Multiple hereditary osteochondromatosis with spinal cord compression: case report.

OBJECTIVE: The purpose of the report is to describe a patient with hereditary osteochondromatosis and spinal cord compression at the thoracic level. CLINICAL FEATURES: An 8-year-old patient with hereditary osteochondromatosis inherited from his father presented paraparesis in the left foot, leading to complete paralysis in both legs. INTERVENTION: In a CT scan, a bony tumor rising from the posterior wall of the T3 body narrowing the spinal canal, and the MRI spinal cord compression at the same level and the hydrosyringomyelic cavity extended to the conus medullaris; with an anterior thoracic approach to T2-T4, the fibro-cartilaginous tumor was removed, and the stabilization was completed with bone graft and a plate. Two months after surgery, the patient recovered strength in both legs. CONCLUSIONS: A detailed family history through examination-guided advanced imaging and biopsy provides useful information for diagnosis and appropriate management of occupative lesions in patients affected with multiple hereditary exostosis.

Stereotactic biopsy for brainstem tumors in pediatric patients.

OBJECTIVE: Our aim is to describe clinical and paraclinical features in patients who underwent stereotactic-guided biopsy for brainstem tumors. METHODS: A study of case series was made by reviewing the records of patients who underwent stereotactic biopsy for brainstem tumors. RESULTS: Stereotactic biopsy for brainstem tumors was performed (between 2000 and 2008) in 20 children (11 girls, and 9 boys), mean age 7.95 +/- 3.12 years at the time of diagnosis. The mean time from onset of symptoms to diagnosis was 6.59 +/- 13.58 months (0.50-60 months). The most frequent symptoms and signs at onset were related to disturbance of cerebellar function and cranial nerve nuclei. Location was pontomesencephalic (35%), pontine (30%), pontomedullar (25%), and in the whole brainstem (10%). The most common type of image was intrinsic-diffused (55%). The histopathology was anaplastic astrocytoma (30%), followed by fibrillary and pilocytic types (25% each), low-grade astrocytoma (5%), high-grade astrocytoma (5%), and normal tissue (10%). Mild complications were observed in only two cases. CONCLUSIONS: Stereotactic biopsy done for clarifiying a diagnostic imaging in brainstem tumors is important in obtaining a definitive diagnosis with a low rate of complications.

[Medulloblastoma in pediatrics. Current prognosis and treatment]. / Meduloblastoma en pediatría. Pronóstico y tratamiento en la actualidad.

In Mexico, Central Nervous System (CNS) tumors are the third most common childhood cancers. Medulloblastoma constitutes 20% of the primary CNS tumors and 40% of all cerebellar tumors, the single most common brain tumor among children. It originates over the external granular layer that normally migrates from the vermis to the surface of the cerebellum hemispheres and from there to the deep portions of the internal granular layer. These tumors infiltrate profusely the cerebellar cortex. The dissemination process can occur through the spinal fluid with seeding along the subarachnoidal space and around the spinal chord They eventually produce metastasis mainly to bone, liver, and bone marrow. There is a group of well identified prognostic factors that are relevant for each individual patient and that can be applied for multidisciplinary treatment purposes. The objective of the present review is to emphasize on new research findings and the overall survival that can be achieved with modern treatment programs.

Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City.

OBJECTIVE: The purpose of this study is to present our experience with ten cases of Central nervous system atypical teratoid rhabdoid tumor (CNS/ATRT). PATIENTS AND METHODS: A series of ten patients with CNS/ATRT, were diagnosed and treated between 1990 and 2005, at the National Institute of Pediatrics, in Mexico City. The gender, age of presentation, clinical features, tumor localization, imaging studies, grade of tumor resection, complications, adjuvant therapy, and survival are presented. RESULTS: The mean age at diagnosis was 37.8 months, seven cases were male, and their average clinical course was 1.3 months. The more common clinical presentation was intracranial hypertension with cranial nerve deficits; location was infratentorial in four patients and supratentorial in six. Hydrocephalus was present as the most common complication (seven cases). In nine patients, the grade of resection was total or subtotal. In one case, it was only possible to perform a biopsy. There were two cases with longer survival (9 and 16 months), and their tumors were resected in total or subtotal manner and received adjuvant therapy (radiotherapy and chemotherapy). CONCLUSIONS: Preliminary results, show that in older children, we can improve their survival with the subtotal or total resection of the tumor and the addition of chemotherapy and radiotherapy.

