Novel Insights from Clinical Practice Pediatr Neurosurg 2013;49:365–368 DOI: 10.1159/000368323
Received: June 20, 2014 Accepted after revision: September 7, 2014 Published online: November 21, 2014
Sylvian Cistern Arachnoid Cyst – A Rare Cause of Precocious Puberty Sunil Upadhyaya a Rajesh Nair a Vinod Kumar a Bhavna Nayal b Arjun Shetty a Departments of a Neurosurgery and b Pathology, Kasturba Medical College, Manipal University, Manipal, India
Established Facts • Sylvian cistern arachnoid cysts constitute 50% of all supratentorial cysts. • Early diagnosis and prompt treatment ensure excellent outcome.
Novel Insights • Sylvian cistern arachnoid cysts with temporal lobe agenesis causing central precocious puberty are very rare. • Though communicating a cyst with an adjacent ventricle or basal cistern is considered to assure surgical success with the rare hydrocephalic patient requiring a ventriculoperitoneal shunt, our patient did well with a cystoperitoneal shunt.
Abstract Precocious puberty still remains an elusive diagnosis in the majority of patients. Infrequently, lesions of the central nervous system are associated with sexual precocity. Depending on their location, these cysts may affect many systems, however, there is little information concerning their involvement in endocrinological disorders. We report a case of a sylvian cistern arachnoid cyst presenting with precocious puberty in a 3-year-old girl. The child recovered following a
© 2014 S. Karger AG, Basel 1016–2291/14/0496–0365$39.50/0 E-Mail [email protected] www.karger.com/pne
cystoperitoneal shunt. The mass effect of the arachnoid cyst upon the hypothalamus was, at least in part, responsible for the development of precocious puberty. To the best of our knowledge, this is the 1st case of a sylvian cistern arachnoid cyst presenting with precocious puberty. The role of surgical decompression of the cyst is also discussed. © 2014 S. Karger AG, Basel
Introduction
Arachnoid cysts are congenital developmental anomalies of the central nervous system associated with various clinical scenarios and long-term outcomes. They are reDr. Rajesh Nair, MS, FAGE, MCh Department of Neurosurgery Kasturba Hospital, Manipal University Manipal, Karnataka 576104 (India) E-Mail rajeshnair39 @ yahoo.com
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
Key Words Arachnoid cyst · Cystoperitoneal shunt · Precocious puberty · Sylvian cistern
duplicated arachnoid membranes with fluid collection resulting in cyst formation [1–3]. Arachnoid cysts are classified into [4]: (1) Supratentorial (a) Sylvian fissure (50%); (b) sellar region, and (c) interhemispheric/parasagittal (2) Infratentorial (a) Retrocerebellar and (b) laterocerebellar (3) Intraventricular/paraventricular (a) Quadrigeminal region/tentorial notch; (b) subependymal/paraventricular, and (c) choroid plexus cyst The Galassi classification system is one used for classifying arachnoid cysts in the middle cranial fossa [5]. Type I – small, spindle shaped, limited to the anterior middle cranial fossa, usually with free communication on CT cisternography. Type II – superior extent along the sylvian fissure with displacement of the temporal lobe. Type III – large, fills the whole middle cranial fossa, with displacement of not only the temporal lobe but also the frontal and parietal lobes; little communication with the subarachnoid space on CT cisternography.
366
Pediatr Neurosurg 2013;49:365–368 DOI: 10.1159/000368323
tern with midline shift and dilatation of the third and lateral ventricles, associated thinning of the hypothalamus and displacement of the pituitary stalk. FIESTA = Fast imaging employing steadystate acquisition; SOL = space occupying lesion.
