[A laboratory multipositional skull base holder].

Authors present an original multipositional holder for skull base cadaveric specimens, either formalin or fresh. It is constructed to be fixated for standard neurosurgical operating table, devoted for laboratory micro neurosurgical dissection and suitable for simulation operation on patient. The holder is made of solid stainless steel, practical and easy for cleaning. The device permits good tree point's fixation, extensive angular mobility of the primary and secondary joints, that the skull base specimen can be placed and supported in any desired position. It fulfills demands for fixation from external skull base surface with thin needles ensuring full surgical dissection field without obstacles or invisible angles.

Resection of orbital rhabdomyosarcoma in an infant with the aid of preoperative partial arterial embolization. A case report.

Since the introduction of non-invasive imaging techniques (CT, US, MRI), super selective cerebrospinal angiography has been playing a major role as a diagnostic tool as well as a therapeutic procedure prior to surgery or as an alternative. Surgical neuroangiography is currently a well-established therapeutic technique in neuropediatrics even in newborns or infants with vascular lesions in different parts of the body. Rhabdomyosarcoma is a malignant solid tumor arising from mesenchymal tissue that primarily affects children. We describe the procedure used to treat a large vascularized orbital rhabdomyosarcoma in a 38-day-old infant with application of partial tumor vascular bed embolization via external carotid end internal maxillary artery using PVA particles prior to gross excisional biopsy. We stress the significance of the preoperative embolization in infants with large highly vascularized tumor in order to reduce surgical blood loss and increase the probability of maximum lesion resection.

Malignant peripheral nerve sheath tumor of the oculomotor nerve.

We present the short-term follow-up magnetic resonance (MR) studies and 1H-MR spectroscopy in a child with malignant peripheral nerve sheath tumor of the oculomotor nerve associated with other less aggressive cranial nerve schwannomas. The tumor revealed perineural extension and diffuse nerve involvement besides rapid growth. 1H-MR spectroscopy was helpful in excluding an intra-axial neoplasm with exophytic growth, mainly due to the absence of creatine and N-acetyl aspartate peaks, and markedly elevated choline peak.

Peritoneo-vulvar catheter extrusion after shunt operation.

We report an unusual case of catheter extrusion through the external genitalia. between the labium majus and the labium minus, in a 6-month-old hydrocephalic baby. The event occurred 5 months after placement of a ventriculoperitoneal shunt.

[Dysconnection syndrome in a boy with tumorous lesion of corpus callosum]. / Sindrom prekida beza kod decaka s tumorskom lezijom zuljevitog tela.

We report on a 12-years-old boy with glioma of the septum pellucidum which invaded and infiltrated the adjacent structures including neocortical commissures. Neuropsychological evaluation revealed the loss of interhemispheric integration and hemisphere dysconnection syndrome. Our patient demonstrated left-sided ideo-motor apraxia to verbal commands and left-sided agrafia, being unable to copy, write to dictation and formulate his own sentences with the left hand. He partly failed in attempts to name or describe objects out of sight in the left hand and showing an unilateral tactile anomia. The patient performed the block-design-test better with the left hand than the right hand, and demonstrated a right-sided constructive apraxia. He had extinction of his left ear on dichotic listening, despite a normal audiogram in both ears. Because of progressive defects of visual function, a tachistoscoping testing was not performed. There was no evidence of the alien hand syndrome, reported earlier. The general dysconnection syndrome caused by lesion of the corpus callosum, was associated with cognitive dysfunctions. Attention, memory and speech disorders were attributed to the progressive extra-callosal cerebral neoplastic lesion. The neurosurgical treatment was performed by transcallosal anterior approach. On neuropsychological retesting, 4 months later symptoms of hemisphere dysconnection were stabilized.

[Craniopharyngiomas in children and adolescents]. / Kraniofaringeomi kod dece i adolescenata.

In sixtheen children and adolescents primary operation of craniopharyngioma over the period from January 1987 to February 1992 was carried out. The age of these patients ranged from nine months to eighteen years. There were nine females and seven males. Microsurgical operation was performed in fifteen cases, and stereotaxic biopsy with intracystic instillation of Y90 in one patient. Within the first month after the operation three patients died (18.8%). The total mortality was 31.2%. In nine out of thirteen patients tumour recurrence occured (69%). No tumour recurrence was noted in four patients: one after radical surgery, two after subtotal resection of craniopharyngioma, and a boy with stereotaxic biopsy and instillation of Y90. Second operation for tumour recurrence was carried out in three patients. Two children were lost from evidence, and four were subjected to radiotherapy. The authors analyse operation proceedings and results of treatment. They work out criteria for the optimal treatment, pointing out the neuroradiological, anatomosurgical and histopathological characteristics of these tumours.