Chordoid meningioma of the foramen magnum in a child: a case report and review of the literature.

We report a 3-year-6-month-old boy with chordoid meningioma in the foramen magnum. Chordoid meningioma represents between 0.5 and 1.0% of all meningiomas, and it is frequently located in the supratentorial region. The patient started with an episode of instability, falls, and headache; after which, he developed cuadriparesis, cervical pain, and neck stiffness, which slightly improved after medical treatment, so he was referred to our hospital. Physical examination revealed left hemiparesis and cervical muscle spasm with left torticollis. Magnetic resonance imaging (MRI) demonstrated an intradural-extramedullary well-circumscribed, homogeneous enhancing mass, in the anterior part of the foramen magnum with cervical extension. The operative technique was the extreme-lateral craniocervical retrocondylar approach with total removal and dural coagulation. Histopathological examination demonstrated a chordoid meningioma, with meningotelial basophilic mucoid chordoma-like component in 80% of the tissue. The immunohistochemical stains were positive to epithelial membrane antigen, vimentin, and S-100 protein. At 10 months follow-up, the patient showed improvement in his neurological deficit with physical rehabilitation, and motor response in his extremities are now normal; neck stiffness with cervical spasm disappeared postoperatively. MRI showed no residual tumor. To our knowledge, this is the first report of a chordoid meningioma on the foramen magnum in a child.

Long-term survival in children under 3 years of age with low-grade astrocytoma.

OBJECTIVE: The purpose of this study is to analyze clinical aspects and disease-free survival (DFS) in children less than 3 years of age diagnosed with low-grade astrocytoma. METHODS: In a period of 24 years (1980-2004), a total of 43 (5.4%) children were registered with these characteristics. Twenty-three patients had pilocytic astrocytoma, 18 diffused, and 2 mixed. Thirty-one (72.1%) children had incomplete surgical tumor resection and 12 (27.9%) had a complete tumor resection. Twelve (27.9%) patients had cranial radiotherapy and 17 (39.5%) received chemotherapy. Overall survival was recorded in 23 (53%). DFS was 50% at 250 months of follow-up for the whole group. DFS for the supratentorial group was 60% at 250 months, whereas, for the infratentorial, it was 22% at 120 months (p = 0.008). CONCLUSION: The only favorable prognostic pattern was the supratentorial presentation. Radiotherapy and chemotherapy did not alter the outcome.

Clinical-etiological and therapeutic profile of 719 Mexican epileptic children.

RATIONALE: We performed this study with the intention of describing the clinical-etiological characteristics and therapeutic responses in a group of epileptic children seen at the Instituto Nacional de Pediatría (INP), a tertiary facility in Mexico City. METHODS: All patients who attended the Epileptic Clinic between March and June 1998 and fulfilled the selection criteria were enrolled in the study. Clinical and therapeutic response data were recorded. Three groups were formed by etiology. Statistical tests were two-tailed, with alpha=0.05. RESULTS: In all, 719 patients were studied. The distribution by etiology was as follows: group I, idiopathic (123 patients); group II, cryptogenic (132); and group III, symptomatic (464). In group I, 56% of the patients were female. Mean age at onset was 5 years 2 months (SD: 3 years 8 months) in group I; 1 year 11 months (SD: 2 years 5 months) in group II; and 2 years 10 months (SD: 3 years 1 month) in group III. The mean evolution time was 5 years 4 months (SD: 4 years) in all groups. The most frequent variety of epilepsy in the three groups was generalized epilepsy, followed by partial epilepsy. The following epileptic syndromes were identified: in group I, 28 patients had epilepsy with generalized tonic-clonic seizures, 15, absence epilepsy, and 6, benign rolandic seizures; in group II, all 32 patients had focal cryptogenic epilepsy; in group III, 235 had generalized symptomatic and 192, focal symptomatic epilepsy. The main etiologies were hypoxic ischemic encephalopathy (24%) and neural infections (22%). Appropriate seizure control was achieved in 108 (87%) patients in group I; 84 (64%) in group II; and 315 (68%) in group III. In group I, no patient needed more than two antiepileptic drugs and 90% had normal psychomotor development. CONCLUSION: When the three groups were compared in terms of appropriate epileptic control and normal psychomotor development, group I differed from the other groups and the difference was statistically significant.

