[Prospective analysis of inflammatory markers and perioperative clinical data in children with craniosynostosis undergoing reconstructive surgery]. / Prospektivnyi analiz dinamiki laboratornykh markerov vospaleniya i klinicheskoi kartiny perioperatsionnogo perioda pri rekonstruktivnykh operatsiyakh u detei s kraniosinostozami.

BACKGROUND: Craniosynostosis (CS) is a group of skull malformations manifested by congenital absence or premature closure of cranial sutures. Reconstructive surgery in the second half of life is traditional approach for CS. The issues of surgical stress response after reconstructive surgery for CS in children are still unclear. OBJECTIVE: To evaluate clinical and laboratory parameters in children undergoing traumatic reconstructive surgery for CS. MATERIAL AND METHODS: Inclusion criteria were CS, reconstructive surgery, age <24 months, no comorbidities and available laboratory diagnostic protocol including complete blood count, biochemical blood test with analysis of C-reactive protein, procalcitonin, ferritin and presepsin. The study included 32 patients (24 (75%) boys and 8 (25%) girls) aged 10.29±4.99 months after surgery between October 2021 and June 2022. Non-syndromic and syndromic forms of CS were observed in 25 (78.1%) and 7 (21.9%) cases, respectively. RESULTS: There were no infectious complications. We analyzed postoperative clinical data, fever, clinical and biochemical markers of inflammation. CONCLUSION: Early postoperative period after reconstructive surgery for CS in children is accompanied by significant increase of inflammatory markers (C-reactive protein, procalcitonin, ferritin). However, these findings do not indicate infectious complications. This is a manifestation of nonspecific systemic reaction. Severity of systemic inflammatory response syndrome with increase in acute phase proteins indicates highly traumatic reconstructive surgery for CS in children. Analysis of serum presepsin allows for differential diagnosis between infectious complication and uncomplicated course of early postoperative period.

[Stereotactic radiosurgery for epilepsy related to hypothalamic hamartoma]. / Stereotaksicheskaya radiokhirurgiya pri epilepsii u patsientov s gamartomoi gipotalamusa.

Hypothalamic hamartoma (HH) is a dysplastic lesion fused with hypothalamus and followed by epilepsy, precocious puberty and behavioral disorders. Up to 50% of patients become free of seizures after surgery, but various complications occur in 1/4 of cases. Radiofrequency thermocoagulation, laser interstitial thermal therapy and stereotactic radiosurgery (SRS) are alternative treatment options. OBJECTIVE: To define the indications for SRS in patients with HH and to clarify the irradiation parameters. MATERIAL AND METHODS: Twenty-two patients with HH and epilepsy underwent SRS at the Moscow Gamma-knife Center. A retrospective analysis included 19 patients with sufficient follow-up data. Median age of patients was 11.5 years (range 1.3-25.8). The diameter of irradiated HHs ranged between 5.5 and 40.9 mm. In 8 (36%) cases, the volume of hamartoma exceeded 3 cm3. Mean prescribed dose was 18±2.0 Gy, mean prescribed isodose - 48±4.2%. Median follow-up period was 14.8 months (range 3.4 - 96.1). RESULTS: Three (15.8%) patients were free of seizures. One patient (5.3%) improved dramatically after treatment with compete resolution of generalized seizures and experienced only rare emotional seizures (Engel IB). Eleven (57.8%) patients reported lower incidence of seizures. Severity and incidence of seizures were the same in 4 patients (21.1%). The best results were achieved in mean target dose over 20-22 Gy, minimal target dose over 7-10 Gy, covering by the prescribed dose of at least 70-80% of hamartoma volume, as well as in patients with the prescribed dose of 12 Gy delivered to almost entire volume of tumor. None patient had any complications after SRS. CONCLUSION: SRS is safe regarding neurological, endocrine or visual disturbances. Careful patient selection for SRS makes it an effective option for HH-related epilepsy. The best candidates for SRS are children with seizures aged over 1 year, hamartoma <3 cm3 and area of fusion with hypothalamus <150 mm2.

[Hepatopulmonary syndrome: a rare manifestation of cirrhosis in patient with diencephalic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma].

