|Year : 2015 | Volume
| Issue : 1 | Page : 32-35
Arachnoid cyst presenting with endocrine manifestations: Tale of two patients
Babulreddy Hanmayyagari1, Mounika Guntaka2, Sridevi Patnala3, Prasun Deb4
1 ESI Hospital, Hyderabad, Andhra Pradesh, India
2 Department of Biochemistry, Prime Hospital, Hyderabad, Andhra Pradesh, India
3 Department of Endocrinology, Apollo Hospital, Secunderabad, Andhra Pradesh, India
4 Department of Endocrinology, Yashoda Hospital, Hyderabad, Andhra Pradesh, India
|Date of Web Publication||16-Mar-2015|
Dr. Babulreddy Hanmayyagari
Flat No. 507, Emerald Block, My Home Jewel, Madinaguda, Hyderabad - 500 049, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Arachnoid cysts are benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane. These are usually congenital and asymptomatic. We herein report endocrine manifestations associated with large intracranial arachnoid cyst in two of our patients, the first case presented with the combination of precocious puberty and growth hormone deficiency and the second case with precocious puberty; subsequently, we discuss the relevant literature on this association.
Keywords: Arachnoid cyst, cerebrospinal fluid, gonadotropin dependent precocious puberty, gonadotropin releasing hormone agonist, growth hormone deficiency
|How to cite this article:|
Hanmayyagari B, Guntaka M, Patnala S, Deb P. Arachnoid cyst presenting with endocrine manifestations: Tale of two patients. J NTR Univ Health Sci 2015;4:32-5
|How to cite this URL:|
Hanmayyagari B, Guntaka M, Patnala S, Deb P. Arachnoid cyst presenting with endocrine manifestations: Tale of two patients. J NTR Univ Health Sci [serial online] 2015 [cited 2020 Apr 6];4:32-5. Available from: http://www.jdrntruhs.org/text.asp?2015/4/1/32/153316
| Introduction|| |
Arachnoid cysts are benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane and that do not communicate with the ventricular system. They contain cerebrospinal fluid (CSF) secreted by arachnoid cells lining the cyst. Although the mechanism of cyst enlargement over time is not well-understood, theories include passive diffusion of CSF into the cyst or progressive entrapment due to a ball valve effect. , They are usually a congenital disorder and rarely due to infection or surgery. Most cases begin during infancy; however, onset may be delayed until adolescence. The male-to-female ratio is 2:1. 
Arachnoid cysts are generally located on the surface of the brain, usually close to the cerebral fissures within the anterior, middle and posterior fossa, but may occur in any part of the nervous system where there is arachnoid. They occur either as an isolated lesion or associated with other brain malformations, such as agenesis of the corpus callosum, absent cava, deficient cerebellar lobulation and Arnold-Chiari type I malformation. ,
Arachnoid cysts are usually asymptomatic, diagnosed incidentally on imaging for other reasons. Symptoms vary by the size and location of the cyst; common symptoms are due to raised intracranial pressure. Endocrine abnormalities occur rarely with supra-tentorial cysts. When they occur, they usually include growth hormone (GH) deficiency and precocious puberty.  They may cause occlusive hydrocephalus by compression of the fourth ventricle. , Morbidity and mortality depend on the location of the arachnoid cyst and on the occurrence of complications, such as acute mass effect by intracystic hemorrhage or the development of a subdural hygroma/hematoma.
Diagnosis is usually done by imaging. Computed tomography findings are consistent with CSF density mass with mild mass effect, without enhancement, unless there is concomitant hemorrhage or infection. Magnetic resonance imaging (MRI) similarly reveals CSF characteristics (low density on T1-weighted and high density on T2-weighted images) and no enhancement. Treatment is required only in symptomatic cases. Serial imaging and neurologic examinations are adequate if lesions are asymptomatic. Surgery is indicated when symptoms of raised intracranial pressure, seizures, focal neurologic deficits, or cognitive impairment occur. Surgical options include craniotomy for partial or complete cystectomy, fenestration into the subarachnoid space, or cyst peritoneal shunting.
We herein report two cases of arachnoid cyst with endocrine manifestations; the first case presented with the combination of precocious puberty and GH deficiency and the second case with precocious puberty; subsequently, we discuss about the relevant literature on this association.
| Case Reports|| |
A 6-year-old child [Figure 1] presented with the complaints of bilateral breast enlargement since 3 years. She was born of a 2 nd degree consanguineous marriage, full-term, normal delivery with no history of obstructed labor. Her motor and mental milestones were normal. Parents noticed breast development at 3 years of age, which has been rapidly progressive for the past 1 year. There was no history of axillary or pubic hair development, history of vaginal bleed, history of growth acceleration, history of intake of estrogen, or history of features suggestive of raised intracranial tension. On examination, height was 109 cm (height age-6 years) upper to lower segment ratio 1.05, arm span 107 cm, weight 15 kg (weight age-4 years). There were no neurocutaneous markers or bony abnormalities. Sexual maturity rating (SMR) was B3A1P1 and systemic examination was normal.
