Lateral-type posterior fossa ependymomas in pediatric patients: a national collaborative study.

PURPOSE: To review a multicentric series of lateral-type posterior fossa ependymomas operated in the last ten years and to analyze the factors related to clinical evolution and tumor survival. METHODS: Descriptive, retrospective study. Active members of the Spanish Society of Pediatric Neurosurgery were invited to participate in this multicentric study. Clinical and radiological data were incorporated to an open database. The role of histologic grade, grade of resection, postoperative morbidities, and clinical follow-up was evaluated through bivariate associations (chi-square), Kaplan-Meier's curves (log-rank test), and multivariate analysis (binary logistic regression). RESULTS: Fourteen centers entered the study, and 25 cases with a minimum follow-up of 6 months were included. There were 13 boys and 12 girls with a mean age close to 3 years. Mean tumor volume at diagnosis was over 60 cc. A complete resection was achieved in 8 patients and a near-total resection in 5 cases. Fifteen tumors were diagnosed as ependymoma grade 2 and ten as ependymoma grade 3. Major morbidity occurred postoperatively in 14 patients but was resolved in twelve within 6 months. There were six cases of death and 11 cases of tumor progression along the observation period. Mean follow-up was 44.8 months. Major morbidity was significantly associated with histologic grade but not with the degree of resection. Overall and progression-free survival were significantly associated with complete surgical resection. At the last follow-up, 16 patients carried a normal life, and three displayed a mild restriction according to Lansky's scale. CONCLUSIONS: Lateral-type posterior fossa ependymomas constitute a specific pathologic and clinical tumor subtype with bad prognosis. Gross total resection is the goal of surgical treatment, for it significantly improves prognosis with no additional morbidity. Neurological deficits associated to lower cranial nerve dysfunction are common, but most are transient. Deeper genetic characterization of these tumors may identify risk factors that guide new treatments and stratification of adjuvant therapies.

Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis.

CASE REPORT: A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child's condition has markedly improved. DISCUSSION AND CONCLUSIONS: A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child's pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.

Intracranial hemorrhage following surgery for occult spinal dysraphism: a case-based update.

BACKGROUND: Intracranial cerebrospinal fluid (CSF) volume depletion causes diverse clinical syndromes most of them constituting the manifestations of decreased intracranial pressure. Subdural collections or chronic subdural hematomas are the best-known consequences of persistent CSF leaks, especially in overshunted hydrocephalus. Continuous CSF escape also occurs after lumbar puncture, spinal anesthesia, and diverse spinal surgeries. CASE DESCRIPTION: A 6-year-old boy submitted to reoperation of spinal cord compression due to partial sacral agenesis complained of postoperative orthostatic headaches and vomiting initially attributed to CSF hypotension. There were neither subcutaneous fluid accumulations nor CSF leakage from the wound. The child was treated with strict bed rest and intravenous hydration for 5 days. On reassuming orthostatism, the patient had syncope but did not hit his head. A cranial computerized tomography scan showed an acute subdural hematoma that was managed conservatively with total recovery. DISCUSSION AND CONCLUSION: A review of current literature showed scanty reports of acute intracranial bleeding occurring after CSF depletion following spinal surgical procedures. To our knowledge, our reported patient represents the second case of this occurrence following surgery for closed spinal dysraphism in a child. The authors briefly review documented instances of acute subdural hematoma following spinal procedures, advise about its diagnosis, and suggest preventive measures.

Wind instruments and headaches.

The authors illustrate the cases of two children with headaches, one diagnosed with Chiari type 1 malformation and the other with hydrocephalus, who played wind instruments. Both patients manifested that their headaches worsened with the efforts made during playing their musical instruments. We briefly comment on the probable role played by this activity on the patients' intracranial pressure and hypothesize that the headaches might be influenced by increases in their intracranial pressure related to Valsalva maneuvers. We had serious doubts on if we should advise our young patients about giving up playing their music instruments.

Severe spinal cord injury in craniocervical dislocation. Case-based update.