We describe a 15-year girl, who developed panhypopituitarism and diencephalic obesity after surgical excision of craniopharyngioma, followed by nonalcoholic fatty liver disease and cirrhosis 5 years after surgery. Cirrhosis in this case manifested by hypoxia due to hepatopulmonary syndrome, and despite cure of craniopharyngioma by surgery and radiosurgery treatment and adequate hormonal substitution therapy patient died 9 years after surgery. Growth hormone substitutional therapy in patients with hypopituitarism, and steatohepatitis may decrease liver triglyceride accumulation and prevent end-stage liver disease.

[Endocrine late-effects and bone mineral density after combined treatment of malignant brain tumors in childhood and adolescence].

BACKGROUND: The implementation of standardized protocols for combined treatment of cancer into clinical practice inevitably leads to a long-term consequence. AIMS: To study the prevalence of endocrine disorders, to assess the prevalence and degree of decline of bone mineral density (BMD) in individuals who have undergone combined treatment of malignant brain tumors in childhood and adolescence. MATERIALS AND METHODS: A retrospective study was conducted with 59 young adults (31 men; 28 women) who have undergone surgical treatment of malignant brain tumour followed by radiation treatment (craniospinal radiation in combination with or without polychemotherapy). Group I consisted of 37 patients, who were treated between the ages of 3 and 16 years. Group II included 22 patients who received treatment between the ages of 16 and 38 years. RESULTS: GH deficiency according to the results of the insulin hypoglycemia test was diagnosed in 48 patients (81%), 22 -patients had secondary adrenal insufficiency (37%). The majority of those examined (33 patients (56%)) did not achieve the target growth. Only 5 people from I group was treated with recombinant GH. Correlation analysis demonstrates that age of treatment is the main factor affecting final growth (r=0,619, p<0,001). Many cases of hypothyroidism (n=39 (66%)) and hypogonadism (19 women; 17 men) were detected. According to the DXA, a decrease of BMD ≤-2.0 SD (Z-score) in L1-L4 was found in 35 of 59 patients (59%). The BMD in the I group was significantly lower than in patients treated at an older age (p<0.001). A moderate correlation was discovered between BMD in L1-L4 at the time of examination and the level of estradiol in women (r=0.596, p<0.05) and testosterone in men (r=0.472, p<0.05). Direct correlation between BMD and age of diagnosis was revealed (r=0.781, p<0.01). CONCLUSIONS: The results show that patients need to be monitored annually and for life after the combined treatment of malignant brain tumors in order to detect the long-term effects of the treatment. The high incidence of osteopenic conditions determines the relevance and need for early diagnosis to prevent further bone loss, reduced bone strength and the risk of fractures.

[Transsphenoid extirpation of pituitary adenomas in patients with McCune-Albright syndrome (two cases from practice and literature review)]. / Transsfenoidal'noe udalenie adenom gipofiza u patsientov s sindromom MakK'iuna-Olbraita (dva sluchaia iz praktiki i obzor literatury).

INTRODUCTION: McCune-Albright Syndrome is a rare genetic disease characterized by the formation of fibrous osteodysplasia foci of various localization, including the bones of skull base. Having a gross lesion of the main bone body and the simultaneous formation of the pituitary adenoma, its transnasal removal becomes very difficult. MATERIAL AND METHODS: Two clinical observations are presented where at patients with the classic manifestation of McCune-Albright syndrome we were able to successfully remove somatotropinomas with endoextrasellar growth. In both cases the presence of visual disorder was the indication for surgery. RESULTS: Despite pronounced deviations in the anatomy of the skull base bones in both cases we managed to access the Turkish saddle. Removal of the pituitary tumor did not differ from standard surgery. Improved vision after surgery was observed in two patients. Tumors were removed subtotally and clinical and biochemical remission of acromegaly was not achieved. In both cases, the tumors had morphological signs of atypia. Patients continued to receive therapy with somatostatin analogues and radiation. CONCLUSION: The possibility of performing transnasal surgery in this category of patients using specific instruments and intraoperative navigation is shown.

[The glucagon test in diagnosis of secondary adrenal insufficiency after craniospinal irradiation: the feasibility of application, the features of performing the test, and its diagnostic informativity].

