Auxological and endocrinological evaluation of children with hydrocephalus and /or meningomyelocele

Hochhaus, Frederike; Butenandt, O; Schwarz, Hans‐Peter; Ring-Mrozik, E.

doi:10.1007/s004310050672

Cited by 46 publications

(39 citation statements)

References 14 publications

(13 reference statements)

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“…8 Hydrocephalus may occasionally be associated with endocrinological abnormalities. A cross-sectional study conducted by Hochhaus et al 4 involved measurement of arm span and an endocrinological evaluation of several pediatric patients with one of the following: shunt-treated hydrocephalus of various origins, myelomeningocele (MMC) with shunt-treated hydrocephalus, and MMC without shunt-treated hydrocephalus. Approximately 40% of these patients demonstrated growth deficiency (as evidenced by short arm span), and more than 20% of these children were diagnosed with growth hormone insufficiency.…”

Section: Discussionmentioning

confidence: 99%

“…Interestingly, 12% of the study patients also exhibited precocious puberty-including 10% of patients with shunt-treated hydrocephalus (without MMC). 4 In fact, there is a well-documented relationship between CPP and hydrocephalus of various origins, such as MMC and other congenital CNS anomalies, and following meningitis and radiation therapy. 9,10,12,15,16 Our case is striking in that his examination was notable only for the abrupt onset of his secondary sex characteristics, a presentation that has not been described in medulloblastoma, and in its immediate reversal following neurosurgical intervention.…”

Section: Discussionmentioning

confidence: 99%

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Resolution of precocious puberty following resection of fourth ventricular medulloblastoma: case report

Medina

Dempsher

Gauvain

et al. 2015

PED

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Medulloblastoma is a malignant embryonal tumor that arises in the cerebellum and invades the fourth ventricle, often resulting in obstructive hydrocephalus. Patients typically present with symptoms related to increased intracranial pressure and cerebellar dysfunction. The authors report a rare case of classic medulloblastoma with central precocious puberty (CPP) as its only presenting symptom. A 7-year-old boy with no prior history of medulloblastoma presented with Tanner Stage IV testicular enlargement and a 4-month history of acne and pubic hair. Laboratory tests of blood samples demonstrated highly elevated luteinizing hormone (LH), follicle-stimulating hormone (FSH), and testosterone. Admission MRI of the brain revealed a mass in the posterior fossa, which bordered and compressed the fourth ventricle. The patient also exhibited mild lateral and third ventriculomegaly. Surgical options were discussed with the neurosurgical department. A suboccipital craniotomy and C-1 laminectomy were performed. A large mass was seen arising from the inferior surface of the vermis, and lying within the fourth ventricle. Gross-total microsurgical resection of the mass was performed. Histopathological investigation characterized the tumor as classic medulloblastoma. Follow-up laboratory tests of blood samples demonstrated a reduction of LH, FSH, and testosterone back to prepubertal levels. The patient then began radiation and chemotherapy. This report demonstrates that mild obstructive hydrocephalus due to a posterior fossa tumor may present with unexpected symptoms, such as CPP. To the authors’ knowledge, precocious puberty has not yet been associated with medulloblastoma, although it has been found with other posterior fossa tumors. Extensive imaging of the CNS for patients presenting with CPP is recommended.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Resolution of precocious puberty following resection of fourth ventricular medulloblastoma: case report

Medina

Dempsher

Gauvain

et al. 2015

PED

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“…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].…”

Section: Discussionmentioning

confidence: 99%

Sylvian Cistern Arachnoid Cyst - A Rare Cause of Precocious Puberty

Upadhyaya¹,

Nair²,

Kumar³

et al. 2013

Pediatr Neurosurg

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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 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.

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“…Although endocrine studies have been performed in children with MMC, most of them have focused on hydrocephalus resulting from MMC and proceeding pituitary dysfunction [50]. Several lines of evidence suggest a genetic component for NTDs [51].…”

Section: Discussionmentioning

confidence: 99%

Nonneural Congenital Abnormalities Concurring with Myelomeningocele: Report of 17 Cases and Review of Current Theories

et al. 2008

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Objective: Meningomyelocele (MMC) is a common central nervous system birth defect. Various congenital and acquired abnormalities have been reported with MMC, some of which are secondary to the pathophysiology and some are morbidities of the underlying disease. The aim of this study was to discuss current possible theories explaining diverse anomalies/abnormalities seen in a series of 390 patients with MMC. Methods: A retrospective study was performed using the records of 390 patients with MMC at Children’s Hospital Medical Center in Tehran, Iran, from January 2001 to January 2007. A series of 17 cases of MMC with attributed organ anomalies, not explained by a causal effect of the underlying disorder, were compiled. There were 3 cardiac anomalies including ventricular septal defect, pulmonary artery atresia and tetralogy of Fallot, 4 musculoskeletal malformations, consisting of missing rib, polydactylia and complex distal limb anomaly, 4 urological anomalies such as bladder exstrophy, horseshoe kidney and dysplastic kidneys, 2 occipital encephaloceles, 2 congenital adrenal hyperplasia patients with ambiguous genitalia, 1 omphalocele, 1 albinism and 1 Klippel-Feil syndrome. A review of the literature and discussion explaining each of these observations, have been performed and some possible theories have been proposed.Conclusions: Although various organ anomalies with different embryological origin had been observed and reported with MMC, it is difficult to explain their development using one of the current theories of MMC formation. It could be attributed to a possible genetic defect or merely an incidental finding. A teratological insult during the embryogenic phase would be an alternative assumption.

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Auxological and endocrinological evaluation of children with hydrocephalus and /or meningomyelocele

Cited by 46 publications

References 14 publications

Resolution of precocious puberty following resection of fourth ventricular medulloblastoma: case report

Resolution of precocious puberty following resection of fourth ventricular medulloblastoma: case report

Sylvian Cistern Arachnoid Cyst - A Rare Cause of Precocious Puberty

Nonneural Congenital Abnormalities Concurring with Myelomeningocele: Report of 17 Cases and Review of Current Theories

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