Tratamento dos craniofaringiomas por via trans-esfenoidal: resultados e seguimento pós-operatórios / Trans-sphenoidal approach to craniopharyngiomas: postoperative and follow-up results

Introdução: O craniofaringioma é uma neoplasia benignade difde difícil tratamento mesmo com ampla variedade de procedimentosterapêuticos disponíveis. Este fato decorre dassuas características biológicas peculiares e de sua localizaçãohabitual. Material e métodos: Este é um estudo retrospectivo depacientes com diagnóstico de craniofaringioma operados pelavia trans-esfenoidal no Hospital das Clínicas da Faculdadede Medicina da Universidade de São Paulo. Trinta e quatropacientes, operados no período de 1981 a 2000, tiveram seusprontuários avaliados. Dados referentes à condição clínica,exames de tomografi a e ressonância magnética, bem como dadosendocrinológicos foram verifi cados antes e após o procedimentocirúrgico, referentes ao último retorno ambulatorial conhecido.Os dados obtidos foram submetidos à análise estatística. Resultados:A faixa etária foi de 4 a 45 anos; 55,88% dos pacienteseram do sexo feminino. A média do tempo de seguimento foi de5,7 anos (d.p.= 5,9 anos); 44% dos casos tinham exame de imageminicial mostrando compressão de vias ópticas pelo tumor.Fístula liquórica foi a complicação mais freqüente associadaao procedimento. Conclusão: Concluímos que a via trans-esfenoidalé um procedimento válido para este tipo de tumor. Osmelhores resultados foram para os tumores de volume menor. As seqüelas endócrinas são muito freqüentes no seguimentoem longo prazo.

Apert syndrome: analysis of associated brain malformations and conformational changes determined by surgical treatment.

Apert Syndrome, also called acrocephalosyndactylia type 1, is characterized by craniostenosis with early fusion of sutures of the vault and/or cranial base, associated to mid-face hypoplasia, symmetric syndactylia of the hands and feet and other systemic malformations. CNS malformations and intracranial hypertension are frequently observed in these patients. Early surgical treatment aims to minimize the deleterious effects of intracranial hypertension. Fronto-orbital advancement, the usual surgical technique, increases the intracranial Volume and improves the disposition of encephalic structures previously deformed by a short skull. This study analyzes CNS alterations revealed by magnetic resonance in 18 patients presenting Apert Syndrome, and the conformational alterations in the encephalic structures after surgical treatment. The patients' age in February 2001 ranged from 14 to 322 Months (m=107). Image study included brain magnetic resonance showing ventricular enlargement in five cases (27.8%), corpus callosum hypoplasia in five cases (27.8%), septum pellucidum hypoplasia in five cases (27.8%), cavum vergae in two cases (11.1%) and, arachnoid cyst in the posterior fossa in two cases (11.1%). Absence of CNS alterations was noted in 44.4% of cases. A corpus callosum morphologic index was established by dividing its height by its length, which revealed values that ranged from 0.4409 to 1.0237. The values of this index were correlated to the occurrence or absence of surgical treatment (p=0.012; t=2.83). Data analysis allowed the conclusion that the corpus callosum morphologic measure quantified the conformational alterations of the cerebral structures determined by the surgical treatment.

Traumatic acute giant epidural hematoma in a hydrocephalic shunted child.

Extradural hematoma (EDH) is considered to be a rare complication of head trauma in children, and represents a serious and urgent pathology from which complete recovery can be expected if specialized treatment is instituted in time. In this article, the authors report the potential danger to a hydrocephalic shunted child who was apparently asymptomatic at the time of hospital admission with a mild head injury and developed an EDH of venous origin. This child had a rapid (time interval from injury to decerebrate posture of about 2 h), atypical (remained asymptomatic most of the time until abruptly deterioration) and fatal course, stressing the importance of early diagnosis and rapid therapy in order to avoid the death of the patient. The authors discuss the role of the ventriculoperitoneal shunting system in the lack of clinical symptoms associated with the presence of a giant EDH and a rapid and fatal course, and stress the importance of computed tomographic (CT) scanning in these patients, even if they are asymptomatic. If a skull fracture is suspected, a CT scan must be performed without delay.

[Acoustic neuroma in children without association with neurofibromatosis. Report of 2 cases]. / Neurinoma do acústico em crianças sem associação com neurofibromatose. Relato de dois casos.

Acoustic schwannoma is the most common tumor of the cerebellopontine angle in adults and is rarely found in children without neurofibromatosis. In the literature there are 18 children under the age of 16 with such tumor. Two female patients with age of 9 and 15 years old with acoustic schwannoma without neurofibromatosis are related. Progressive deafness followed by signs and symptoms of a posterior fossa tumor were the initial complaint of both, as well as of the other related cases and in adulthood.