BACKGROUND: The glucagon test (GT) is a promising alternative to the insulin hypoglycemia test (IHT) in diagnosis of secondary adrenal insufficiency (SAI). AIM: To study the feasibility of using the GT in patients after craniospinal irradiation and to determine the cut-off value to rule out SAI. METHODS: A total of 28 patients (14 males and 14 females) with the median age of 19 years (17; 23) who had undergone combination treatment (surgery, craniospinal irradiation (35 Gy) with boost to the tumor bed, and polychemotherapy) of extrapituitary brain tumors no later than 2 years before study initiation and 10 healthy volunteers of matching sex and age were examined. All the subjects underwent the GT and IHT with an interval of at least 57 days. The cortisol, ACTH, and glucose levels were measured. RESULTS: Twelve out of 28 patients were diagnosed with SAI according to the IHT results. ROC analysis revealed that cortisol release during the GT 499 nmol/L ruled out SAI [100% sensitivity (Se); 62% specificity (Sp)], while the absence of a rise 340 nmol/l verified SAI (Sp 100%; 55% Se). For GT, the area under a curve (AUC) was 93.6%, which corresponds to a very good diagnostic informativity. In 19 patients, the IHT and GT results were concordant (in ten patients, the release of cortisol occurred above the cut-off value in both tests; no release was detected in nine patients). In nine cases, the results were discordant: the maximum cortisol level detected in the GT was 500 nmol/l, but the IHT results ruled out SAI (the GT yielded a false positive outcome). Contrariwise, in three (10.7%) patients the release of cortisol detected in the GT was adequate, while being insufficient in the IHT test. Adverse events (nausea) were reported during the GT test in 9 (25%) subjects; one patient had hypoglycemia (1.8 mmol/l). CONCLUSION: GT is highly informative and can be used as a first-level stimulation test for ruling out SAI in patients exposed to craniospinal irradiation performed to manage brain tumors. The cortisol level of 500 nmol/L is the best cut-off value for ruling out SAI according to the GT results. The insulin hypoglycemia test is used as the second-level supporting test in patients with positive GT results.

[Clinical features and diagnosis of secondary adrenal insufficiency followed complex treatment nonpituitary brain tumors].

BACKGROUND: The most of the current studies include patients who are different by the etiology of secondary adrenal insufficiency (SAI), or investigate SAI among other late effects of the radiation therapy. AIMS: To describe the features of SAI and to select the best method of screening SAI in adult patients followed complex treatment of nonpituitary brain tumors in childhood. MATERIALS AND METHODS: It was the retrospective cross-sectional study. 31 patients after the complex treatment of nonpituitary brain tumors in childhood and 20 healthy volunteers were examined. Age and sex ratio were comparable between the groups. Biochemical and clinical blood tests, levels of cortisol, ACTH, DHEA-C were evaluated. The insulin tolerance test (ITT) was performed for all patients and 11 volunteers. RESULTS: The prevalence of SAI by ITT was 45.2%. The levels of basal cortisol (BC) were significantly higher in patients without SAI in comparison with the SAI group and volunteers (505 [340; 650] vs 323 [233; 382] and 372 [263; 489] nmol / l; pSAI- without_SAI=0.001; pwihtout_SAI-healthy = 0.04). The SAI group had DHEA-C significantly lower than in other groups one (3.1 [1.8; 3.4] vs 5.1 [2.5; 6.4] and 6.8 [4.1; 8.9]; рSAI- without_SAI = 0.036; pSAI-healthy = 0.001). ROC analysis showed that BC and DHEA-S can be used as high-quality screening tests for SAI (AUC = 89.3% and 88.3%). The maximum level of cortisol (656 [608-686] vs 634 [548-677]; p = 1) and the time of its increase (45 and 60 min) did not differ during ITT in patients without SAI and volunteers. Side effects: delayed hypoglycemia occurred in 4/14 patients of the SAI group 4090 minutes late of injection 60-80 ml of 40% glucose solution for stopping hypoglycemia in the test. CONCLUSIONS: 45.2% of patients followed craniospinal irradiation had SAI that is characterized by a decrease in DHEA-C levels. A highly normal level of basal cortisol was observed in 45% of patients without SAI. DHEA-C and blood cortisol can be used for SAI screening.

[Radiotherapy and radiosurgery in treatment of craniopharyngiomas]. / Luchevaia terapiia i radiokhirurgiia v lechenii kraniofaringiom.

