Estenosis acueductal en acondroplasia: a propósito de un caso / Aqueductal Stenosis in Achondroplasia: a Clinical Case Report

Resumen: La acondroplasia es la condición asociada a talla baja desproporcionada más frecuente, caracterizada por un crecimiento óseo anormal, que resulta en talla baja con extremidades cortas e inteligencia normal. Una de las complicaciones más habituales es la compresión medular, que puede ocurrir a cualquier nivel, siendo más frecuente en la unión cráneo cervical, generando alta morbimortalidad en los primeros años de vida, principalmente por muerte súbita. Presentamos una paciente de 1 año 10 meses con diagnóstico precoz de acondroplasia, que presentó en su evolución estenosis acueductal con compresión medular, sintomática, pesquisada en control rutinario, que requirió cirugía descompresiva con buena evolución posterior. Palabras clave: Acondroplasia, estenosis acueductal, compresión medular sintomática, hidrocefalia, craniectomía suboccip

Achondroplasia is the most frequent cause of disproportionate short stature, it is characterized by abnormal growth of long bones, rendering a short-limbed individual of normal intelligence. A serious potential complication is spinal compression, which can happen at any level but is particularly common at the craniocervical junction. It can cause important morbidity during the first few years of life, including sudden death. We present a 22-monthold patient diagnosed with achondroplasia, who developed aqueductal stenosis with symptomatic spinal cord compression, diagnosed during a routine consultation, requiring decompressive surgery with excellent results. Key words: Achondroplasia, aqueductal stenosis, symptomatic spinal cord compression, hydrocephalus, suboccipital craniectomy.

Occipito-thoracic Fusion in Incidental Dystropic Os Odontoideum in a patient suspected with Osteogenesis Imperfecta: A Case Report.

Introduction: Os odontoideum is a rare lesion of the axis. Described as an ossicle, consisting of smooth and separate caudal portions of the odontoid process wherein its etiology has remained controversial. Os odontoideum complicating a possible osteogenesis imperfecta has not been reported before in the literature. Methods: We report the case of a 14-yr male patient presented with progressive weakness of both lower and upper extremities after a head trauma 10 months prior to admission, which presented as transient quadreparesis. Results: Magnetic resonance imaging of cervical spine showing cervicomedullary junction compression. Patient underwent surgical intervention and 3 months post operation, patient was reported to have steady gait and muscle grading of 5/5 on all extremities. Conclusion: Surgical fi xation and fusion in patients with instability may prevent catastrophic neurologic insult after minor trauma in the future.

Estenosis acueductal en acondroplasia: a propósito de un caso / Aqueductal stenosis in achondroplasia: report of a case

Achondroplasia is the most frequent cause of disproportionate short stature. Characterized by abnormal growth of long bones, it renders a short-limbed individual of normal intelligence. A serious potential complication is spinal compression, which can happen at any level but is particularly common at the craniocervical junction. It can cause important morbility during the first few years of life, including sudden death. We present a 22-month-old patient diagnosed with achondroplasia, who developed aqueductal stenosis with symptomatic spinal cord compression, diagnosed during a routine consultation, requiring decompressive surgery with excellent results.

La acondroplasia es la condición asociada a talla baja desproporcionada más frecuente, caracterizada por un crecimiento óseo anormal, que resulta en talla baja con extremidades cortas e inteligencia normal. Una de las complicaciones más habituales es la compresión medular, que puede ocurrir a cualquier nivel, siendo más frecuente en la unión cráneo cervical, generando alta morbimortalidad en los primeros años de vida, principalmente por muerte súbita. Presentamos una paciente de 1 año 10 meses con diagnóstico precoz de acondroplasia, que presentó en su evolución estenosis acueductal con compresión medular, sintomática, pesquisada en control rutinario, que requirió cirugía descompresiva con buena evolución posterior.

Suboccipital craniectomy with or without duraplasty: what is the best choice in patients with Chiari type 1 malformation? / Craniectomia suboccipital com ou sem duroplastia: qual a melhor escolha em pacientes com malformação de Chiari tipo 1?

The best surgical treatment for Chiari malformation is unclear, especially in patients with syringomyelia. We reviewed the records of 16 patients who underwent suboccipital craniectomy at our institution between 2005 and 2008. Of the six patients who did not undergo duraplasty, four showed improvement postoperatively. Two patients without syringomyelia showed improvement postoperatively. Of the four patients with syringomyelia, three showed improvement, including two with a decrease in the cavity size. One patient showed improvement in symptoms but the syringomyelia was unchanged. The cavity size increased in the one patient who did not show improvement. Among the 10 patients who underwent duraplasty, improvements were noted in four of the five patients without syringomyelia and in all of the five with syringomyelia. There is a suggestion that patients with syringomyelia may have a higher likelihood of improvement after undergoing duraplasty.

