S258
Pediatric Neurosurgery
IP.6-7051
Adjuvant chemotherapy using ifosfamide, cisplatin and etoposide for PNET/medulloblastoma in children
Yutaka sawamura, Jun-Ichi Murata, Tsutomu Kato, Jun Ikeda,Mitsuhiro Tada, HiroshiAbe. Department of Neurosurgery. University of Hokkaido, Schoolof Medicine, Sapporo, Japan Introduction: The major problems currently associated with management of PNETlmedulioblastoma are high mortalityand late morbidity derivedfrom craniospinalradiotherapy. Thirty-fivechildren with either PNETor medulloblastoma wereassessed to define the role of adjuvantchemotherapy. Methods: Prior to 1991, 23 children were treated mainly with surgery and craniospinal radiotherapy. Since 199t , 12 newly-diagnosed children were treated witha cisplatin-based intensivechemotherapy (ifosfamide 900 mglsqm, cisplatln 20 mg/sqm, etoposide60 mg/sqm, Day 1-5), every4 weeks for 8 cycles. Children under2 years old were treatedwith8 cyclesof the chemotherapy alone, and then irradiated after the age of 2 years. Results: In 23 children treated prior to 1991, 2- and 5-year survival rates were 52% (12/23) and 39% (9/23),respectively. Sevenchildren are alive with a mean observation periodof 161 monthsafter surgery, however, six of them had serious mental retardation and endocrinological deficits. In 12 children treated withnewchemotherapy protocolafter 1991 , complete remissions wereachieved in 10 of 12 patients. Two-yearsurvival rate was 83% (10/12) and disease-free survivalrate was 67% (8/12)with a meanobservation period of 29 months after surgery. Discussion and Conclusions: Cisplatin-based chemotherapy appeared to be an effective adjuvant chemotherapy for management of the children with PNET/medulloblastoma.
IP-6-7061 fossa Brainstem auditory evoked potentials for posterior mass lesions in pediatric patients K. Sestakauskas, I. Uloziena, A.-J. Gruodis. Clinicof Neurosurgery, Kaunas MedicalAcademyHospital, Kaunas, Lithuania Brainstem auditory evoked potentials (BAEP) - a method of objectiveassessment of auditory pathway conduction is not a routinetool in the neurosurgical department. We tried to compare pathologic changesof BAEPwith clinicaland radiological findings in 14 pediatric patients (pts.) wi1h posterior fossa mass lesions. CT revealed brainstem gliomasin 8, cerebellar gliomas- in 5, cerebellopontine angle (CPA)traumatic haematoma - in 1 pt. Abnormalities of BAEP were found in most cases, presenting from prolongation of interpeak latencies to disappearances of BAEP waves. Brainstem gliomasresulted in impaired conductivity in brainstem segments manifesting by almostsymmetric disappearance of waves in 3 pts., prolongation of brainstem conduction time unilaterally in 1, bilaterally - in 1 pt., bilateral prolongation of brainstem and prolonged nerve conduction times unilaterally - in 2 pts., bilaterally - 1 pt. Cerebellar gliomas caused prolongation of brainstem conduction time unilaterally - in 2 patients, bilaterally - in 2 pts., showing worse conduction on the side of major growth of the tumour, according to CT. One pt. with cerebellar glioma had no BAEPabnormalities and only slight clinicalsignsof cerebellar dysfunction. CPA traumatichaematoma resulted in impairednerve and brainstem conduction on the ipsilateral side. Follow-up after 2 weeks of conservative treatment in the latter case revealed improved to almost normalconduction with only increased intersidedifference. We found that BAEP abnormalities corresponded well with CT data and reflected the clinical course of the disease. BAEP could be a routine tool for objectiveassessment of the functional state of the brainstem, especially in not alwayscooperative pediatricpatients.
Ip.6-7071
The dermoid of the calvarium. Case report
A. Dzubera, P. Hlincik, J. Klepoch. Dept. of Neurosurgery of Commenius University DerersHospital, Bratislava, SlovakRepublic The dermoid of the calvarium is a very rare diagnosis. Histologically it is a disgerminal benign cystic tumor, composed of all dermal cells, tissues and structures. Localisation of the dermoids is usually the middle sagittal line. There is a possibility of subdural growthof the cyst. The largestsampledescribedin the literature was to casesover a periodof 18 years. In our 10-yearmaterial there were 2 cases. We report one of them. 14-yearold girt admitted in October 1994. Elasticslow-growth tumorin the right frontoparietal region of calvarium since birth. Twice misdiagnosed calvarium. As an atheroma in an out-patient surgical office after radiodiagrams and CT examination successfulmanagement in our department. CT-scans after 1 year withoutany pathology.