BACKGROUND: Craniocervical distraction injuries, including atlanto-axial dislocation (AAD) and atlanto-ocipital dislocation (AOD), are often associated with severe spinal cord involvement with high morbidity and mortality rates. Many patients with these injuries die at the accident scene, but advances in emergency resuscitation and transport permit that many patients arrive alive to hospitals. DISCUSSION: Children with craniocervical distraction injuries usually present with a severe cranioencephalic traumatism that is the most relevant lesion at admission. After resuscitation and hemodynamic stabilization, the spinal cord damage appears as the main lesion. Apnea and quadriparesis, or quadriplegia, are usually present at the onset. Early diagnosis and management perhaps decrease life-threatening manifestations of the spinal lesion. But even so, the primary spinal cord insult is often irreversible and precludes obtaining a satisfactory functional outcome. PATIENTS AND METHODS: We report the findings of four children with craniocervical distraction injuries (AOD and AAD) who presented with severe spinal cord damage. All patients were admitted with respiratory distress or apnea together with significant brain injuries. The medical records pertaining to these patients are summarized in regard to clinical features, management, and outcome. CONCLUSIONS: In spite of timely and aggressive management, craniocervical injuries with spinal cord involvement continue to have a dismal prognosis. Outcome is closely related to the severity of the initial brain and spinal cord damage and is nearly always fatal in cases of complete spinal cord transection. Priority should be given to life-threatening complications. Ethic issues on indications for surgery deserve a detailed discussion with the children's parents.

Pediatric head injuries from earthquakes.

By means of some illustrations, the authors briefly report the effects of some accidental head injuries caused by diverse mechanisms occurring in children. Many of these accidents seem to be preventable, but others are completely unavoidable and escape prevention as the one that is depicted in the cover of this issue.

Pediatric crushing head injury: biomechanics and clinical features of an uncommon type of craniocerebral trauma.

BACKGROUND: Head injuries constitute one of the leading causes of pediatric morbidity and mortality. Most injuries result from accidents involving an acceleration/deceleration mechanism. However, a special type of head injury occurs when the children sustain a traumatism whose main component is a static load in relation to a crushing mechanism with the head relatively immobile. PATIENTS AND METHODS: We report a series of children who sustained a craniocerebral injury of variable severity produced by head crushing. We also analyze epidemiological and clinical data, and biomechanics in these injuries. RESULTS: Mean age of the group (13 boys/6 girls) was 4.1 years. All patients showed external lesions (scalp wounds or hemorrhage from the nose, ears, or throat). Eleven children were initially unconscious. Six children presented cranial nerve deficits in addition to impaired hearing. Skull base fractures were seen in most cases with extension to the vault in 11 instances. Fourteen patients had an associated intracranial lesion, including two with diffuse axonal injury. Surgery was performed in three instances. Only seven patients were left with sequelae. DISCUSSION AND CONCLUSIONS: The observed skull, brain, and cranial nerve lesions corresponded to a mechanism of bilateral compression of the children's heads mainly occasioned by a static load, although an associated component of dynamic forces was also involved. The skull and its covering and the cranial nerves were the most severely affected structures while the brain seemed to be relatively well preserved. Most crush injuries appear to be preventable by the appropriate supervision of the children.

Chiari type 1 anomaly in pseudohypoparathyroidism type Ia: pathogenetic hypothesis.

AIM: This study aims to report a patient with Chiari type 1 malformation (CM1) occurring in the context of pseudohypoparathyroidism type 1a (PHP-Ia) that we believe represents the first instance of this association in the current literature. CASE REPORT: The authors describe the case of a 6-year-old girl diagnosed with PHP-Ia who presented an associated tonsillar descent. During the follow-up, the skull vault and the occipital squama became extremely thickened at the same time as the tonsillar herniation showed a marked regression. DISCUSSION: Chronic tonsillar descent has been reported in diverse genetic and metabolic diseases of bone. A constant finding in PH-Ia consists of changes that mainly involve the bones of the patients' hands and feet. Cerebral anomalies have also been documented in PHP-Ia, especially cerebral calcifications, but in contrast involvement of the skull bones has seldom been described in this condition. The authors briefly discuss the probable role played by the observed skull changes in the origin and subsequent regression of the tonsillar descent in this child. CONCLUSIONS: We suggest that CM1 may develop in patients with PHP-Ia and that it should be actively sought, especially in individuals diagnosed with PHP-Ia presenting with neurological manifestations. Probably, the seeming rarity of chronic tonsillar descent in PHP-Ia is due to the fact that many patients with this condition are rarely investigated with magnetic resonance.

Hydrocephalus and arachnoid cysts.

AIM: The management of arachnoid cysts (AC) remains controversial. An additional problem derives from the management of hydrocephalus associated with an AC. In this work, we discuss existing procedures proposed in the current literature for their treatment. METHODS: We reviewed selected reports on intracranial ACs placing special interest in those about the association of hydrocephalus and ACs. We also briefly surveyed data of our patients with this association. RESULTS AND DISCUSSION: Hydrocephalus is often found in midline and posterior fossa ACs. Interhemispheric lesions may also evolve with ventriculomegaly, while middle fossa lesions rarely produce hydrocephalus. Patients' age, cyst location and size, and macrocephaly have all been related to the development of hydrocephalus. Some authors remark on the role played by hydrocephalus and hypothesize that some ACs would result from disturbed cerebrospinal fluid (CSF) dynamics. They also propose that ACs might represent a localized form of hydrocephalus. We also comment on hydrocephalus in relation to the diverse locations of ACs. Neuroendoscopic techniques have transformed previous ways of management as cystoperitoneal shunting and open fenestration. CONCLUSIONS: ACs may be pathogenetically related with hydrocephalus, and conversely, ACs may cause hydrocephalus. In some patients, aberrant CSF dynamics seems to play a major role in the development of both cyst and hydrocephalus. Hydrocephalus and ACs may be treated exclusively with neuroendoscopic procedures, although some patients will still require CSF shunting. The ideal option seems to consist of choosing the method that offers the highest success with a single procedure for treating the hydrocephalus and the AC simultaneously.