Case Report A 3-year-old girl was admitted to the hospital for evaluation of accelerated development of secondary sexual characters and intermittent vaginal bleeding since 2 months. Four months prior to admission, her mother noticed that the child had vaginal bloodtinged white discharge. On physical examination, her height and weight were 110 cm and 24 kg, respectively, which exceeded five standard deviations from the mean level of the normal population. Her head circumference was 57 cm and chest circumference 63 cm. In addition, she had development of breast tissue classified as Tanner’s stage II and pubic hair (pubarche). The bone age by skeletal survey of the hand was rated as 3 years. Vaginal smear showed features compatible with menarche. Endocrinological examination showed elevated serum levels of luteinizing hormone (LH; 104.7 mIU/ml), follicle-stimulating hormone (FSH; 110.4 mIU/ml) and estradiol (146.78 pg/ml), which were equivalent to females at puberty. An LH-releasing hormone (LHRH) test revealed an excessive LH reaction. There were no definite neurological deficits. Magnetic resonance imaging (MRI) of the brain demonstrated the presence of a large arachnoid cyst (12 × 13.4 × 7.6 cm) involving the suprasellar region, an extra-axial right temporoparietal cystic lesion within the sylvian fissure, compressing the whole frontal, temporal and parietal lobe with right temporal pole agenesis. It appeared hypointense on T1 and hyperintense on T2, with no diffusion restriction and extension into the suprasellar, interpeduncular and perimesencephalic cistern, midline shift and dilatation of the third and lateral ventricles (fig. 1). There was associated thinning of the hypothalamus and displacement of the pituitary stalk. Our case, based on MRI findings, was categorized
Upadhyaya/Nair/Kumar/Nayal/Shetty
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
Fig. 1. MRI of the brain (T1 axial and sagittal, T2 axial and coronal, diffusion and FIESTA) showing an SOL which is hypointense on T1, hyperintense on T2 with no diffusion restriction and extension into the suprasellar, interpeduncular and perimesencephalic cis-
Fig. 2. Postoperative CT scans (at 2 weeks, 2 and 6 months; column-wise from left to right): axial, sagittal and coronal cuts showing a
progressive decrease in the size of the cyst with the proximal end of the shunt in situ with a right frontoparietal chronic subdural hygroma.
Discussion
No cases of sylvian cistern arachnoid cysts presenting with precocious puberty have ever been reported in the literature. Clinical manifestations include epilepsy, features of raised intracranial pressure (diplopia, headache, apathy and vomiting) and focal neurological deficits [6]. Suprasellar arachnoid cysts (SSACs) are uncommon, accounting for 9% of all intracranial arachnoid cysts [7]. Isosexual precocity may be central or peripheral in origin. The probable explanation for central precocious puberty Sylvian Cistern Arachnoid Cyst
(CPP) is the premature release of LHRH from the hypothalamus, which in turn stimulates the secretion of pituitary gonadotropins. Pituitary gonadotropins like LH and FSH result in the secretion of gonadal sex steroids [8, 9]. An arachnoid cyst is a rare cause of CPP. One plausible theory suggests aqueductal compression by SSACs causing obstructive hydrocephalus and resultant hypothalamopituitary insufficiency. This hinders the homeostatic negative feedback mechanism resulting in continuous secretion of sex hormones [10]. Other causes for CPP include hypothalamic hamartomas, postencephalitic scars, tubercular meningitis, head trauma, hydrocephalus, tuberous sclerosis, porencephaly and suprasellar space-occupying lesions, including arachnoid cysts [11]. Elevated levels of FSH, LH and LHRH help to differentiate between CPP and peripheral precocious puberty. Early menarche, pubarche and thelarche along with radiological features (in the present case) point to the central origin of precocious puberty. Further, clinical and Pediatr Neurosurg 2013;49:365–368 DOI: 10.1159/000368323
367
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
anatomically as the sylvian subtype of supratentorial arachnoid cyst, Galassi type III. The patient was treated with a a cystoperitoneal shunt following which the cyst decreased in size (fig. 2). The child’s symptoms such as genital bleeding and breast growth also disappeared. At the 8-month follow-up, serum levels of LH (1.0 mIU/ml), FSH (0.9 mIU/ml) and estradiol (14.69 pg/ml) also returned to normal values.