Craniopharyngiomas are benign epithelial tumors having a dysembryogenetic origin, which are usually located in the sellar/parasellar and/or third ventricle regions. Gross total resection is the modern standard of treatment for these tumors because of a low recurrence rate. However, this surgery in some patients with craniopharyngioma often leads to the development or worsening of diencephalic disorders poorly responding to treatment. Perhaps, in these cases, subtotal or partial tumor resection or implantation of an Ommaya reservoir into the tumor cyst followed by stereotactic radiotherapy/radiosurgery may provide better functional outcomes and higher life quality in patients, with tumor growth control being reasonable (according to the published data, the mean 10-year disease-free survival is 66.9% after total tumor resection and 79.6% after combined treatment). The paper presents a review of the literature on radiological treatment of craniopharyngiomas. We discuss the issues of indications, optimal timing of radiotherapy/radiosurgery, its efficacy, and treatment outcomes in terms of complications and quality of life. Particular attention is paid to enlargement of craniopharyngioma cysts during and after radiological treatment.

[Transient enlargement of craniopharyngioma cysts after stereotactic radiotherapy and radiosurgery]. / Tranzitornoe uvelichenie ob''ema kist kraniofaringiom posle stereotaksicheskoi luchevoi terapii i radiokhirurgii.

Stereotactic radiotherapy/radiosurgery (RT/ES) is an effective technique for treating craniopharyngiomas (CPs). However, enlargement of the cystic part of the tumor occurs in some cases after irradiation. The enlargement may be transient and not require treatment or be a true relapse requiring treatment. MATERIAL AND METHODS: In this study, we performed a retrospective analysis of 79 pediatric patients who underwent stereotactic RT or RS after resection of craniopharyngioma. RESULTS: Five-year relapse-free survival after complex treatment of CP was 86%. In the early period after irradiation, 3.5 months (2.7-9.4) on average, enlargement of the cystic component of the tumor was detected in 10 (12.7%) patients; in 9 (11.4%) of them, the enlargement was transient and did not require treatment; in one case, the patient underwent surgery due to reduced visual acuity. In 8 (10.1%) patients, an increase in the residual tumor (a solid component of the tumor in 2 cases and a cystic component of the tumor in 6 cases) occurred in the long-term period after irradiation - after 26.3 months (16.6-48.9) and did not decrease during follow-up in none of the cases, i.e. continued growth of the tumor was diagnosed. A statistical analysis revealed that differences in the terms of transient enlargement and true continued growth were statistically significant (p<0.01). CONCLUSION: Enlargement of a craniopharyngioma cyst in the early period (up to 1 year) after RT/RS is usually transient and does not require surgical treatment (except cases where worsening of neurological symptoms occurs, or occlusive hydrocephalus develops).

[The rate of hyponatremia in neurosurgical patients (comparison between the data from the Burdenko Neurosurgical Instutite and the literature) and recommendations for the diagnosis and treatment].

UNLABELLED: Hyponatremia is a relatively frequent and serious complication in patients with various neurosurgical pathologies. OBJECTIVE: This study is aimed at assessing the incidence of hyponatremia in neurosurgical patients depending on the pathology. MATERIAL AND METHODS: This paper presents a retrospective analysis 39 479 cases of patients operated on at the Burdenko Neurosurgical Institute from 2008 to 2014. RESULTS: A total of 785 hyponatremic patients with Na level lower than 130 mmol/l (2% of all operated patients) were identified. Mortality in patients with hyponatremia was 14.3%, which is tenfold higher compared to the rest of population of patients without hyponatremia who were operated on during the same period. In adults, hyponatremia most frequently occurred after resection of craniopharyngiomas (11%) and as a result of acute cerebrovascular accident (22%). In children, it occurred after resection of craniopharyngiomas (10%), astrocytomas (7%), ependymomas (24%), and germ cell tumors (10.5%). CONCLUSION: This study, which was mainly statistical one, was not aimed at detailed investigation of hyponatremia in different groups of neurosurgical patients. We only tried to draw the attention of various experts to those categories of patients, where focused and in-depth developments are more than important. Obviously, already gained international experience should be taken into account for this PURPOSE: Therefore, this article presents the literature data on the etiology and pathogenesis of hyponatremia. We describe the details of the various classifications of hyponatremia, its clinical symptoms, diagnosis, and treatments, primarily based on the recommendations of the last European consensus of various specialists (2014).