[Medulloblastoma in adults: analysis of a casuistics and surgical results]. / Meduloblastoma em adultos. Análise de uma casuística e resultados cirúrgicos.

We report on 15 cases of medulloblastoma of adult onset (8 male and 7 female) operated upon posterior fossa approach from February 1988 to October 1995. Tumors were localized in cerebellar hemisphere in 7 cases (one with extension to supratentorial notch and another case reaching the cerebello-pontine angle cistern), in vermis and hemisphere in four, only in vermis in another four. Resection was total in seven patients, subtotal in other seven, and partial in one. There was no operative mortality. Aspects regarding biological behavior, diagnosis, pathological findings, surgery and survival are discussed as well as prognostic factors.

[Infections of cerebrospinal fluid shunts in children. Review of 100 infections in 87 children]. / Infecções de derivações liquóricas em crianças. Revisão de 100 infecções em 87 crianças.

An analysis of 100 infections in 87 children treated with shunts in the period of 1982 to 1995 is reported. The clinical presentation has been more frequently secondary to inflammatory signals. Staphylococcus were the most frequently microorganisms found. Infection by Gram negative agents was more aggressive and directly related with failure of therapy. Treatment included since only systemic antibiotics until withdrawal of shunt with use of systemic and intrathecal antibiotics. The best therapeutic results were obtained with withdrawal of shunt system and replacement by external shunt system associated to systemic antibiotics. In our experience this management must be accepted for treatment of this severe complication.

[Cerebellar astrocytomas in childhood. Experience with 25 cases]. / Astrocitomas do cerebelo na infância. Experiência em 25 casos.

The experience with the surgical treatment of cerebellar astrocytomas in 25 children is reported. The clinical presentation, incidence, CT-scan diagnostic studies, pathology, recurrence and treatment aspects are discussed. The series included children until 10 years old with peak (7 cases) in the 7th year of age. The more frequent opening symptoms were: headache, vomit and gait disturbances. No surgical mortality occurred in the series. The authors conclude that surgical radical resection is the best therapeutics for this type of tumor and that radiotherapy is indicated only for tumors with malignant histology.

[Arachnoid cyst and pseudotumor cerebri: case report]. / Cisto de aracnóide e pseudotumor cerebral.

Report on a 12 years old patient with an arachnoid cyst of posterior cranial fossa and pseudotumor cerebri. This patient is a shunt dependent of his cyst-peritoneal shunt. This association and evolution of this pacient suggest a common and specifical pathogenic mechanism of these two pathologies based in a disturbance of the cerebral fluid circulation.

[Depressed skull fractures in children under 2 years of age. Retrospective study of 43 cases]. / Fratura com afundamento craniano em crianças com menos de dois anos de idade. Estudo retrospectivo de 43 casos.

Depressed skull fractures (DSF) in infancy and childhood are frequent but only a few articles make an analysis in children with age between 0 and 2 years. This is a retrospective study of 43 patients with DSF and age ranged from 0 to 2 years. Falls and traffic accidents were the most common causes. Most patients were admitted in alert state. 69.8% of the patients were submitted to surgical treatment. The parietal bone was more frequently injured (55.8%). Most of the patients had type 1 DSF (the depressed bone remains connected to the cranial vault). The incidence of associated lesions of nervous system was lower than reported in the literature.

[Lipoma of the corpus callosum with extracranial extension through frontal bone defect]. / Lipoma do corpo caloso com extensão extracraniana através de falha óssea frontal.

Intracranial lipoma are usually localized in the corpus callosum and rarely extends to the subgaleal region. Only eight cases of lipoma of the corpus callosum with extracranial extension were reported in the literature. Seizures and mental retardation were the most common clinical findings in these patients. Data from the literature show that resection of intracranial lipoma has catastrophic results. The resection must be restricted to the extracranial portion. We report a child with lipoma of the corpus callosum with extracranial extension with no neurologic deficit submitted to resection of the extracranial extension.

Transient mutism following a posterior fossa approach to cerebellar tumors in children: a critical review of the literature.

Transient mutism has been known as a rare complication following a posterior fossa approach to cerebellar tumors and its cause has not been clearly elucidated. The cerebellar mutism is not accompanied by cranial nerve deficits and disorders of consciousness. Since 1985 only 23 cases of mutism following removal of a cerebellar tumor in children have been reported in the literature. Two additional cases have been operated upon in our department. Extensive injury to the vermian and paravermian cerebellar area, involving the hemispheric cortex, cerebellar peduncles, fibers from the dentato-thalamocortical pathway, and dentate and interpositum nuclei may be the most important anatomical substrate of mutism. The mechanism of such transient mutism seems to be a complex of two or more factors (vascular disturbances due to manipulation or retraction of the cerebellar region around the IV ventricle and emotional factors). On the basis of these 25 cases the major features of the cerebellar mutism are discussed.