biochemical improvement following cystoperitoneal shunt placement confirms the arachnoid cyst as the cause of precocious puberty. A careful neurological examination along with radiology is essential to rule out other causes of precocity. Radiologically, SSACs appear as extra-axial hypodense nonenhancing lesions. Various treatment modalities have been described for symptomatic supratentorial arachnoid cysts, including endoscopic fenestration [12], craniotomy and excision of the cyst lining (marsupialization) with optional ventriculoperitoneal shunting [13], a cysto-peritoneal shunt [6] and a percutaneous ventriculocystostomy [14]. Reports suggest that creating a single CSF space by surgically communicating the cyst with the ventricular system or basal cisterns offers the best chance of success in the treatment of SSACs. Occasionally, a shunt may still be required in hydrocephalic patients. Prognosis is usually good, with regression of hormonal imbalance postoperatively. However, in a few cases, postoperative complica-
tions may occur. These include persistence of the cyst or postoperative development of endocrinological disturbances attributed to hypothalamic damage secondary to ventriculocystostomy [14]. Partial excision of the cyst wall may obviate the risk of damage to the hypothalamic region and result in a favourable clinical response. In view of the principles involved in cyst fluid diversion, a cystoperitoneal shunt was attempted in our patient, and she responded well with significant reductions in follow-up hormonal levels (FSH/LH).
Conclusion
A sylvian cistern arachnoid cyst with temporal lobe agenesis causing CPP is very rare and calls for a vigilant clinical evaluation. Early and correct diagnosis, after ruling out peripheral and central causes of precocious puberty in the differential diagnosis, enables the surgeon to offer appropriate treatment with an excellent outcome.
References
368
6 Van Der Meche FGA, Braakman R: Arachnoid cysts in the middle cranial fossa: cause and treatment of progressive and non-progressive symptoms. J Neurol Neurosurg Psychiatry 1983;46:1102–1107. 7 Harsh NGR, Edwards MSB, Wilson CB: Intracranial arachnoid cysts in children. J Neurosurg 1986;64:835–842. 8 Rieth KG, Comite F, Dwyer AJ, et al: CT of cerebral abnormalities in precocious puberty. AJR Am J Roentgenol 1987;148:1231–1238. 9 Segall HD, Hasoun G, Ling SM, Cabton C: Suprasellar cysts associated with isosexual precocity. Radiology 1974;111:607–616. 10 Hochhaus F, Butenandt O, Schwarz HP, Ring-Mroz ik E: Auxological and endocrinological evaluation of children with hydrocephalus and/or meningomyelocele. Eur J Pediatr 1997;156:597–601.
Pediatr Neurosurg 2013;49:365–368 DOI: 10.1159/000368323
11 DiGeorge AM: The endocrine system; in Nelson WE, Vaughan VC, Behrman RE (eds): Nelson Textbook of Pediatrics, ed 14. Philadelphia, Saunders, 1992, pp 1407– 1414. 12 Okamoto K, Nakasu Y, Sato M, et al: Isosexual precocious puberty associated with multilocular arachnoid cysts at the cranial base. Report of a case. Acta Neurochir (Wien) 1981; 157:87–93. 13 Hoffman HJ, Hendrick EB, Humphreys RP, et al: Investigation and management of suprasellar arachnoid cysts. J Neurosurg 1982; 57: 597–602. 14 Pierre-Kahn A, Capelle L, Brauner R, et al: Presentation and management of suprasellar arachnoid cysts. Review of 20 cases. J Neurosurg 1990;73:355–359.
Upadhyaya/Nair/Kumar/Nayal/Shetty
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
1 Rengachary SS, Watanabe I: Ultrastructure and pathogenesis of intracranial arachnoid cysts. J Neuropathol Exp Neurol 1981;40:61– 88. 2 Rengachary SS: Intracranial arachnoid and ependymal cysts; in Wilkins RH, Rengachary SS (eds): Neurosurgery. New York, McGrawHill, 1985, pp 2160–2172. 3 Rengachary SS, Watanabe I, Brackett CE: Pathogenesis of intracranial arachnoid cysts. Surg Neurol 1978;9:139–144. 4 Osborn AG, Preece MT: Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology 2006;239:650–664. 5 Galassi E, Tognetti F, Gaist G, Fagioli L, Frank F, Frank G: CT scan and metrizamide CT cisternography in arachnoid cysts of the middle cranial fossa: classification and pathophysiological aspects. Surg Neurol 1982;17:363–369.