[Basal ganglia germinomas in children. Four clinical cases and a literature review].

UNLABELLED: Basal ganglia germinomas are a specific group of intracranial germinomas. Their early diagnosis is complicated due to their atypical localization and diversity of neuroimaging and clinical signs. MATERIAL AND METHODS: We describe 4 cases of basal ganglia germinoma in boys of 13, 14, 15, and 16 years of age. The medical history data, clinical features, neuroimaging and histological characteristics of basal ganglia germonomas, and preliminary results of the treatment are presented. CONCLUSION: Basal ganglia germinomas are usually verified at the late stage of the disease when patients are detected with extended lesions of the basal ganglia and severe neurological and neuroendocrine deficits. This situation is due to clinical and imaging signs that are untypical of common germinomas.

[Growth disorders after treatment for acute lymphoblastic leukemia in childhood].

AIM: To evaluate the character of growth disorders and risk factors of their development after treatment of acute lympho- blastic leukemia (ALL) patients in childhood. MATERIALS AND METHODS: In this study 25 patients (16 women and 9 men) who had undergone treatment for ALL in childhood were assessed. Patients underwent polychemo- therapy and cranial irradiation. Average age at the time of the survey - 21,2±3,9 years; average age at the time of treatment - 6,9±3,4 years; average follow-up - 13,8±4,9 years. Healthy volunteers were included in the control group (10 women and 6 men) at the age of 25,9±3,6 years. Patients' anthropometric and laboratory parameters were measured. RESULTS: SDS of the final height in ALL survivors was significantly lower in comparison with the control group (p=0,009). ALL survivors had significantly higher difference between final and target height compared to control (p=0,049). 4 of 8 men (50,0% CI: 24,5% - 75,5%) and 13 of 15 women (86,7% CI:68,1-95,7%) have reached the target height. 73,9% (CI: 56,3% - 86,8%) of ALL survivors have reached target height which is significantly lower than in the control group (p<0,001). We found a significant backward correlation be- tween the age at the time of treatment and reaching of the target height (r=-0,415, p=0,049). ALL survivors also suffered from obesity - 12%, dyslipidemia - 36,8%, insulin resistance - 66,7%. CONCLUSION: Treatment for ALL in childhood causes a de- crease of final height. Its main risk factor is the age at the time of the treatment. ALL survivors are diagnosed with the other endocrine disorders and they need an endocrinologist's observation.

[Growing teratoma syndrome in a patient with intracranial germ cell tumor].

A six-year-old patient with non-germinomatous germ cell tumor of the chiasmatic-sellar area developed polyuria and polydipsia as the first symptoms of the disease. Then there were signs of precocious puberty and vision impairment. MRI examination revealed a shiasmatic sellar tumor and occlusive hydrocephalus. Tumor marker levels in blood serum were elevated. The alpha-fetoprotein level was increased 5-fold; human chorionic gonadotropin 20-fold. These levels increased over time. The patient received 2 cycles of PEI multiagent chemotherapy (Ifosfamide 1.5 g/m(2), Cisplatin 20 mg/m(2), Etoposide 100 mg/m(2)) during 5 days and 1 cycle of second-line multiagent chemotherapy (Cisplatin 100 mg/m(2) for 1 day and Endoxan 1500 mg/m(2) for 2 days). Despite the decrease in tumor marker levels to normal values, the patient's vision still deteriorated. MRI examination revealed that tumor size increased and its structure changed. Total tumor resection led to vision improvement and regression of intracranial hypertension. Histological analysis of tumor tissue only revealed a mature teratoma. This phenomenon, known as growing teratoma syndrome, is very rare among patients with intracranial non-germinomatous germ cell tumors.

[Characteristics of medulloblastoma in children under age of three years].

We present a series of 51 medulloblastoma in children under three years, collected in N.N. Burdenko Neurosurgical Institute from 2000 to 2010. 57% of the tumors showed desmoplastic/nodular histology. Performed fluorescence in situ hybridization (FISH) analysis revealed the MYC oncogene amplification in 4%, the MYCN oncogene amplification - in 8%, isochromosome 17q - in 16% of cases. 9q deletion was found in 8% of desmoplastic/ nodular medulloblastomas. Our results showed that desmoplastic/nodular medulloblastoma has a positive predictive value for progression-free survival. Another feature of a biology of medulloblastomas in children younger than three years is the lack of nuclear accumulation of beta-catenin, and 6q deletion. Medulloblastomas with MYCN oncogene amplification often exhibit desmoplastic/nodular histology and a relatively favorable outcome. The most unfavorable prognostic marker is the MYC oncogene amplification, which in our series of 100% combined with the large cell/anaplastic medulloblastoma and isochromosome 17q - such tumors should be included in the "high risk" protocol.