Jackson-Weiss syndrome registered in four successive generations. The facies of Crouzon's syndrome with foot abnormalities.

A family with 15 individuals in four successive generations affected by Jackson-Weiss syndrome, craniosynostosis with Crouzon-variant-like phenotype and feet's abnormalities, is presented. An autosomal dominant inheritance pattern with complete penetrance, variable expressivity, and wide intrafamilial variation, more among, less within the same generation, was observed. Concerning the frequency and severity of complications, the evolution of craniofacial deformities seems to parallel those described with Crouzon syndrome, suggesting the similar evaluation and management.

[Spontaneous intracranial hemorrhage in term neonates and infants]. / Spontano intrakranijumsko krvavljenje kod donesene novorodjene dece i odojcadi.

Twenty nine full term haemophiliac neonates and babies, aged eight months with acute intracerebral haemorrhage are reported: four babies with haemophiliac status, one with intracerebellar bleeding due to microvascular malformation, and 24 babies in whom the cause of bleeding could not be established (probably vitamin-K deficiency). Fourteen surgeries were performed in ten babies. When dramatic neurological signs of spontaneous cerebral stroke develop in a term newborn or a baby they require immediate ultrasonographic examination. This noninvasive method can be repeated without risk to disclose all changes into the bleeding focus before and after surgical treatment. Selective cerebral angiography was exceptionally recommended for detection of suspected vascular malformation. Lumbar puncture was performed in more than one third of patients to confirm subarachnoid bleeding. However, this method has not been accepted being the purposeless and risky. Pharmacotherapy should be quickly used to correct the supposed vitamin-K deficiency and for replacement of relevant missing plasma factor of the prothrombin complex. To stop bleeding and to secure surgery the replacement substitute must raise the factors' level at least by 50 percent. The treatment of choice is vitamin-K1, 1-3 mg and fresh frozen plasma 10ml/kg of body weight. The neurological recovery was in general satisfactory after the stabilization of acute clotting disorder was achieved. Eight babies died (28 percent), five of them were admitted in deep comatose state.

Surgical treatment of epilepsy: our experiences with 34 children.

Surgical treatment of medically intractable epileptic fits in children is highly specialized, owing to the specific etiology of epilepsies in this age group and the epileptogenic property of the brain in childhood. In our series of 340 patients operated on for medically intractable epileptic seizures, there were 34 (10%) patients up to 15 years of age (the youngest was 2 years old; mean age was 11 years). This group of patients comprises four subgroups: 9 patients with temporal foci, 6 patients with extratemporal foci, 16 patients with infantile hemiplegia and epilepsy, and 3 patients with epilepsia partialis continua (Kozhevnikov's disease). Preoperatively, detectable brain lesions were present in 30 (88%) cases, a much higher frequency than in adult surgical series. Postoperative follow-up so far is 1-14 years (mean, 4 years). Surgical outcome in this group of 34 patients is as follows: 21 (62%) are seizure-free, 8 (23%) have improved, and 5 (15%) have shown no improvement.

[Transplantation of nigral and adrenal tissue into the striatum in rats--an experimental model for the surgical treatment of Parkinson disease]. / Transplantacija nigralnog i adrenalnog tkiva u strijatum pacova-- eksperimentalni model za hirursko lecenje Parkinsonove bolesti.

Transplantation of fetal nigral tissue, fetal and adult adrenal tissue into the dorsal segment of caudate nucleus was performed in 28 rats (wistar). Ventral mesencephalon of fetuses aged 16-17 weeks was used as a nigral graft. Adrenal medulla was taken from fetuses aged 17-20 weeks and from adult rats. In 12 rats denervation of striatum was previously performed. In six of them the lesion was made by mechanical cut of nigrostriatal pathway and in other six by 6-OH DA injection into ventral mesencephalon, according to previously established coordinates. Tissue pieces of 1 mm3 were placed in previously prepared cavity trough the cortex and corpus callosum into the dorsal surface of the caudate nucleus. In two rats transplantation was performed 4 weeks after preparing the cavity. By determining the level of DA and NA 2-3 weeks after transplantation the authors found that the graft substitutes a deficit of these monoamines in this period. In all cases with denervated striatum, the reduction of motor disorders occurred immediately after transplantation. Survival of intracaudate fetal cell grafts was noted in 23%, as well as the fatal and adult adrenal medulla grafts in 38,9% of biochemically studied cases.