Received: June 20, 2014 Accepted after revision: September 7, 2014 Published online: November 21, 2014
Sylvian Cistern Arachnoid Cyst – A Rare Cause of Precocious Puberty Sunil Upadhyaya a Rajesh Nair a Vinod Kumar a Bhavna Nayal b Arjun Shetty a Departments of a Neurosurgery and b Pathology, Kasturba Medical College, Manipal University, Manipal, India
Established Facts • Sylvian cistern arachnoid cysts constitute 50% of all supratentorial cysts. • Early diagnosis and prompt treatment ensure excellent outcome.
Novel Insights • Sylvian cistern arachnoid cysts with temporal lobe agenesis causing central precocious puberty are very rare. • Though communicating a cyst with an adjacent ventricle or basal cistern is considered to assure surgical success with the rare hydrocephalic patient requiring a ventriculoperitoneal shunt, our patient did well with a cystoperitoneal shunt.
Abstract Precocious puberty still remains an elusive diagnosis in the majority of patients. Infrequently, lesions of the central nervous system are associated with sexual precocity. Depending on their location, these cysts may affect many systems, however, there is little information concerning their involvement in endocrinological disorders. We report a case of a sylvian cistern arachnoid cyst presenting with precocious puberty in a 3-year-old girl. The child recovered following a
© 2014 S. Karger AG, Basel 1016–2291/14/0496–0365$39.50/0 E-Mail [email protected] www.karger.com/pne
cystoperitoneal shunt. The mass effect of the arachnoid cyst upon the hypothalamus was, at least in part, responsible for the development of precocious puberty. To the best of our knowledge, this is the 1st case of a sylvian cistern arachnoid cyst presenting with precocious puberty. The role of surgical decompression of the cyst is also discussed. © 2014 S. Karger AG, Basel
Introduction
Arachnoid cysts are congenital developmental anomalies of the central nervous system associated with various clinical scenarios and long-term outcomes. They are reDr. Rajesh Nair, MS, FAGE, MCh Department of Neurosurgery Kasturba Hospital, Manipal University Manipal, Karnataka 576104 (India) E-Mail rajeshnair39 @ yahoo.com
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
Key Words Arachnoid cyst · Cystoperitoneal shunt · Precocious puberty · Sylvian cistern
duplicated arachnoid membranes with fluid collection resulting in cyst formation [1–3]. Arachnoid cysts are classified into [4]: (1) Supratentorial (a) Sylvian fissure (50%); (b) sellar region, and (c) interhemispheric/parasagittal (2) Infratentorial (a) Retrocerebellar and (b) laterocerebellar (3) Intraventricular/paraventricular (a) Quadrigeminal region/tentorial notch; (b) subependymal/paraventricular, and (c) choroid plexus cyst The Galassi classification system is one used for classifying arachnoid cysts in the middle cranial fossa [5]. Type I – small, spindle shaped, limited to the anterior middle cranial fossa, usually with free communication on CT cisternography. Type II – superior extent along the sylvian fissure with displacement of the temporal lobe. Type III – large, fills the whole middle cranial fossa, with displacement of not only the temporal lobe but also the frontal and parietal lobes; little communication with the subarachnoid space on CT cisternography.
366
Pediatr Neurosurg 2013;49:365–368 DOI: 10.1159/000368323
tern with midline shift and dilatation of the third and lateral ventricles, associated thinning of the hypothalamus and displacement of the pituitary stalk. FIESTA = Fast imaging employing steadystate acquisition; SOL = space occupying lesion.
