-
World Neurosurgery Oct 2019Intracranial epidermoid cysts are benign, congenital, keratinizing, squamous epithelial-lined cysts filled with keratin. They are uncommon and often pose a surgical...
BACKGROUND
Intracranial epidermoid cysts are benign, congenital, keratinizing, squamous epithelial-lined cysts filled with keratin. They are uncommon and often pose a surgical challenge owing to the adherence to surrounding structures. They are typically found at the cerebellopontine angle or in the parasellar region, where they are associated with abnormal development of the Rathke pouch; involvement of the pituitary stalk is rare.
CASE DESCRIPTION
The patient's electronic health record was queried for relevant data. A systematic review of the literature using dedicated search terms for cases of infundibular epidermoid cysts was conducted. We present a unique case of a 55-year-old male who presented with vision changes and was found to have a parasellar epidermoid cyst confined to the pituitary stalk. The patient underwent endoscopic transsphenoidal resection, and gross total resection was achieved. The patient's postoperative course was significant for possible chemical meningitis and the development of panhypopituitarism. The patient's vision subjectively improved after surgery.
CONCLUSIONS
Although 3 other cases of epidermoid cysts involving the pituitary stalk were identified, our patient's tumor was unique in that it was confined to the stalk. Our patient's case highlights a surgical approach to parasellar epidermoid cysts and the possible complications associated therewith.
Topics: Epidermal Cyst; Humans; Male; Middle Aged; Pituitary Gland
PubMed: 31284058
DOI: 10.1016/j.wneu.2019.06.205 -
World Neurosurgery Jul 2019The optimal surgical strategy for management of adult patients with craniopharyngioma remains controversial. To analyze the functional outcomes ofadult patients with... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The optimal surgical strategy for management of adult patients with craniopharyngioma remains controversial. To analyze the functional outcomes ofadult patients with gross total resection (GTR) and subtotal resection (STR) of craniopharyngioma.
METHODS
MEDLINE, EMBASE, Scopus, and Cochrane databases were searched from inception to July 19, 2018, for articles comparing postoperative endocrine function, vision, complications, and recurrence rates for adult patients with GTR and STR of craniopharyngioma. The articles were analyzed by meta-analysis of proportions using a random-effects model to calculate summary odds ratios (ORs).
RESULTS
The initial search resulted in 2468 studies and 540 studies selected for full text review. Seventeen studies were included in the final analyses with 748 patients in the GTR cohort and 559 patients in the STR cohort. GTR resulted in a significantly lower likelihood of recurrence when compared with STR (OR, 0.106; 95% confidence interval [CI], 0.067-0.168; P < 0.001), but a significantly greater likelihood of panhypopituitarism (OR, 2.063; 95% CI, 1.058-4.024; P = 0.034) and permanent diabetes insipidus (OR, 2.776; 95% CI, 1.321-5.832; P = 0.007). There was no significant difference between the groups for postoperative worsened vision (P = 0.868), improved vision (P = 0.876), pathologic weight gain (P = 0.724), cerebrospinal fluid leak (P = 0.788), complications (P = 0.656), or death (P = 0.261).
CONCLUSIONS
This is the first systematic review of functional outcomes of adult patients with craniopharyngioma. GTR results in decreased likelihood of recurrence, but increased likelihood of postoperative panhypopituitarsm and permanent diabetes insipidus. Surgeons should be aware of these associations when determining the optimal operative strategy for adult patients with craniopharyngioma.
Topics: Adult; Craniopharyngioma; Endocrine System Diseases; Female; Humans; Male; Pituitary Neoplasms; Postoperative Complications; Treatment Outcome; Vision Disorders
PubMed: 30947004
DOI: 10.1016/j.wneu.2019.03.239 -
Child's Nervous System : ChNS :... Jul 2019Hydranencephaly is a congenital condition characterized by the complete or near-complete absence of the cerebral cortex and basal ganglia, while central diabetes...