A melhor opção de tratamento cirúrgico na malformação de Chiari é desconhecido, especialmente em paciente com siringomielia. Nós revisamos casos de 16 pacientes submetidos à craniectomia suboccipital em nossa instituição de 2005 à 2008. Dos 6 pacientes que não foram submetidos à duroplastia, quatro tiveram melhora pós operatória. Dois pacientes sem siringomielia tiveram melhora pós operatória. Dos quatro pacientes com siringomielia, três tiveram melhora, incluindo dois com diminuição do tamanho da cavidade. Um paciente teve melhora da sintomatologia mas a siringomielia não se modificou. Dez pacientes foram submetidos à duroplastia. Melhora foi observada em 4 de 5 pacientes sem siringomielia e 5 de 5 casos com siringomielia. Há uma sugestão de que pacientes com siringomielia possam ter uma maior taxa de melhora após serem submetidos à duroplastia.

Malformación de Chiari y siringomielia: experiencia 2000-2008 / Chiari malformation and syringomyelia: experience 2000-2008

Objetive: to describe the postoperative outcome of patients with Chiari malformation (CM) and/or syringomyelia (SM). Methods: the clinical records of patients with CM and SM, treated from 2000 to 2008, were retrospectively reviewed. Data about sex, age, previous history, admission symptoms, magnetic resonance imaging (MRI), treatment modalities, outcome (Odom´s sacale) and postoperative complications were collected. Results: we treated 17 patients (1 male, 16 women); mean age: 39 years old (10-66). The admission symptoms were: pain in 11 cases, cerebellum/brain stem in 4 cases and spinal cord in 14 cases. Diagnosis was made by MRI: CM type I with SM 9 cases, CM I without SM 6 cases, CM type II with SM 1 case and idiopathic SM 1 case. MC II had myelomeningocele and ventricular shunting surgery at 3 months old. In 15 cases we performed a suboccipital craniectomy with duraplasty and C1 posterior arch resection (n:12) or C1-C2 posterior arch resection (n:3). One case had surgery al another institution and 1 case was not operated because symptoms did not progressed. SM persisted in 4 of the operated cases; in 3 of them a syringopleural shunting (SPS) was performed. In the idiopathic SM a SPS was also performed, but it became obstructed 3 months later. Complications were: meningitis 1 case, CSF fistula 1 case and meningitis + CSF fistula 1 case. In CM outcome after 1 year was: excellent-good in 13 cases, fair in 1 case and poor in 2 cases. Conclusion: in patients with CM and/or SM, postoperative clinical outcome was excellent-good in 13/16 cases.

Surgical Management of Acute Cerebellar Infarction

OBJECTIVE: The aim of this study is to determine which patients with progressively deteriorating acute cerebellar infarction would benefit from surgical treatment and which surgical procedure would best benefit them. METHODS: Seventy six patients were treated at our hospital for cerebellar infarction over the past 3 years. Sixty nine patients received conservative management in the neurological department of our hospital. Among them, 7 patients (5 males and 2 females; average age, 49 yrs) were referred to neurosurgical department because of mental deterioration and underwent emergency surgery. Five patients underwent external ventricular drainage with suboccipital craniectomy and two patients underwent suboccipital craniectomy alone. RESULTS: Of the 7 surgically treated patients, 4 patients experienced good recovery and 2 patients experienced moderate disability (disabled but independent) and 1 patient experienced severe disability (conscious but disabled). There was no death. CONCLUSION: In patients conservatively treated for cerebellar infarction and showing mental deterioration and radiologically evident brainstem compression and ventricular enlargement, we strongly recommend suboccipital craniectomy (plus optional external ventricular drainage in case of showing hydrocephalus) as a first treatment option.

Teratoma in the Posterior Cranial Fossa

A rare case of teratoma occuring in the posterior cranial fossa is reported. The tumor was partially encapsulated, and a surgical removal was accomplished. Teratoma is defined as a true tumor, composed of several types of tissues representing more than one germinal layer, which are foreign to the part in which it arises. The literature on teratomas located in the posterior cranial fossa is reviewed.