Craniosynostosis in Kabuki syndrome.

Niikawa-Kuroki, or Kabuki syndrome (KS), is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, short stature, and mental retardation. Neurological manifestations and CNS anomalies have been described in some patients with this condition. However, craniosynostosis has been documented in only 4 patients with KS who did not undergo operations. The authors report a case of KS with unicoronal synostosis that constitutes the first documented instance of a patient with this syndrome submitted to surgery. Previous reported instances of craniosynostosis occurring in KS are briefly reviewed. Although rarely documented, craniosynostosis might represent a relatively frequent feature of this syndrome. Kabuki syndrome should be considered at the time of evaluating children with craniosynostosis. The diagnosis of KS can be suspected from the patients' characteristic facial features. Kabuki syndrome appears to be an underdiagnosed condition in the craniosynostosis population. Given that most patients with this syndrome suffer from only mild to moderate mental retardation, surgical correction can be considered in instances of KS with craniosynostosis.

Bilateral ethmoidal dural arteriovenous fistula: unexpected surgical diagnosis.

Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies.

Posterior fossa arachnoid cysts and cerebellar tonsillar descent: short review.

The objective of this study was to analyze the association of cerebellar tonsillar descent and syringomyelia in patients with posterior fossa arachnoid cysts. We reviewed the medical records of ten patients (mean, age 33; range, 24-49 years) diagnosed with posterior fossa arachnoid cyst and tonsillar descent. Symptoms evolved over a mean of 12 months (range, 6 months to 3 years). Syringomyelia was present in six cases. Six patients underwent a suboccipital craniectomy, three cases underwent an additional C1 laminectomy, and a further case had a limited craniectomy and tonsillar reduction. Three patients were also treated for hydrocephalus: one with a ventriculoperitoneal shunt and two with endoscopic third ventriculostomy. Two patients had conservative treatment. The posterior fossa arachnoid cysts were located at the vermis-cisterna magna (n = 4), the cerebellar hemispheres (n = 2), the cerebellopontine angle (n = 3), and the quadrigeminal cistern (n = 1). A patient with achondroplasia showed features of platybasia. Associated malformations included craniofacial dysmorphism in a patient diagnosed of trichorhinophalangeal syndrome and a case with a primary temporal arachnoid cyst. After a mean follow-up of 2 years (range, 3 months to 5 years), four patients showed resolution of their neurological symptoms, and two exhibited persisting ocular findings. Headaches and nuchalgia improved in four cases and persisted in four. Syringomyelia was resolved in four patients and improved in two. Patients harboring a posterior fossa arachnoid cyst may evolve with acquired Chiari malformation and syringomyelia. Initial management should be directed to decompressing the foramen magnum and should include the resection of the arachnoid cyst's walls. A wait-and-see attitude can be implemented in selected cases. In our experience, hydrocephalus should be properly addressed before treating the arachnoid cyst.

Syringomyelia with quadriparesis in CSF shunt malfunction: a case illustration.

BACKGROUND: Shunt malfunction typically presents with headaches, vomiting, and impaired consciousness. Paraparesis has been rarely reported as the initial manifestation of valve dysfunction. CASE ILLUSTRATION: A 17-year-old boy was admitted with invalidating quadriparesis that after neuroimaging evaluation was found to be produced by communicating syringomyelia attributed to shunt malfunction. DISCUSSION AND CONCLUSION: Persistence of the central canal in association with communicating hydrocephalus may lead to the development of communicating syringomyelia. In this context, insufficient drainage of CSF produced by shunt dysfunction may evolve exclusively with symptoms and signs of spinal cord involvement in the absence of the usual cerebral manifestations related to shunt failure.

Cavernous hemangiomas of the cranial vault in infants: a case-based update.