PURPOSE
Hydranencephaly is a congenital condition characterized by the complete or near-complete absence of the cerebral cortex and basal ganglia, while central diabetes insipidus (CDI) is a condition characterized by the inability to concentrate urine due to a deficiency in antidiuretic hormone (ADH). CDI is known to occur in midline congenital malformations such as holoprosencephaly and septo-optic dysplasia, but its association with hydranencephaly is less well-established.
METHODS
We reported two cases of hydranencephaly complicated by CDI. We also performed a systematic review of the SCOPUS and PubMed databases for case reports and case series of patients with hydranencephaly and CDI, and compiled data on the clinical features and treatment options.
RESULTS
Seven cases of hydranencephaly complicated by CDI were identified from the systematic review in addition to the two cases reported here, resulting in a total of nine cases. The patients' age ranged from 4 days to 4 years, and there was a female sex predilection (3.5:1). Patients most commonly presented with macrocephaly, developmental delay, and seizures, with dysmorphic features noted in 33%. In addition to CDI, other endocrinologic derangements included hypothyroidism (22%), hypocortisolemia (22%), and panhypopituitarism (22%). CDI was treated using sublingual or oral desmopressin while hypopituitarism was treated with the appropriate hormone replacement therapy. Insertion of a ventriculoperitoneal (VP) shunt was reported in 44% of cases.
CONCLUSION
The case reports and systematic review suggest a previously unknown association between hydranencephaly and CDI. Clinicians managing cases of hydranencephaly are advised to have a high index of suspicion for CDI in patients presenting with the characteristic signs and symptoms.
Topics: Diabetes Insipidus, Neurogenic; Female; Humans; Hydranencephaly; Infant; Tomography, X-Ray Computed; Treatment Outcome; Ventriculoperitoneal Shunt
PubMed: 30929071
DOI: 10.1007/s00381-019-04137-9 -
Clinical Neurology and Neurosurgery Jun 2018It is unclear whether the endoscopic technique has any advantages over transcranial approach in the aspect of endocrine outcomes in patients with craniopharyngiomas,... (Comparative Study)
Comparative Study Meta-Analysis
It is unclear whether the endoscopic technique has any advantages over transcranial approach in the aspect of endocrine outcomes in patients with craniopharyngiomas, thus, we conducted a systematic review and meta-analysis. We conducted a comprehensive search of PubMed to identify relevant studies. Pituitary, hypothalamus functions and recurrence were used as outcome measures. A total of 39 cohort studies involving 3079 adult patients were included in the comparison. Among these studies, 752 patients across 17 studies underwent endoscopic transsphenoidal resection, and 2327 patients across 23 studies underwent transcranial resection. More patients in the endoscopic group (75.7%) had visual symptoms and endocrine symptoms (60.2%) than did patients in the transcranial group (67.0%, p = 0.038 and 42.0%, p = 0.016). There was no significant difference in hypopituitarism and pan-hypopituitarism after surgery between the two groups: 72.2% and 43.7% of the patients in endoscopic group compared to 80.7% and 48.3% in the transcranial group (p = 0.140 and p = 0.713). We observed same proportions of transient and permanent diabetes insipidus in both groups. Similar recurrence was observed in both groups (p = 0.131). Pooled analysis showed that neither weight gain (p = 0.406) nor memory impairment (p = 0.995) differed between the two groups. Meta-regression analysis revealed that gross total resection contributed to the heterogeneity of recurrence proportion (p < 0.001). We observed similar proportions of endocrine outcomes and recurrence in both endoscopic and transcranial groups. More recurrences were observed in studies with lower proportions of gross total resection.