Case Report A 3-year-old girl was admitted to the hospital for evaluation of accelerated development of secondary sexual characters and intermittent vaginal bleeding since 2 months. Four months prior to admission, her mother noticed that the child had vaginal bloodtinged white discharge. On physical examination, her height and weight were 110 cm and 24 kg, respectively, which exceeded five standard deviations from the mean level of the normal population. Her head circumference was 57 cm and chest circumference 63 cm. In addition, she had development of breast tissue classified as Tanner’s stage II and pubic hair (pubarche). The bone age by skeletal survey of the hand was rated as 3 years. Vaginal smear showed features compatible with menarche. Endocrinological examination showed elevated serum levels of luteinizing hormone (LH; 104.7 mIU/ml), follicle-stimulating hormone (FSH; 110.4 mIU/ml) and estradiol (146.78 pg/ml), which were equivalent to females at puberty. An LH-releasing hormone (LHRH) test revealed an excessive LH reaction. There were no definite neurological deficits. Magnetic resonance imaging (MRI) of the brain demonstrated the presence of a large arachnoid cyst (12 × 13.4 × 7.6 cm) involving the suprasellar region, an extra-axial right temporoparietal cystic lesion within the sylvian fissure, compressing the whole frontal, temporal and parietal lobe with right temporal pole agenesis. It appeared hypointense on T1 and hyperintense on T2, with no diffusion restriction and extension into the suprasellar, interpeduncular and perimesencephalic cistern, midline shift and dilatation of the third and lateral ventricles (fig. 1). There was associated thinning of the hypothalamus and displacement of the pituitary stalk. Our case, based on MRI findings, was categorized
Upadhyaya/Nair/Kumar/Nayal/Shetty
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
Fig. 1. MRI of the brain (T1 axial and sagittal, T2 axial and coronal, diffusion and FIESTA) showing an SOL which is hypointense on T1, hyperintense on T2 with no diffusion restriction and extension into the suprasellar, interpeduncular and perimesencephalic cis-
Fig. 2. Postoperative CT scans (at 2 weeks, 2 and 6 months; column-wise from left to right): axial, sagittal and coronal cuts showing a
progressive decrease in the size of the cyst with the proximal end of the shunt in situ with a right frontoparietal chronic subdural hygroma.
Discussion
No cases of sylvian cistern arachnoid cysts presenting with precocious puberty have ever been reported in the literature. Clinical manifestations include epilepsy, features of raised intracranial pressure (diplopia, headache, apathy and vomiting) and focal neurological deficits [6]. Suprasellar arachnoid cysts (SSACs) are uncommon, accounting for 9% of all intracranial arachnoid cysts [7]. Isosexual precocity may be central or peripheral in origin. The probable explanation for central precocious puberty Sylvian Cistern Arachnoid Cyst
(CPP) is the premature release of LHRH from the hypothalamus, which in turn stimulates the secretion of pituitary gonadotropins. Pituitary gonadotropins like LH and FSH result in the secretion of gonadal sex steroids [8, 9]. An arachnoid cyst is a rare cause of CPP. One plausible theory suggests aqueductal compression by SSACs causing obstructive hydrocephalus and resultant hypothalamopituitary insufficiency. This hinders the homeostatic negative feedback mechanism resulting in continuous secretion of sex hormones [10]. Other causes for CPP include hypothalamic hamartomas, postencephalitic scars, tubercular meningitis, head trauma, hydrocephalus, tuberous sclerosis, porencephaly and suprasellar space-occupying lesions, including arachnoid cysts [11]. Elevated levels of FSH, LH and LHRH help to differentiate between CPP and peripheral precocious puberty. Early menarche, pubarche and thelarche along with radiological features (in the present case) point to the central origin of precocious puberty. Further, clinical and Pediatr Neurosurg 2013;49:365–368 DOI: 10.1159/000368323
367
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
anatomically as the sylvian subtype of supratentorial arachnoid cyst, Galassi type III. The patient was treated with a a cystoperitoneal shunt following which the cyst decreased in size (fig. 2). The child’s symptoms such as genital bleeding and breast growth also disappeared. At the 8-month follow-up, serum levels of LH (1.0 mIU/ml), FSH (0.9 mIU/ml) and estradiol (14.69 pg/ml) also returned to normal values.