Brain tumors in children under 1 year of age: emphasis on the relationship of prognostic factors.

OBJECTS: Primary brain tumors in infants under 12 months of age have a different prognosis from older children. MATERIAL: A retrospective analysis was done in all patients less than 12 months old with primary brain tumors. RESULTS: Out of 1682 children with primary brain tumors, 61 (3.6%) were infants under 12 months old. The mean age at diagnosis was 181.6 days (SD 128) with a range of 1 to 364 days. There were 37 males (60.6%). The most common tumor was astrocytoma ( n=22) (36%). Supratentorial tumors were present in 63.9% but this was not related to survival ( p=0.1095). Complete surgical resection ( n=14) was favorable for survival ( p=0.039). Intracranial hypertension at diagnosis did not influence survival ( p=0.89). The overall survival rate was 32%, mean 42.08 (SD 7.38). A total of 24 patients are alive and without evidence of disease. CONCLUSION: Complete surgical resection was necessary for a favorable prognosis, and the long-term effects are a valid problem.

Survival of children under 3 years old with medulloblastoma: a study from the Mexican Cooperative Group for Childhood Malignancies (AMOHP).

BACKGROUND: The prognosis of medulloblastoma in children under 3 years of age is poor. METHODS: A retrospective analysis was performed to evaluate children under 3 years of age with medulloblastoma. Overall survival (OS) and progression-free survival (PFS) were assessed in children with and without metastasis. RESULTS: A total of 534 children were diagnosed with medulloblastoma during the study period, 49 (9.1%) of whom were under 3 years of age and were evaluated. Their ages ranged from 5 to 35 months with a mean of 18.5 months. In 39 (79.6%) of these patients the tumors were staged as T3M0 or under, while 10 (20.4%) had metastasis at diagnosis. The OS was 38% and PFS 37% in the whole series, while PFS was 32% in those with metastasis and 40% in those without ( P=0.78). For those who received radiotherapy the PFS was 62%, and in those not treated with radiotherapy PFS was nil ( P=0.0001). When the children were divided into those who received surgical treatment plus chemotherapy and those who received surgery plus radiotherapy plus chemotherapy, the PFS was nil and 66%, respectively ( P=0.00001). CONCLUSION: Because of the high morbidity of radiotherapy in children under 3 years old, surgery continues to be the basis of improved prognosis, followed by chemotherapy.

Meduloblastoma en pediatría: pronóstico y tratamiento en la actualidad / Medulloblastoma in pediatrics: current prognosis and treatment

En México, los tumores del sistema nervioso central representan el tercer lugar de todas las neoplasias malignas. El meduloblastoma constituye 20% de los tumores primarios del sistema nervioso central y 40% de los que se originan en el cerebelo; es el tumor maligno más frecuente en la niñez. Su origen se sitúa en la capa granular externa, normalmente migra del vermis hacia la superficie de los hemisferios cerebelosos y de ahí hacia las porciones profundas para poblar la capa granular interna de las folias. Estos tumores infiltran difusamente a través de las capas moleculares de la corteza cerebelosa por debajo de la pía, similar a lo que ocurre normalmente en las etapas embrionarias. Se diseminan por la circulación del líquido cefalorraquídeo con siembras a lo largo del espacio subaracnoideo y alrededor de la médula espinal para eventualmente salirse del sistema nervioso central y diseminarse a hueso, hígado, médula ósea y otros sitios menos frecuentes. Existen en la actualidad factores pronósticos bien definidos, así como el concepto de tratamiento multidisciplinario que ha condicionado mejores expectativas de supervivencia. El objetivo de esta revisión es actualizar el conocimiento de este tipo de tumores en nuestro país, así como los resultados terapéuticos.

