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.

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.

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.

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.