Suprasellar arachnoid cyst resulting in the syndrome of inappropriate antidiuretic hormone secretion

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is occasionally seen after hypothalamic injury or dysfunction, although it typically occurs in association with other endocrine disturbances. It is has never been described as a presenting feature of a suprasellar arachnoid cyst (SAC) in the pediatric population. The authors describe the case of an enlarging SAC resulting in SIADH as the only presenting feature, with an otherwise normal hypothalamic-pituitary axis. An SAC was diagnosed in utero in this 5-month-old girl who had a normal functioning hypothalamic-pituitary axis on presentation. Because of cyst enlargement and hydrocephalus, the patient was scheduled for surgery; however, preoperative labs revealed SIADH. After stabilizing the serum sodium concentration with fluid restriction and the administration of 3% sodium chloride, the patient underwent endoscopic cyst fenestration. Postoperatively, she had complete resolution of the SIADH. Syndrome of inappropriate antidiuretic hormone secretion as the presenting symptom of an SAC has not been previously described. In the aforementioned patient, the proposed mechanism for SIADH was enlargement of the suprasellar arachnoid cyst causing compression of the supraoptic and paraventricular nuclei and thus overstimulating the secretion of arginine vasopressin, which resulted in SIADH. The association of SIADH with an SAC is reportable, as is the resolution of the SIADH via cyst fenestration. The authors suggest that SIADH is an uncommon presenting feature of SACs and that syndrome resolution is possible with cyst decompression.     

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

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 μg/l) in 16 patients, confirmed by a second evaluation in 8/11 of them. The plasma free thyroxine was low in five 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 deficiency. 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 deficiencies 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. Received: 5 May 1999 / Accepted: 28 October 1999     

Precocious puberty caused by a suprasellar arachnoid cyst. Analysis of 6 cases

The authors report 6 cases (4 girls and 2 boys) with central precocious puberty associated with a suprasellar arachnoid cyst. Precocious puberty is rarely the presenting sign of arachnoid cysts. It was characterized by early onset, patent symptoms and frequent association (3 of 6 cases) with growth hormone deficiency. The latter represents a further risk of short stature. Evolution of precocious puberty varied from one case to another, without any relation with the quality of control of the arachnoid cyst and associated hydrocephalus. Half-yearly follow-up of height and bone age allowed for deciding a suppressive treatment of precocious puberty and a substitutive growth hormone therapy when needed.     

Spinal intradural arachnoid cyst

The authors report the case of a 13 year-old boy who presented after minor cranial trauma with severe gastrointestinal symptoms (acute hemorrhage) followed by neurologic signs evoking a spinal compression. Gastro-intestinal hemorrhage was related to a duodenal lesion induced by acetylsalicylic acid. Neurologic symptoms were related to an intra dural cervical arachnoid cyst. After surgical resection of this congenital malformation outcome was favorable.     

Spinal intramedullary arachnoid cyst

Spinal arachnoid cysts are a relatively uncommon lesion. They may be either intra or extradural; intradural cysts being less common. Symptomatic spinal arachnoid cysts in the pediatric age group are rare. To the best of our knowledge only 2 cases of intramedullary arachnoid cysts have been reported to date. We report an unusual case of intramedullary cyst diagnosed in a 10-year-old female who presented with progressive quadriparesis. MRI scans revealed an intramedullary cystic lesion from C4 to T2. She underwent a C3 to T2 laminectomy with partial excision of the cyst. Histopathological findings confirmed the diagnosis of an arachnoid cyst. Postoperatively the patient showed dramatic recovery with marked improvement in neurological status. Arachnoid cysts should be considered in the differential diagnosis of progressive quadri / paraparesis in the pediatric age group as surgical decompression leads to marked improvement in the symptoms.     

Congenital arachnoid cyst mimicking meningocele

The subject of this report is a rare case of a 5-year-old girl who developed an arachnoid cyst with a bony and dural defect in the parietal convexity. She had no history of head trauma or infection. Surgical exploration revealed the bulging lesion to consist of cerebrospinal fluid-containing spongy subcutaneous tissue and to extend into the bony and dural defect. The arachnoid cyst cavity was found beneath the subcutaneous lesion and was not connected to the adjacent subarachnoid space. Histologically, the subcutaneous tissue contained a complex of sinusoidal channels formed by an abundance of migrating arachnoidal cells, thus mimicking meningocele.     

Spinal intramedullary arachnoid cyst in children

The authors report an unusual case of a child who presented with progressive paraparesis that lasted 15 days, revealing an intramedullary cystic lesion extending from T(3) to T(4) as detected with spine magnetic resonance imaging. A laminotomy from T(3) to T(4) was performed and the lesion removed. Histopathological findings confirmed the diagnosis of arachnoid cyst. After surgery, the patient's neurological status improved. At the 4-month follow-up examination, the results of his neurological examinations remained normal. Arachnoid cyst is an entity that should be included in the differential diagnosis of intramedullary cystic lesions.     

Suprasellar germ cell tumor presenting as diencephalic syndrome and precocious puberty

We report a 7-9/12 year-old boy presenting with precocious puberty and diencephalic syndrome. On investigation a suprasellar germ cell tumor was found. This is an uncommon tumor causing this rare syndrome and an unusual presentation.     

Diagnosis of delayed puberty]

Puberty is the phenomenon that conducts once to reproductive maturation. Delayed puberty (DP) is defined by the absence of testicular development in boys beyond 14 years old (or a testicular volume lower than 4 ml) and by the absence of breast development in girls beyond 13 years old. DP occurs in approximatively 3% of cases. Most cases are functional DP, with a large amount of constitutional delay of puberty. Others etiologies are hypogonadotrophic hypogonadism like Kallmann syndrome, or hypergonadotrophic hypogonadism. Turner syndrome is a diagnostic one should not forget by its frequency. Treatment is hormonal replacement therapy and of the etiology. During the last decade, many genes have been identified and elucidated the etiological diagnosis of some hypogonadotrophic hypogonadism syndrome. Further studies are required in collaboration with molecular biologists to better understand the mechanism of hypothalamic pituitary gonadal axis abnormalities and of the neuroendocrine physiology of the onset of puberty.     

Lateral intrathoracic meningocele associated with a spinal intradural arachnoid cyst

A case of lateral intrathoracic meningocele associated with a spinal intradural arachnoid cyst is reported and the cases from the literature are reviewed. Both of these lesions were detected by magnetic resonance imaging and treated surgically. Intrathoracic meningoceles are often asymptomatic and diagnosed incidentally during the evaluation of an unrelated pathology. A patient with a spinal intradural arachnoid cyst may present with paraparesis mimicking an intradural tumor, and lateral intrathoracic meningocele may seldom accompany this lesion. A comprehensive radiological examination must be conducted and a higher index of suspicion by neurosurgeons is necessary.