Topics: Cohort Studies; Craniopharyngioma; Craniotomy; Endocrine System Diseases; Humans; Neuroendoscopy; Pituitary Neoplasms; Treatment Outcome
PubMed: 29655011
DOI: 10.1016/j.clineuro.2018.04.009 -
Endocrine, Metabolic & Immune Disorders... Nov 2017The current meta-analysis aims at evaluating whether the existing clinical evidence may ascertain the effects of growth hormone (GH) replacement therapy on... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND AND OBJECTIVE
The current meta-analysis aims at evaluating whether the existing clinical evidence may ascertain the effects of growth hormone (GH) replacement therapy on cardiovascular risk, both in isolated GH deficiency (GHD) and in compensated panhypopituitarism including GH deficit.
METHODS
Original articles published from 1991 to 2015 were searched on Medline (Pubmed). Among an overall number of 181 potentially suitable studies, 24 fulfilled the selection criteria and were included in the analysis. Data aggregation was carried out through the calculation of the absolute risk reduction. The meta-analysis was then conducted by means of a fixed-effects model, according to the heterogeneity test (Chi-square statistic).
RESULTS
Fat-free mass (FFM) increase and fat mass (FM) reduction were found, together with a C-LDL reduction, a wide variation in glycaemia and a neutral effect on glycated haemoglobin (HbA1c) and blood pressure. These effects were valid both for isolated GHD patients and for those with compensated panhypopituitarism. The global outcome D showed a nonsignificant reduction of the overall cardiovascular risk (0.53; 95% C.I. -1.23, 2.85).
CONCLUSION
Our meta-analysis shows no signnificatly positive trend in cardiovascular risk after both short and long-term GH supplementation therapy in adult GHD patients. However, a reduction of LDL cholesterol levels has been found. No differences were found between isolated GHD participants and those affected by panhypopituitarism well compensated since at least 3 months.
Topics: Cardiovascular Diseases; Dietary Supplements; Dwarfism, Pituitary; Hormone Replacement Therapy; Human Growth Hormone; Humans; Risk Factors; Treatment Outcome
PubMed: 28925898
DOI: 10.2174/1871530317666170919121729 -
Clinical Endocrinology Dec 2015Combined pituitary hormonal deficiency (CPHD) can result from mutations within genes that encode transcription factors. This study evaluated the frequency of mutations... (Review)
Review
OBJECTIVE
Combined pituitary hormonal deficiency (CPHD) can result from mutations within genes that encode transcription factors. This study evaluated the frequency of mutations in these genes in a cohort of 144 unrelated Italian patients with CPHD and estimated the overall prevalence of mutations across different populations using a systematic literature review.
MATERIAL AND METHODS
A multicentre study of adult and paediatric patients with CPHD was performed. The PROP1, POU1F1, HESX1, LHX3 and LHX4 genes were analysed for the presence of mutations using direct sequencing. We systematically searched PubMed with no date restrictions for studies that reported genetic screening of CPHD cohorts. We only considered genetic screenings with at least 10 individuals. Data extraction was conducted in accordance with the guidelines set by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).
RESULTS
Global mutation frequency in Italian patients with CPHD was 2·9% (4/136) in sporadic cases and 12·5% (1/8) in familial cases. The worldwide mutation frequency for the five genes calculated from 21 studies was 12·4%, which ranged from 11·2% in sporadic to 63% in familial cases. PROP1 was the most frequently mutated gene in sporadic (6·7%) and familial cases (48·5%).
CONCLUSION
The frequency of defects in genes encoding pituitary transcription factors is quite low in Italian patients with CPHD and other western European countries, especially in sporadic patients. The decision of which genes should be tested and in which order should be guided by hormonal and imaging phenotype, the presence of extrapituitary abnormalities and the frequency of mutation for each gene in the patient-referring population.