[Modern aspects of treatment of medulloblastomas/primitive neuroectodermal tumors of the posterior cranial fossa in children]. / Savremeni aspekti lecenja meduloblastoma/primitivnih neuroektodermnih tumora zadnje lobanjske jame kod dece.

The authors present 34 medulloblastoma/primitive neuroectodermal tumours of the posterior fossa in children operated during the last ten years (1979-1989). Total removal of tumours has been performed in 13, and maximal-subtotal resection in 19 patients. Only four tumours were classified as small (group T1-T2). Partial reduction and biopsy were possible in four cases. The average period from surgery to the beginning of radio-oncologic treatment was 31 days. Radio- and chemotherapy were applied according to international standards. Permanent cerebrospinal fluid derivation preceded surgery in 9 patients (VA shunt in 6 and VP in 3) and after surgery of the posterior fossa in four children. Early postsurgical mortality rate, related to the first five years of this study, was 5.6%. Seven children (20.6%) survived from five to ten years after operation. The authors are advocating for standardization of diagnostical and surgical procedures, and for unification of postoperative control with intention to obtain longer duration of survival of these seriously sick children.

[Implantation of radioactive isotopes in intracranial tumors]. / Implanticija radioaktivnih izotopa u intrakranijalne tumore.

From may 1986 to July 1988 ten patients have been treated by interstitial implantation of radioactive isotopes using Yttrium 90 colloidal solution (9 cases) and Aurum 198 grains (1 case). There were 7 cystic out of 8 craniopharyngiomas, one malignant pituitary adenoma and one hemispheric Astrocytoma grade III-IV. In all but one patient the tumors were recurrent after one or more reductive or palliative operations. To external radiation undervent preoperatively two cases (one craniopharyngioma and one pituitary adenoma). Target volume was established by CT data and X-ray studies after stereotaxic injection of contrast medium (one case). Doses for intracystic irradiation were calculated using the Backlund's formula. The lowest activity was calculated to be 3.84 mCi, and the highest 12.9 mCi (m 6.8 mCi or 252 MBq). The delivered activity was 100-200 Gy of Y90 (m 140 Gy). The activity of Au198 was determined using the producers dosimetric tables. The radionuclide implantation was performed by stereotaxic techniques with Leksell's system in 5 patients. In 5 patients the surgical procedures were open: 3 osteoplastic supratentorial and 2 craniectomies for direct instillation of Y90 into the craniopharyngiomatous cavities spread to posterior fossa. Early short lasting side effects of endocavitary irradiation were observed in 5 patients (headache and somnolence; adynamy, pseudobulbar symptoms and rigor; insomnia and agressiveness; lack of orientation and increased mental irritability). The longest follow up was 26 months. The clinical response to intracystic instillation of Y90 was very favorable in 8 cases: 7 craniopharyngiomas and one pituitary adenoma. A satisfactory anatomical result with diminution or retraction of cystic cavities was evident in all cases. The more pronounced achievement of intracystic irradiation therapy in our series were the effects on stabilization or recuperation of vision and on improvement of visual field finding. The recovery of endocrine insufficiency was also noted. Two patients died: the 3-year old child, one year after implantation of Au198 grains in a huge calcified craniopharyngioma, and a woman, 67 old, twelve days after Y90 instillation to a hemispheric glioma grade III-IV.

Frequency of toxoplasmosis in the appearance of congenital hydrocephalus.

Thirty-eight children with signs of hydrocephalus have been tested for toxoplasmosis. On the basis of clinical data, including roentgenographical and ophthalmological findings, and serological data (Sabin-Feldman and IgM-IFA tests), congenital toxoplasmosis was confirmed in 15 (39%) infants. The diagnosis was established by demonstration of the persistence of Toxoplasma antibodies. In 13 (34%) infants aged 1 to 3 months there was not enough serological data and these were placed in a group of cases with "suspected congenital toxoplasmosis." Ten children with negative serology for toxoplasmosis and with hydrocephalus were considered not to be infected.