Thursday, 10 July 1997
Ip-B-7oal
An orbital Langerhans histiocytosis case in childhood
AhmetBekar, KayaAksoy, Seref Dogan, BeWI Sevinir, Ahmet Ozmen. Uludag University, Schoolof Medicine, Departments of Neurosurgery, Ophthalmology and Pediatric Oncology, Bursa, 16059, Turkey Introduction: Histiocytosis X is characterized by the localand diffuseinfiltration of monocytes which originate from the promonocytes of the bone marrow. It is classified as eosinophilic granuloma, Hand-Schueller-Chrisfian disease and Letterer-Siwe syndrome from the point of the central nervous system involvement. In prognostic evaluation. There are monocytic, polyocytic disseminated and unifocal skeletal forms. Histiocytosis X of the orbit is reported rarely in childhood. Clinical Presentation: A one-year old body presented with a mass at the temporal side of the right orbit. This mass was present for a month and CT of the orbit revealed a massand bonedestruction at the temporal side of the right orbit. Intervention: The mass was resected totally. The histological findings of positive S-100 protein in the immunocytochemical study suggested the possibility of Langerhans histiocytosis. The patient showed no systemic symptoms. He wastreated withVinblastine 0.001 mg/kg/perweek (intravenously 6 months) and prednisolon 30 mglm2/per day (per orally 4 weeks). Conclusion: During one year follow-up, the patient showedno recurrence. Inthe literature the prognosis is reported to be good in the localizedand unifocal formsof the Langerhans histiocytosis cases.
IP-6-70gl Sinus pericranii: A case of childhood KayaAksoy, AhmetBekar, KudretTOreyen, Suat E. Yelik. U1udag University, Schoolof Medicine, Departments of Neurosurgery, Bursa, 16059, Turkey Introduction: Sinuspericranii is a rarevascularanomalyinvolving an abnormal communication between the extra cranial and intracranial venous circulations. A sinus pericranii case of the left frontoparietal regionwhich appeared to be a posttraumatic case is presented. Clinical Presentation: A 13-yearold patientwas admitted to our clinic with a left frontoparietal extra cranial soft mass. 10 years prior to admission he had fallen down and had an injury of the left frontoparietal region. His clinical evaluation revealed contusion without fracture. The mass was more apparent when the patient was lying down. The direct injection of the contrast medium into the mass revealed that the ertracranial mass cavity was connected to the superiorsagittalsinus, throughthe emissaryveins. Intervention: The mass located in the subgaieal. epiperiosteal space was totally resected and the connection of the intracranial sinus was closed. The mass was diagnosed as sinus pericranii and considered to be secondary to trauma. Conclusion: During3 monthsfollow-up, he showed no recurrence.
IP-6-710 I Arteriovenous malformations of the brain in children W. Szymanski, L. Polis,E. Blaszczyk. PolishMother'sMemorialHospital, Department of Neurosurgery. Lodz, Poland Arteriovenous malformations (AVM) of the brain are not rare in childhood. Pathological arteries, arterioles, veins and venules constitute the nodus of AVM. The combination of an abnormal vessel wall lacking the muscularlayer. and an elevation of blood pressure may produce hemorrhage or changes in the surrounding brain tissue. In the department of neurosurgery 56 children with AVM were diagnosed in the last 5 years. The clinical symptoms were: intracranial hemorrhage in 33 (50.7%): seizures in 12 (21.%); headache in 6 (15.5%); neurological deficit in 4 (8.5%) and other symptoms in 3 (4.2%). CT scansweremadein all patients. Angiography wasperformed in 46 patients. The clinical condition was characterized by the Glasgow Coma Scale (GCS) and the Children Coma Scale (CCS). 47.2% of the children had a score of 13-15 (good condition) , and 20.8% were in poor condition with a GCS score of < 8. The AVMwas classified according to the Spetzler-Martin grading system. In 21 children an emergency operation had to be carried out. The results of surgery were characterized by the Glasgow Outcome Scale (GOS), and relatedto AVM size, clinicalcondition and the grade according to the Speller-Martin system.
I
, P-6-711 ~iag~ostics and surgical treatment of brain abscess In
children
.