BACKGROUND: Mass lesions arising in the scalp or the cranial vault of children require a significant diagnostic work-up but their nature, in most instances, can only be clarified after histopathological study. Tumors of osseous consistency or merging from within cranial bones are seldom susceptible of fine needle aspiration biopsy and require excision. Ossified cephalhematoma is a rare entity that is usually related to perinatal or accidental trauma. Exceptionally, ossified cephalhematoma has a non-traumatic origin and arises from a different condition, as vascular tumor or malformation. DISCUSSION: Cavernomas of the skull are infrequent and occur in middle-aged individuals. Skull cavernomas in infants lack the sunburst radiographic pattern seen in adults and may present as a bone-like tumor whose appearance is identical to that of ossified cephalhematoma. A literature search showed only two previous reports of patients younger than 1 year with skull cavernomas presenting with associated ossified cephalhematomas. We hypothesize that this type of skull cavernomas are congenital in origin and that they manifest precociously due to the occurrence of early bleeding. CASE ILLUSTRATION: We report a 12-month-old boy presenting with a hard tumor arising on his right parietal bone that was present since birth. After diagnostic work-up, the bone tumor and the associated intraosseous hematoma were removed. Interestingly, the source of the hematoma was a cavernous hemangioma originating in the skull. CONCLUSIONS: Calvarial cavernous hemangiomas may bleed precociously, even before birth, and manifest as large intraosseous hematomas. Differential diagnosis against ossified cephalhematoma can only be established through histopathological study.

Massive hemorrhage in hemangioblastomas Literature review.

Massive hemorrhage is a very uncommon event among hemangioblastomas. Forty-four cases have been reported before this review. Thorough analysis of all reported cases on literature was accomplished. The majority presented as parenchymatous or subarachnoid bleedings. Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment. To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma. When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor. Solid type, large size, and spinal-radicular locations are more frequently related to hemorrhage. Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.

Hydrocephalus and Chiari type 1 malformation in macrocephaly-cutis marmorata telangiectatica congenita: a case-based update.

BACKGROUND: Macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) is a newly described condition characterized by macrocephaly (megalencephaly), cutis marmorata telangiectatica congenita, macrosomia and/or asymmetric growth, central nervous system abnormalities and neurological manifestations. DISCUSSION: This condition is usually documented in the Genetics' literature but it has been scarcely disseminated among neurosurgeons. Sudden death of uncertain origin has been reported in children with M-CMTC. Hydrocephalus and tonsillar herniation often occur in this syndrome. The appearance of symptoms and signs of intracranial hypertension or of brain stem compression in M-CMTC patients may herald the development of irreversible neurological damage or death. ILLUSTRATIVE CASES: We report two children diagnosed with M-CMTC and hydrocephalus who were given a ventriculo-peritoneal shunt. In addition, one of them exhibited tonsillar herniation. CONCLUSIONS: Given the high incidence of hydrocephalus and/or tonsillar herniation that occurs in M-CMTC, serial neuroimaging studies and neurosurgical referral seem to be warranted in children with this syndrome.

Cervical extramedullary mass lesion due to chronic CSF overshunting: case report and literature review.

INTRODUCTION: Several emerging clinical and neuroimaging conditions due to CSF intracranial hypotension have been documented. Recently, a few instances of a severe form of cervical myelopathy due to CSF overshunting have also been reported. Patients with this type of cervical myelopathy usually evolve with marked clinical manifestations of spinal cord involvement. MATERIALS AND METHODS: In this article, we report a 20-year-old girl, previously given a ventriculoperitoneal shunt at age 6 years, who presented with neck pain after a whiplash injury. RESULTS AND DISCUSSION: Magnetic resonance imaging revealed a cervical extramedullary mass that mimicked a cervical extradural hematoma. Further neuroimaging studies demonstrated that it corresponded to an engorged cervical epidural venous plexus that we attributed to chronic CSF overdrainage. After expectant management, the patient experienced a total recovery of her symptoms. To our knowledge, this is the first report of this condition occurring in an asymptomatic patient. Our aim in reporting this patient is twofold: (1) to warn the clinician about these neuroimaging findings that may mimic a cervical extradural hematoma and (2) to illustrate that engorged cervical epidural plexus may occur in patients in the absence of clinical manifestations of myelopathy. Recognition of this entity is important to distinguish it from a mass lesion in which a surgical intervention might be indicated.

Acute cholecystitis complicating ventriculo-peritoneal shunting: report of a case and review of the literature.

CASE: A 3-year-old boy underwent emergency external ventricular drainage and excision of a fourth ventricle anaplastic ependymoma. A week later, the child was given a ventriculo-peritoneal shunt. Fourteen days after shunting, the child developed a subphrenic abscess and acute cholecystitis that required surgery. RESULTS: A Staphylococcus epidermidis was isolated both from the ventricular catheter and CSF and from the subphrenic abscess and the gallbladder. To our knowledge, this is the first report of cholecystitis evolving as a descending shunt infection. The current literature related with this unique complication is briefly reviewed.