[Magnetic resonance imaging in spinal dysraphisms]. / Ressonância nuclear magnética nos disrafismos espinais.

We studied by magnetic resonance imaging the incidence of associated malformations in a group of 38 patients with diagnosis of spina bifida followed in our outpatient clinic. Twenty-two children were born with spina aperta and 16 with spina bifida occulta. Tethered cord was found in the majority of patients of both groups. Syringomyelia was found in 13 patients. The craniocervical region was studied in 11 patients with the diagnosis of spina bifida aperta; Chiari malformation was found in 8. The results point to the fact that asymptomatic malformations detected by MRI techniques are frequent in patients with spina bifida.

Extradural hematomas in children.

The authors studied 58 patients, under 12 years of age, admitted to the Emergency Room of the Sao Paulo University Hospital between September 1987 and November 1991 with the diagnosis of traumatic extradural hematoma. Emphasis was given to the etiology of injury, the time interval from head injury to emergency room evaluation, the clinical features and the outcome after surgical and nonsurgical management. The site of hematoma was defined and correlated with the presence of skull fractures. Computerized tomography is the main diagnostic method, although it is not definitive. Fifteen patients were treated nonsurgically according to rigid clinical and radiological parameters. The overall mortality rate was 3.4%.

[Conservative treatment of laminar extradural hematomas in children]. / Tratamento conservador dos hematomas extradurais laminares na infância.

We successfully treated by nonsurgical methods 15 children with laminar epidural hematomas (EH), with minimal neurological symptoms and no signs of brain herniation. These EH were discovered 30 minutes to 5 days after head injury. The majority were localized in the parietal region. All children recovered without surgery from 2 to 12 days after hospitalization and all had evidence on CT scan of spontaneous clot reabsorption. We discuss the criteria for patient selection for this kind of treatment on EH in children.

[Traumatic extradural hematoma in childhood and normal early computed tomography: report of 2 cases]. / Hematoma extradural traumático na infância e tomografia computadorizada precoce normal. Relato de dois casos.

The cases of two children with traumatic extradural hematoma with an early normal CT scan are reported. Consciousness impairment occurred in them respectively 20 and 60 hours after admission, and CT at this occasion evidenced extradural hematoma. Serial neurological examinations are recommended for children with large traumatic cephalohematoma associated or not to skull fracture and in whom an initial early CT was normal, since they can lately develop extradural hematoma.

Steroid receptors in meningiomas.

Cytosolic estrogen (ER), progesterone (PR), androgen (AR) and glucocorticoid receptors (GR) were evaluated in 10 meningiomas using a dextran charcoal coated method. We consider as positive specific receptor values greater than or equal to 10 fMol/mg protein. In this study 20% of the meningiomas contained very low titers of specific ER. PR was detectable in 90% of the tumors, at high levels. The mean PR content of PR+ tumors was 60 +/- 38 fMol/mg prot. GR and AR were present in moderate levels, in 70% of the tumors. Competition studies demonstrated steroid specificity for these hormone-binding proteins. Female patients have a higher receptor incidence and titer. In conclusion, it can be hypothesized that the meningioma are a target tissue for steroids and that endocrine therapy may be relevant to unoperable and/or recurrent tumors.

Steroid receptors in intracranial tumors.

Cytosolic estrogen (ER), progesterone (PR), androgen (AR), and glucocorticoid (GR) receptors were evaluated in 6 meningiomas and 12 gliomas (6 astrocytomas and 6 glioblastomas) using a dextran-coated charcoal method. Thirty-three percent (two) of the meningiomas and 17% (two) of the gliomas contained positive (greater than or equal to 10 fmol/mg protein) but low ER levels. On the other hand, positive PR, GR, and AR levels were detectable in 100% (six) of the meningiomas. PR was present in 50% (six), AR in 42% (five), and GR in 58% (seven) of the gliomas. PR, GR, and AR values were statistically higher in meningiomas than in neuroepithelial tumors.

The use of prophylactic systemic antibiotics in compound depressed skull fractures in infancy and childhood.

The authors described their experience with the use of prophylactic antibiotics in children with compound depressed skull fractures. The analysis of the results of the treatment of patients with or without systemic antibiotics and with or without the replacement of the bone fragments showed no difference in the infection rate, any one was the method used.