C.M. Secara, V.L. Petrachi, A.S. Bajurea, N.G. Predenciuc, A.A. Plesco. Institute for Motherand Child (IMC), Kishinev, Moldova We have analyzed 34 children with brain abscesses operated in the pediatric surgerydepartment of the IMCin Moldova. A total of 42 interventions had been carried out. Etiology: the cause of the abscess was ear-nose-throat pathology requiring anthromastoidotomy in 9 cases; trauma with infection in 8 cases, in-
Thursday, IO July 1997 flammatory disease(parotitis, tonsillitis and appendicitis) in 6 cases, congenital heartdisease in 4 cases, meningoencephalitis in 4 cases. Level of consciousness: comaI-II in3 children, somnolence in8 children. In mostcasesintracranial hypertension andfocalsymptoms werefound. Thediagnosis wasconfirmed by ultrasonography in 12 cases. We usedCT beforeand aftersurgical intervention in the last 5 years. Classical craniotomy was undertaken in 24 patients (20 supratentorial, 4 infratentorial). In 8 cases puncture of the abscess was performed with drainage and washing out with antibiotics. In 2 cases ultrasound guided aspiration and an endoscopic technique was used.In the postoperative period8 patients (23%)died as a resultof variouscomplications.
IP-6-712! Medical treatment of haemophilus influenzae subdural empyema in infancy Teng-Yuan Shih. Chang-Gung MemorialHospital, Kaohsiumg, Taiwan Four infants with bacterial meningitis and subdural empyema were treated by medical treatment and surgical procedure. Sincethe use of computed tomography (eT), showing enhancement of the subdural collection, for diagnosis of subdural empyema, the importance of bacterial culturecanbe neglected. Based on data obtained from this review and from studiesprevious published in the literature, Haemophilus influenzae meningitis appears to have a low incidence of positive culture from subdural space. The patient is unlikely to benefitfrom surgical treatment. A noninvasive approach inan otherwise stablepatient seems appropriate. We thinkthat surgery is not alwaysindicated by CT enhancement, to determine the causing organism is anotherspecificindication.
IP-6-713I split Treatment of simple and complex skull defects by calvaria cranioplasty S. Asgari, H.A.Trost, D. Stolke. Department of Neurosurgery, University Hospital, Essen, Germany Introduction: Skull defects are mostlycaused by head injury. After removing a tumour like a meningioma, which invades the bone, it is customary to reject the boneflap. Usuallya reconstruction of the skull by usingmethylmethacrylate or other alloplastic material is possible. If the patient is very young, especially a child, autologous bone for cranioplasty is desired. Herefore the split calvaria method is available. A separate bone flap has to be divided in tabula intema and externa, The tabula externa then comes into its original place and tabula internafills the formerdefect. Method: From1990-1994 13 patients underwent split calvaria cranioplasty. The meanage was 19 years. Sevenpatients suffered from severehead injury with extensive skull impression. The remaining cases had miscellaneous diseases. After operation all the patients were frequently examined clinically and by X-raystudieswitha meanobservation periodof 2 years. Results: Without any peri- or postoperative complication all patients had got excellent wound-healing. The X-raystudiesdemonstrated that even extensive defects were well treated by split calvaria cranioplasty. In every case the cranioplasty showedbonyintegration. Conclusion: Especially in infantsand youngadultsthe splitcalvaria cranioplasty is an efficientmethod to treat skull defects. The autologous bone grows withinthe skull.
I P-6-7141
GrOWing skull fracture: A clinical study in 15 children
J.N. Guilburd, A. Rakier. Dept. Neurosurg. (Pediat. Div.)Rambam Medical Center, Haifa, Israel Fifteen cases of growing skull fractures (GSF) were surgically treated in our institution duringa periodof 3 years. GSF is not an entityobserved onlyduring infancy, we have treated cases in which the head trauma occurred after the age of two years. As not commonly known, seizures were rarely seen before surgical intervention. The main feature for deciding surgical intervention was enlargment of the skulldefectwith development of a leptomeningeal cyst revealed by CT. Surgery should include: enlarging of craniectomy in orderto find normal borders of dura, adhesiolysis and resection of cerebra-periosteal scar, opening of the cyst to subarachnoid space or ventricle, hermetic closing of dura (covering the dural suturewith biologicglue)and cranioplasty. Post operative complications were seen in only 2 caseswith accumulation of CSF requiring localdrainage of CSFand repeated lumbarpuncture (twice) in one case and ventriculo-peritoneal shunt in anotherpatient. Onecase required removal of the cranioplasty one and half year after the operation because of post traumatic infection after falling. No post operative seizures or neurological deterioration wereseen.