In Mexico, Central Nervous System (CNS) tumors are the third most common childhood cancers. Medulloblastoma constitutes 20% of the primary CNS tumors and 40% of all cerebellar tumors, the single most common brain tumor among children. It originates over the external granular layer that normally migrates from the vermis to the surface of the cerebellum hemispheres and from there to the deep portions of the internal granular layer. These tumors infiltrate profusely the cerebellar cortex. The dissemination process can occur through the spinal fluid with seeding along the subarachnoidal space and around the spinal chord They eventually produce metastasis mainly to bone, liver, and bone marrow. There is a group of well identified prognostic factors that are relevant for each individual patient and that can be applied for multidisciplinary treatment purposes. The objective of the present review is to emphasize on new research findings and the overall survival that can be achieved with modern treatment programs.

Tumores orbitarios en niños. Su abordaje quirúrgico transcraneal / Orbitary tumors in children. The transcranial surgical approach

De marzo de 1980 a diciembre de 1995 se trataron 23 pacientes con tumores orbitarios en el Departamento de Neurocirugía del Instituto Nacional de Pediatría (INP). En este trabajo se analiza edad, características clínicas, alteraciones radiológicas y de potenciales evocados; tipo de cirugía, diagnóstico histopatológico y resultados. En todos ellos se efectuó un abordaje craneoorbitario, tipo destechamiento simple, o bién, por fronto-orbitotomía. De estos pacientes, 11 tenían glioma del nervio óptico, tres displasia ósea fibrosa, dos meningioma, neurofibroma, histocitosis de células de Languerhans; uno astrocitoma anaplásico, teratoma, tumor neuroectodérmico primitivo y germinoma con áreas de coriocarcinoma. Se realizó, craneotomía con destechamiento orbitario (Nafziger) en 19 casos y fronto-orbitotomía en 4. Los resultados fueron clasificados de acuerdo a la escala de karnofsky; potenciales evocados postoperatorios, presencia de defecto estético y proptosis pulsátil. Buenos resultados fueron: Karnofsky mayor de 70, no recurrencia tumoral y no emperoramiento de potenciales Visuales; regulares: sin recurrencia tumoral y no empeoramiento de Potenciales Visuales; regulares: sin recurrencia tumoral, cambio en potenciales evocados, escala de Karnofsky entre 50 y 60; malos: recurrencia, lesión visual agregada en potenciales evocados y Karnofsky menor de 50. El reporte muestra la variedad de lesiones intraorbitarias que pueden ser abordadas por vía transcraneal

Plagiocefalia: etiología, diagnóstico y tratamiento / Plagiocephaly: etiology, diagnosis and treatment

La plagiocefalia o deformidad craneal en paralelogramo es una alteración que causa un cambio estético del niño que la sufre por lo que debe procurarse su diagnóstico temprano y su oportuno tratamiento. Esta deformidad tiene varias etiologías que inculyen entre otras, moldeamientos craneales en útero, tortícolis y las sinostosis prematuras de la sutura coronal o de la sutura lambdoidea. En el caso de la sinostosis de la sutura lambdoidea o coronal, el tratamiento es quirúrgico. En el trabajo se describe la técnica quirúrgica empleada en los casos de sinostosis lambdoidea que consiste en una amplia craniectomía occipital con resección de tres a cinco mm. de la porción lateral del peñasco en el lado afectado. Se intervinieron 24 niños con este procedimiento en los que se obtuvieron resultados muy satisfactorios por lo cual se recomienda el uso de esta técnica quirúrgica.