[Thyrotropin secreting pituitary adenoma in a child: case report and literature review].

We present a case of 11-year old boy with Type 1 Autoimmune Polyglandular Syndrome and thyrotropin secreting pituitary adenoma, which was diagnosed by elevated TSH and thyroid hormones levels and MRI signs of pituitary tumor and without clinical symptoms of hyperthyroidism. He underwent partial resection of the tumor via transnasal approach and subsequent radiation therapy. Consequently 1 year after XRT patient developed growth hormone deficiency, 3.5 years later patient became euthyroid, and 5.5 years after treatment - hypothyroid. This is the first described case of coexistence of this 2 rare endocrine diseases in the same patient.

[Stereotactic radiotherapy and radiosurgery in treatment of patients with deep-seated pilocytic astrocytomas].

Pilocytic astrocytoma (PA) is a low-grade glial tumor (WHO grade I) with predominant occurrence in pediatric patients. According to many authors, stereotactic radiosurgery (SRS) and radiotherapy (SRT) promote long-term remission or retardation of tumor progression in patients with in inoperable lesions after incomplete resection or recurrence. Therefore it is essential to determine the role of SRS and SRT in complex management of patients with deep-seated PA. Since April 2005 till May 2010 101 patient with intracranial PA was treated in department for radiation therapy of Burdenko Neurosurgical Institute. The series consisted of 70 pediatric patients (below 17 years inclusively) and 31 adults, of them--51 male and 50 female patients. Mean age was 15.1 years (9.8 years in children and 28.7 in adults). In 90 patients (89.2%) tumors were previously histologically verified (tumor resection in 83 cases and biopsy in 7). In 11 (10.8%) patients diagnosis of PA was based on clinical and neurovisualization data. In most cases SRT (66 (66.3%) patients) was preformed, the rest 35 (34.7%) patients were treated by SRS. Median follow-up from the onset of disease reached 52 months (2-228 months). Catamnestic data were available in 88 (87%) patients. By the end of catamnestic follow-up (December 2010) 87 (98.8%) patients treated by SRS and SRT were alive. Median follow-up from the start of radiation treatment was 22.7 months (6-60 months). Progression of tumor was observed in 20 patients (22.7%), in 18 of them due to cyst growth. 18 patients were reoperated. In 12 operated patients histological examination and its comparative analysis were performed. We found that alterations in the tumor tissue, accompanied by regression of solid component and progression of cystic portion, represent reactive-degenerative changes in the tumor as a consequence of radiation-induced pathomorphism. SRS and STR are effective techniques for treatment of patients with primary and recurrent PA despite regardless of localization of the tumor. There procedures should be performed shortly after non-radical resection. Control of tumor growth by the present time (median follow-up is 22.7 months) reaches 98%. "Progression" of the tumor due to enlargement of cystic portion shortly after SRT and SRS represents reactive-degenerative alterations in the tumor tissue and should not be evaluated as true recurrence; without neurological deterioration these cases do not require special treatment.

[Implantation metastasizing of craniopharyngioma].

The paper describes two cases of implantation metastasizing of craniopharyngioma after its resection using combined (transcallosal and subfrontal) and pterional approach. The mechanisms of metastasizing and possible ways of prevention are discussed.

[Stereotactic radiosurgery in treatment of patients with hypothalamic hamartomas].