Pediatric Neurosurgery
S259
IP-6-71S! pseudomeningocele Diastatic fracture of the skull with in childhood Ajay Sharma, MaryAbraham, Medha Tatke. G.B. PantHospital, New Delhi, India Diastatic fracture of the skull associated with pseudomeningocele is a rare complication of traumain infancy. Although the syndrome of "Growing fracture ofthe skull"is a recognized clinical entity, its pathogenesis andoptimaltreatment is still debatable. The role of dural tear in the genesis of diastaticskull fracture is wellestablished, however, the contribution of associated arachnoid and local braindamage is not clear. On the basis of personal experience of 28 surgically treated children with clastauc fracture of the skull with pseudomeningocele, an attempt is madeto redefine the pathophysiology and optimalsurgical treatment. Thesechildren have a progressively increasing swelling at the fracture site at a variable interval follOWing closed head injury, mostly by a fall. Progressive neurological deficitand convulsions were other common features. All patients had serial Plain Skullfilms, C.T. and M.R./. which helped in understanding the exact pathological changes and guided the surgical approach. Surgical intervention included a uniform procedure involving a craniotomy, excision of leptomeningeal cyst and meningocerebral cicatrix with dura and cranioplasty. C.S.F. diversion procedure was undertaken in 4 patients because of altered C.S.F. dynamics. Observations on preoperative andpostoperative C.T.and M.R./. studiesneuropathology of the surgical material and the outcome of the surgicalintervention in thesechildren havehelped in the betterunderstanding of this disorder. However, the mechanism of progressive braindamage, despitesurgicalcorrection, as observed in most of the children, in M.A.1. undertaken after severalmonths, is not clear. Linearfracture and burstfractures in a infant with a scalp swellingmust be corrected earlyto preventa growing fracture.
IP-6-716I rehabilitation Primary cranial plastic surgery as a measure of early K.S.Ormantaev, G.K. Kurmanbekov. Almaty lnst. for Pediatrics, Kazakhstan The large number of children with severe skull and brain injury warrants considering them as a social group. Although surgical treatment is paramount, rehabilitation should insurefurtherrestoration of impaired function. In the period of 1976to 1995therewere246children withsevereinjuryto the skull whowere treated by primary cranialplasticsurgery. All the patients were treated within 6 hoursafter sustaining the injury. In the postoperative periodearly rehabilitation was undertaken with prolonged clinical observation. According to the severity of injury, 68% had brain concussion with localcompression by bonefragments, 14% had an epidural hematoma, 9% had a subdural hematoma, 7% had only localconcussion, and 2% had intracerebral hematoma. Follow-up ranged from 2 to 10 years and revealed full recovery in 182 patients, partial disability in 47, and severe disability requiring nursing care in 17. This demonstrates the efficacy of primary cranial surgery in the treatment of children withcranial injury.
IP-6-717I
Diagnosis and treatment of laceration of the brain and the dura
K.S.Ormantaev, G.K. Kurmanbekov. ScientificCenterfor Pediatrics and Child Surgery, 146 AI-Farabi Avenue, Almaty, Kazakhstan It is known that children up to the age of 3 years have their dura mater adherent to the cranial bone. From 1978 to 1994 we have treated 28 patients with fracture of the skull and laceration of the brain and dura. We have used pneumoencephalography for earlydiagnosis at the first day after the injury. The patientwas laid down on the side opposite to the area of injury, the head was lifted 20-30 degrees, 10-25 ml of sterile air was introduced intrathecally after withdrawal of 2 ml of CSFfor laboratory examination. Fifteento twentyminutes later, X-rays were made with horizontal ray course. On the pneumoencephalogram the presence of air in the fracture zone, subcutaneously or epidurally, reveals laceration of the brainand meninges. It is an indication for emergency operation. In 6 patients admitted on the 7th day after injury CSF accumulation was demonstrated subcutaneously in the area of the fracture. The early diagnosis and emergency surgical treatment proposed allows the prevention of growing skull fractures and the development of scarring and atrophyof the brain,and is important for optimal recovery of the injuredbrain in children.
IP·6-718I
Severe pediatric head injury. Analyzing the better outcome over a decade
LionLevi,JosephN. Guilburd, Menashe 2aaroor, Jean F.Soustiel, Moshe Feinsod. Rambam MedicalCenter, Haifa, Israel A decade ago,we seta database on traumapatients. Interimanalysis disclosed muchbetteroutcome of children with severehead injury.