biochemical improvement following cystoperitoneal shunt placement confirms the arachnoid cyst as the cause of precocious puberty. A careful neurological examination along with radiology is essential to rule out other causes of precocity. Radiologically, SSACs appear as extra-axial hypodense nonenhancing lesions. Various treatment modalities have been described for symptomatic supratentorial arachnoid cysts, including endoscopic fenestration [12], craniotomy and excision of the cyst lining (marsupialization) with optional ventriculoperitoneal shunting [13], a cysto-peritoneal shunt [6] and a percutaneous ventriculocystostomy [14]. Reports suggest that creating a single CSF space by surgically communicating the cyst with the ventricular system or basal cisterns offers the best chance of success in the treatment of SSACs. Occasionally, a shunt may still be required in hydrocephalic patients. Prognosis is usually good, with regression of hormonal imbalance postoperatively. However, in a few cases, postoperative complica-
tions may occur. These include persistence of the cyst or postoperative development of endocrinological disturbances attributed to hypothalamic damage secondary to ventriculocystostomy [14]. Partial excision of the cyst wall may obviate the risk of damage to the hypothalamic region and result in a favourable clinical response. In view of the principles involved in cyst fluid diversion, a cystoperitoneal shunt was attempted in our patient, and she responded well with significant reductions in follow-up hormonal levels (FSH/LH).
Conclusion
A sylvian cistern arachnoid cyst with temporal lobe agenesis causing CPP is very rare and calls for a vigilant clinical evaluation. Early and correct diagnosis, after ruling out peripheral and central causes of precocious puberty in the differential diagnosis, enables the surgeon to offer appropriate treatment with an excellent outcome.
References
368
6 Van Der Meche FGA, Braakman R: Arachnoid cysts in the middle cranial fossa: cause and treatment of progressive and non-progressive symptoms. J Neurol Neurosurg Psychiatry 1983;46:1102–1107. 7 Harsh NGR, Edwards MSB, Wilson CB: Intracranial arachnoid cysts in children. J Neurosurg 1986;64:835–842. 8 Rieth KG, Comite F, Dwyer AJ, et al: CT of cerebral abnormalities in precocious puberty. AJR Am J Roentgenol 1987;148:1231–1238. 9 Segall HD, Hasoun G, Ling SM, Cabton C: Suprasellar cysts associated with isosexual precocity. Radiology 1974;111:607–616. 10 Hochhaus F, Butenandt O, Schwarz HP, Ring-Mroz ik E: Auxological and endocrinological evaluation of children with hydrocephalus and/or meningomyelocele. Eur J Pediatr 1997;156:597–601.
Pediatr Neurosurg 2013;49:365–368 DOI: 10.1159/000368323
11 DiGeorge AM: The endocrine system; in Nelson WE, Vaughan VC, Behrman RE (eds): Nelson Textbook of Pediatrics, ed 14. Philadelphia, Saunders, 1992, pp 1407– 1414. 12 Okamoto K, Nakasu Y, Sato M, et al: Isosexual precocious puberty associated with multilocular arachnoid cysts at the cranial base. Report of a case. Acta Neurochir (Wien) 1981; 157:87–93. 13 Hoffman HJ, Hendrick EB, Humphreys RP, et al: Investigation and management of suprasellar arachnoid cysts. J Neurosurg 1982; 57: 597–602. 14 Pierre-Kahn A, Capelle L, Brauner R, et al: Presentation and management of suprasellar arachnoid cysts. Review of 20 cases. J Neurosurg 1990;73:355–359.
Upadhyaya/Nair/Kumar/Nayal/Shetty
Downloaded by: Kungliga Tekniska Hogskolan 198.143.54.65 - 8/15/2015 8:41:21 PM
1 Rengachary SS, Watanabe I: Ultrastructure and pathogenesis of intracranial arachnoid cysts. J Neuropathol Exp Neurol 1981;40:61– 88. 2 Rengachary SS: Intracranial arachnoid and ependymal cysts; in Wilkins RH, Rengachary SS (eds): Neurosurgery. New York, McGrawHill, 1985, pp 2160–2172. 3 Rengachary SS, Watanabe I, Brackett CE: Pathogenesis of intracranial arachnoid cysts. Surg Neurol 1978;9:139–144. 4 Osborn AG, Preece MT: Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology 2006;239:650–664. 5 Galassi E, Tognetti F, Gaist G, Fagioli L, Frank F, Frank G: CT scan and metrizamide CT cisternography in arachnoid cysts of the middle cranial fossa: classification and pathophysiological aspects. Surg Neurol 1982;17:363–369.