Topics: Female; Genetic Predisposition to Disease; Homeodomain Proteins; Humans; Hypopituitarism; Italy; LIM-Homeodomain Proteins; Male; Transcription Factor Pit-1; Transcription Factors
PubMed: 26147833
DOI: 10.1111/cen.12849 -
Pituitary Dec 2015Hypopituitarism is a possible complication of the surgical treatment of acromegaly. However, there is a wide variability in the incidence rates of surgery-induced... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Hypopituitarism is a possible complication of the surgical treatment of acromegaly. However, there is a wide variability in the incidence rates of surgery-induced hypopituitarism. The purpose of this study was the systematic collection and synthesis of information on the incidence rates of hypopituitarism, panhypopituitarism, specific axis deficiencies and diabetes insipidus after surgery for acromegaly treatment.
METHODS
We systematically reviewed all the papers that have reported pituitary deficits after surgery for acromegaly published up until December 2014, in the PubMed database. We identified 92 studies enrolling 6988 patients. A meta-analysis was performed to evaluate the incidence rates. We also performed several subgroup analyses to evaluate the impact of both surgical technique, and treatment prior to surgery, on the results.
RESULTS
The weighted incidence rates were 12.79 % for hypopituitarism (95 % CI 9.88-16.00 %), 2.50 % for panhypopituitarism (95 % CI 1.24-4.15 %), 6.50 % for ACTH deficiency (95 % CI 4.07-9.44 %), 4.39 % for TSH deficiency (95 % CI 2.99-6.04 %), 6.70 % for FSH/LH deficiency (95 % CI 3.89-10.17 %), 14.95 % for GH deficiency (95 % CI 7.25-24.64 %), 10.05 % for transient (95 % CI 7.18-13.33 %) and 2.42 % for permanent diabetes insipidus (95 % CI 1.70-3.27 %).
CONCLUSION
Our study provides new data on the incidence rates of hypopituitarism, specific pituitary axis deficiencies and diabetes insipidus after surgical treatment of acromegaly. Somatotroph function appears to be more prone to deficit than the other axes. However, there is a high heterogeneity between studies and several factors may influence the incidence of hypopituitarism.
Topics: Acromegaly; Diabetes Insipidus; Humans; Hypopituitarism; Treatment Outcome
PubMed: 26113357
DOI: 10.1007/s11102-015-0661-6 -
Pituitary Aug 2014Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary. There is debate in the scientific literature as to whether IGH represents a... (Review)
Review
Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary. There is debate in the scientific literature as to whether IGH represents a continuum of disease with lymphocytic hypophysitis or has a distinct pathogenesis. Due to the rare nature of the disease, previous descriptions have been limited to single case reports or small series. In the present study, a systematic review of the literature was performed for cases of IGH. 82 cases met inclusion criteria. Data was gathered on IGH clinical aspects, in order to elucidate any associations useful in determining pathogenesis, appropriate clinical treatment, or prognosis. Univariate and multivariate analysis was performed on available data. Female sex was significantly associated with IGH (p < 0.0001). Fever (p = 0.002), nausea or vomiting at presentation (p = 0.031), and histological evidence of necrosis (p = 0.022) correlated with reduced time to presentation. Panhypopituitarism at presentation predicted need for long term hormone replacement (p = 0.014). Hyperprolactinaemia (p = 0.032), normal gonadal (p = 0.037) and thyroid axes (p = 0.001) were associated with reduced likelihood of long-term hormone replacement. Anorexia (p = 0.017), cold intolerance (p = 0.046), and fatigue (p = 0.0033) were associated with death from IGH. Patients who had excisional surgery alone trended towards increased rates of symptom resolution, compared with patients who received corticosteroids as an adjunct to excisional surgery (p = 0.11). This article details the first systematic review of IGH, and presents evidence for a female predilection of the disease. Implications for pathogenesis, and a suggested clinical approach are discussed. An online disease registry has been established to facilitate further IGH research.