Stereotaxic Evacuation of Brain Stem Hemorrhage

Usually cases of brain stem hemorrhage reveal high mortality and poor prognosis in spite of meticulous medical or surgical treatment. Recently we experienced stereotaxic evacuation of brain stem hemorrhage with Brown-Robert-Wells(B.R.W.) system in four cases. If there were residual hemorrhage after initial aspiration, those were evacuated with Urokinase irrigation via stereotaxically placed catheter in the hematoma. The entry point of our operation get on the crossing point of midpupillary line and 1cm behind coronal structure. Preliminary results : 1) Volume of removed hematoma : 5ml(average). 2) Dates of urokinase irrigation : 2 to 4 days after operation. 3) No hydrocephalus, no surgical mortality. 4) Average Glasgow coma scale was improved from 6 in preoperative state to 12 in postoperative state. 5) Operation was performed within 24 hours. 6) Advantages of our procedure. (1) Accurate, simple and safe. (2) Under local anesthesia. (3) Less traumatic procedure. (4) Could remove hematoma completely with Urokinase.

An Ependymoma in the 4th Ventricle: Report of 1 Case

Storch is credited as being the first to describe in 1899 what we call "ependymoma" today. And ependymomas were first set apart as a single group by Bailey in 1924. The literature up to the present contains numerous reports of tumors classified as "ependymoma". From 1 to 6 percent of all intracranial tumors are ependymomas. As the above, the tumors are very rare. The incidence is higher in those with large proportion of young patients and average age is approximately 20 years. About 40 percent of intracranial ependymomas are supratentorial and the remainders are infratentorial. We experienced a case of 4th ventricular ependymoma with subarachnoid hemorrhage. A 23 year old female was admitted to the hospital because of severe headache and frequent vomiting, who had been intermittently suffered from the headache for 8 months prior to admission. On admission, the neurologic examinations revealed irritable mentality, vomiting and papilledema, and xanthochromic CSF was identified on lumbar puncture. X-ray films of skull series showed evidence of increased ICP. A mass lesion was suspected in the posterior fossa on vertebral angiogram and brain C-T films revealed a mass lesion in the midline of the posterior fossa, slightly enhanced on contrast study and associated with hydrocephalus. Following Torkildsen's shunting procedure suboccipital craniectomy was performed, and we removed the tumor mass incompletely within the 4th ventricle. The pathologically microscopic finding was papillary ependymoma, grade II. Total neuraxis irradiation was applied for 6 weeks. The post-operative course was satisfactory.

Subtentorial Empyema: Report of 2 Cases

Intracranial subdural empyema is a neurosurgical emergency and an unusual condition which carries a serious incidence of mortality and morbidity if untreated. The subdural empyema developes from varied sources, but paranasal sinus, the ear and the mastoid process are predominantly affected and subtentorial empyema usually originates from otogenic intracranial complication. Its manifestations are variable such as impaired consciousness, headache, hemiparesis, acute fits, pyrexia, meningism, vomiting, papilledema. ophthalmoplegia, hemianopsia and dysphasia. Subtentorial empyema has additional cerebellar dysfunction syndromes. Early accurate diagnosis and surgical drainage are the most important factors affecting the prognosis. Recently, we experienced two cases of subtentorial empyema which were very rare otogenic intracranial complications. These patients were treated with suboccipital craniectomy, but reaccumulations of empyema and subsequent cerebellar abscess in one case occurred. Finally these patients were recovered without neurologic deficits. Reviewing our two cases and the literature, we are going to describe about causes, symptoms and signs, method of diagnosis, treatment and prognosis of subtentorial empyema.

A Case of Intracanial Multiple Reticulum Cell Sarcoma

Reticulum cell sarcoma occurs rarely in central nervous system. It occupies 1-2.5% of all brain tumors. The prognosis is known to be very poor: 3 weeks of mean survival time in case of no treatment, 2 months of mean survival time in case of only surgery, 9 months-3 years of mean survival time in case of both surgery and radiation. We have experienced a case of reticulum cell sarcoma in brain and obtained a good result after surgery and radiation. We report our case with a brief review of the articles.

A Case of Intracanial Multiple Reticulum Cell Sarcoma

Reticulum cell sarcoma occurs rarely in central nervous system. It occupies 1-2.5% of all brain tumors. The prognosis is known to be very poor: 3 weeks of mean survival time in case of no treatment, 2 months of mean survival time in case of only surgery, 9 months-3 years of mean survival time in case of both surgery and radiation. We have experienced a case of reticulum cell sarcoma in brain and obtained a good result after surgery and radiation. We report our case with a brief review of the articles.

Surgical Treatment of Pontine Hematoma

Three cases of pontine hematoma accompanied by serious neurological symptoms with coma were treated surgically. All patients underwent suboccipital craniectomy with removal of hematoma and subsequently ventriculoperitoneal shunts were needed in all cases. Postoperatively all cases showed improvement with some neurological sequelae remained. Most of untreated pontine hematoma may be fatal, whereas surgical evacuation of the hematoma is safe and promising. We emphasize surgical treatment of pontine hematoma should be considered in any cases though they show serious neurological deficits.