Investigations revealed normal routine biochemistry, T3 was 1.67 ng/ml, T4-8.37 mcg/dl, thyroid stimulating hormone-1.95 mIU/ml, ultrasound pelvis showed uterus-3.4 cm × 1.4 cm × 2.2 cm. Bone age was 10 years, luteinizing hormone (LH)-2.49 mIU/ml, estradiol-62.87 pg/ml and poststimulation GH-7 mcg/dl (with clonidine), MRI brain showed a large arachnoid cyst occupying fronto-temporo-parietal area with mass effect and obstructive hydrocephalus [Figure 2] and [Figure 3].
|Figure 2: Computed tomography scan is showing large arachnoid cyst with hydrocephalus|
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|Figure 3: Computed tomography scan is showing the extent of arachnoid cyst|
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Patient underwent cysto-peritoneal shunt surgery. Postoperative was uneventful and she was started on medroxyprogesterone acetate for precocious puberty and since her height velocity did not improve as expected, she was also started on GH therapy and she is under regular follow-up.
A 5-year-old girl was admitted in our hospital with chief complaints of headache and vomiting for 6 months and referred to the Department of Endocrinology as a child had bilateral breast enlargement for 7 months. Parents denied any development of pubic or axillary hair or any history of usage of estrogenic drugs, but noticed increased height velocity for 6 months and she was taller than her peers. On examination, height was 115 cm, weight was 19 kg, and neither neurocutaneous markers nor bony deformities were noted on general examination. Her sexual maturity scoring was B3, P2 and A1. Systemic examination was normal.
Routine biochemistry and thyroid function tests were normal, basal LH was 9.56 mIU/ml, estradiol 92 pg/ml, bone age was 8 years. MRI brain showed a large arachnoid cyst extending into the third ventricle with obstructive hydrocephalus [Figure 4].
|Figure 4: Magnetic resonance imaging is showing the extent of arachnoid cyst and hydrocephalus in our second patient|
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Then, the patient underwent right frontal craniotomy with transcortical and transventricular excision of arachnoid cyst under general anesthesia. In the postoperative period, child developed generalized seizures, which were treated with anticonvulsants. As a child had not shown any regression in secondary sexual characters, she was started on gonadotropin releasing hormone (GnRH) analogue therapy and the patient is doing well. At present, she is 12 years 6 months old and her height is 147 cm. GnRH analogue treatment was stopped recently as parents were concerned about puberty. Her SMR was B4, P3 and A3 when she last presented for review.
| Discussion|| |
Suprasellar arachnoid cysts are uncommon, accounting for 9% all intracranial arachnoid cysts.  They produce endocrine manifestations due to their close proximity to hypothalamo pituitary area.  As both our patients had large suprasellar arachnoid cysts, we could explain these endocrine abnormalities. Although, the variety of endocrine disorders can be associated with arachnoid cyst, the most common is sexual precocity , followed by GH deficiency.  It is distinctly rare to see the combination of sexual precocity and GH deficiency occurring in the same patient ,, as was observed with our first case.
Gonadotropin dependent sexual precocity is caused by premature activation of the hypothalamic pituitary gonadal axis, which is possibly related to the disruption of the neural pathway that normally inhibits the GnRH pulse generator. Though exact pathogenesis not known, it is hypothesized that gonadotropin dependent precocious puberty and GH deficiency occur in certain patients with hydrocephalus and is thought to be caused by the damage to the hypothalamus as a result of increased intracranial pressure or increased mass of CSF by the expanding arachnoid cyst. 
Available literature reveals that decompressive surgery is the cornerstone of therapy for symptomatic arachnoid cysts. , Hence, we did advocate surgery as the first line therapy for our patients. However, even after successful decompression, some patients continue to have endocrine dysfunction as seen in our patients and this is due to the permanent tissue damage that has occurred in hypothalamo-pituitary area before successful surgery. After the initiation of GnRH analog therapy, our second patient has shown appropriate growth velocity, whereas our first patient improved only after the initiation of GH replacement in addition to GnRH analog therapy. We are optimistic about the final height of this patient as she is doing well and gained 12 cm in 1 st year.
Hence, to conclude, in patients with suprasellar arachnoid cysts we suggest baseline and follow-up evaluation of growth and pubertal status, investigation of bone age, baseline follicle stimulating hormone, LH, estradiol and if needed, GnRH stimulation and GH provocation tests to rule out endocrine dysfunction. This approach is essential to start treatment at an opportune time before significant disability.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]