Hypothalamic hamartoma (HH) is malformation of the brain manifesting by paroxysmal disorders (gelastic seizures) and endocrine disorders (mostly precocious sexual development). Surgical resection of HH is associated with the risk of severe neurological and neuroendocrine complications. Alternative ways of treatment are being investigasted. Stereotactic radiosurgery is one of the leading techniques in treatment of pharmacoresistant seizures due to high effectiveness and low complication rate. Since 2005 till 2009 7 patients with HH were treated in Burdenko Moscow Neurosurgical Institute. Age of patients varied between 9 months and 20 years 9 mean - 8.5 years). In all cases the disease manifested by gelastic seizures with frequency differing between 2-3 per months and 17 per day. All patients received long-term combined anticonvulsant treatment (using valproates, carbamazepine, lamotrigine, oxcarbazepine, topiramate) with temporary and insufficient effect or without any effect. In 4 patients frequency of seizures increased during anticonvulsant therapy. I patient had been operated (partial resection of HH) with temporary effect which consisted of decrease in frequency of seizures for 8 months; later frequency of paroxysms increased. Main indications to radiosurgical treatment included progressive course of disease, low effectiveness of anticonvulsant therapy and high risk of direct surgical treatment. 6 patients were treated using linear accelerator "Novalis" and 1 using "Cyber-knife". Treatment was performed under pharmacological sedation (midazolam, ketamine). Marginal dose was 11-18 Gy (mean 15.7 Gy). Volume of hamartomas was 0.3-3 cm3. Follow-up period varied between 3 and 50 months. Decreased frequency of seizures was observed in 100% of cases. Stereotactic radiosurgery is an effective and safe method of treatment for HH and may be considered as treatment of choice for pharmacoresistant seizures caused by HH.

Growth in children with craniopharyngioma following surgery.

OBJECTIVE: Hypopituitarism, including severe GH deficiency, is an almost inevitable outcome of craniopharyngioma. However, some GH deficient children with this tumour may grow normally or even have accelerated growth postoperatively. To study this phenomenon we have investigated the endocrine status, including IGF-1 and its binding proteins IGFBP-1 and IGFBP3, in children referred for follow-up at various time intervals after surgery. PATIENTS: Twenty-five patients (14 boys and 11 girls, aged 3.8-18.9 years), were studied on 34 occasions between 0.5 to 10.8 years after surgery. The tumour was intrasellar in 11 cases and suprasellar in 14. METHODS: Height and height velocity were recorded as SDS values. Body mass index (BMI) was calculated as weight/height2. GH secretory status was evaluated by stimulation both with oral clonidine and with GH releasing hormone. Serum insulin (INS) levels were studied following an oral glucose load. IGF-1, IGFBP-3, IGFBP-1 and prolactin serum concentrations were evaluated in fasting samples. All hormones were measured by radioimmunoassay. RESULTS: All patients had growth hormone (GH) deficiency. Height (HtSDSCA) and height velocity SDS for chronological age (HVSDSCA) decreased progressively after surgery (r = -0.47, P = 0.005, and r = -0.4, P = 0.032, respectively) but four patients had normal HtSDSCA 6.1 to 10.8 years following their first surgery. There was a significant correlation between BMI and HtSDSCA (r = 0.37, P = 0.03). BMI in children with suprasellar craniopharyngioma was significantly greater than that in patients with intrasellar tumour (23.3 +/- 7.0 vs. 17.3 +/- 1.4 kg/m2; P = 0.001). In 13 of 33 cases oral glucose load was accompanied by hyperinsulinaemia with serum INS levels greater than 50 mU/l. The mean area under the curve (AUC) of INS after glucose load (INSAUC) in the suprasellar group was greater than in the patients with intrasellar lesion (6945.5 +/- 4411.8 vs. 2495.5 +/- 1768.8 mU/l. min P = 0-001). The log INSAUC correlated significantly with HtSDSCA (0.37, P = 0.03). Fasting serum IGF-1 and IGFBP3 levels were normal in 8 and 12 of the 31 measurements, respectively. Both IGF-1SDS and IGFBP-3SDS correlated significantly with HtSDSCA (r = 0.77, P = 0.0002 and r = 0.65, P = 0.0001, respectively) and the log INSAUC (r = 0.39, P = 0.035, r = 0.56, P = 0.002, respectively). As determined by forward stepwise regression analysis, IGF-1SDS was the single most important predictor of HtSDSCA (R2 = 0.33, P = 0.001) in the subset mode. CONCLUSION: A few children with craniopharyngioma grew normally after surgery, in spite of being GH-deficient. This growth phenomenon, which is usually accompanied by obesity, was more common in patients with suprasellar tumour and is likely to be associated with the effect of IGF-1 bioavailability, which, in turn, may be modulated largely by insulin.