid Abbreviation: SSAC = suprasellar arachnoid cyst
space. The cyst contained clear, colourless fluid and histologically its wall was composed of connective tissue and arachnoid cells. Six months later, the patient's movement disorder had disappeared and the serum testosterone level was 1.7 ng/ml. Four years after surgery, the patient continues to do well. SSAC are congenital abnormalities, secondary to an imperforate membrane of Liliequist [1] resulting in obstruction of CSF flow at the level of the suprasellar cistern. They must be differentiated from ependymal cysts of the third ventricle, parasitic cysts and marked third ventricle enlargement caused by aqueductal stenosis [3, 4]. Since partial excision of the cyst resulted in the disappearance of both signs, we believe that precocious puberty and the nodding head p h e n o m e n o n were clinical manifestations of a single pathogenetic process rather than independent disorders.
References 1. Fox JL, A1, Merry O (1980) Suprasellar arachnoid cysts: an extension of the membrane OfLiliequist. Neurosurgery 7 : 615-618 2. Gonzales CA, Villarejo FT, Blazquez MG, Castroviejo IP, Higueras AP (1982) Suprasellar arachnoid cysts in children-report of 3 cases. Acta Neurochir (Wien) 60 : 281-296 3. Harsh IV GR, Edwards MSB, Wilson CB (1986) Intracranial arachnoid cysts in children. J Neurosurg 64: 835-842 4. Hoffman H J, Hendfick EB, Humphreys RP, Armstrong EA (1982) Investigation and management of suprasellar arachnoid cysts. J Neurosurg 57 : 597-602
Pediatrics
Letter to the editors
9 Springer-Verlag1992
Eur J Pediatr (1992) 151 : 76
Suprasellar arachnoid cyst as a cause of precocious puberty and bobble-head doll phenomenon M. Turgut and O. Ekin Ozcan Department of Neurosurgery, Hacettepe University Medical Centre, 06100, Ankara, Turkey
Sir: Suprasellar arachnoid cysts (SSAC) are relatively rare, comprising 1 % - 1 5 % of all arachnoid cysts [2, 3]. Their association with both precocious puberty and curious head nodding has been noted [1-4]. However, we know of no report describing these two clinical signs in the same patient with SSAC. An 8-year-old boy was admitted with a 2-year history of early pubertal development, a curious head-nodding motion and moderate mental retardation. Examination showed a Tanner pubic hair stage III and a testicular volume of 8 ml. Irregular, involuntary head motions were noted. The testosterone level was elevated to 8.3 ng/ml (normal 0.15 + 0.05 ng/ml). CT showed a large oval lucency in the region of the third ventricle. On metrizamide CT cisternography, contrast material failed to enter the lateral ventricles and lesion. Part of the cyst walt was surgically excised allowing wide communication between the cyst and subarachnoid Abbreviation: SSAC = suprasellar arachnoid cyst
space. The cyst contained clear, colourless fluid and histologically its wall was composed of connective tissue and arachnoid cells. Six months later, the patient's movement disorder had disappeared and the serum testosterone level was 1.7 ng/ml. Four years after surgery, the patient continues to do well. SSAC are congenital abnormalities, secondary to an imperforate membrane of Liliequist [1] resulting in obstruction of CSF flow at the level of the suprasellar cistern. They must be differentiated from ependymal cysts of the third ventricle, parasitic cysts and marked third ventricle enlargement caused by aqueductal stenosis [3, 4]. Since partial excision of the cyst resulted in the disappearance of both signs, we believe that precocious puberty and the nodding head p h e n o m e n o n were clinical manifestations of a single pathogenetic process rather than independent disorders.
References 1. Fox JL, A1, Merry O (1980) Suprasellar arachnoid cysts: an extension of the membrane OfLiliequist. Neurosurgery 7 : 615-618 2. Gonzales CA, Villarejo FT, Blazquez MG, Castroviejo IP, Higueras AP (1982) Suprasellar arachnoid cysts in children-report of 3 cases. Acta Neurochir (Wien) 60 : 281-296 3. Harsh IV GR, Edwards MSB, Wilson CB (1986) Intracranial arachnoid cysts in children. J Neurosurg 64: 835-842 4. Hoffman H J, Hendfick EB, Humphreys RP, Armstrong EA (1982) Investigation and management of suprasellar arachnoid cysts. J Neurosurg 57 : 597-602