Topics: Female; Humans; Hypopituitarism; Male; Pituitary Diseases; Pituitary Neoplasms
PubMed: 23990347
DOI: 10.1007/s11102-013-0510-4 -
Acta Neurologica Belgica Sep 2013The objective of this study was to review patients with intrasellar cysticercosis to outline the features of this form of neurocysticercosis. A MEDLINE and manual search... (Review)
Review
The objective of this study was to review patients with intrasellar cysticercosis to outline the features of this form of neurocysticercosis. A MEDLINE and manual search of patients with intrasellar cysticercosis were done. Abstracted data included clinical manifestations, neuroimaging findings, therapy, and outcome. Twenty-three patients were reviewed. Ophthalmological disturbances, including diminution of visual acuity and visual field defects following a chiasmatic pattern, were recorded in 67 % of cases. Endocrine abnormalities were found in 56 % of patients (panhypopituitarism, hyperprolactinemia, diabetes insipidus, and isolated hypothyroidism). In addition, some patients complained of seizures or chronic headaches. Neuroimaging studies showed lesions confined to the sellar region in 47 % of cases. The remaining patients also had subarachnoid cysts associated or not with hydrocephalus, parenchymal brain cysts, or parenchymal brain calcifications. Thirteen patients underwent surgical resection of the sellar cyst through a craniotomy in nine cases and by the transsphenoidal approach in four. Visual acuity or visual field defects improved in only two of these patients. Five patients were treated with cysticidal drugs without improvement. Intrasellar cysticercosis is rare and probably under-recognized. Clinical manifestations resemble those caused by pituitary tumors, cysts, or other granulomatous lesions. Neuroimaging findings are of more value when intrasellar cysts are associated with other forms of neurocysticercosis, such as basal subarachnoid cysts or hydrocephalus. Prompt surgical resection is mandatory to reduce the risk of permanent loss of visual function. There seems to be no role for cysticidal drug therapy in these cases.
Topics: Humans; MEDLINE; Neurocysticercosis; Pituitary Diseases; Sella Turcica; Vision Disorders
PubMed: 23605125
DOI: 10.1007/s13760-013-0199-1 -
Journal of Neurosurgery. Pediatrics Oct 2012Craniopharyngiomas are benign tumors but their close anatomical relationship with critical neurological, endocrine, and vascular structures makes gross-total resection... (Comparative Study)
Comparative Study Review
OBJECT
Craniopharyngiomas are benign tumors but their close anatomical relationship with critical neurological, endocrine, and vascular structures makes gross-total resection (GTR) with minimal morbidity difficult to achieve. Currently, there is controversy regarding the extent, timing, and modality of treatment for pediatric craniopharyngioma.
METHODS
The authors performed a systematic review of the published literature on pediatric craniopharyngioma to determine patterns of clinical practice and the reported outcomes of standard treatment strategies. This yielded 109 studies, which contained data describing extent of resection for a total of 531 patients. Differences in outcome were examined based upon extent of resection and choice of radiation treatment.
RESULTS
Gross-total resection was associated with increased rates of new endocrine dysfunction (OR 5.4, p < 0.001), panhypopituitarism (OR 7.8, p = 0.006), and new neurological deficits (OR 9.9, p = 0.03) compared with biopsy procedures. Subtotal resection (STR) was not associated with an increased rate of new neurological deficits. Gross-total was associated with increased rates of diabetes insipidus (OR 7.7, p = 0.05) compared with the combination of STR and radiotherapy (RT). The addition of RT to STR was associated with increased rates of panhypopituitarism (OR 9.9, p = 0.01) but otherwise similar rates of morbidities.
CONCLUSIONS
Although subject to the limitations of a literature review, this report suggests that GTR is associated with increased rates of endocrinopathies compared with STR + RT, and this should be considered when planning goals of surgery.
Topics: Adolescent; Biopsy; Chi-Square Distribution; Child; Child, Preschool; Craniopharyngioma; Humans; Hypophysectomy; Hypopituitarism; Infant; Morbidity; Neoplasm Recurrence, Local; Pituitary Neoplasms; Radiotherapy, Adjuvant; Retrospective Studies; Young Adult
PubMed: 22920295
DOI: 10.3171/2012.7.PEDS11436