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.

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.

Spinal dermal sinus and pseudo-dermal sinus tracts: two different entities.

BACKGROUND: Occult spinal dysraphism (OSD) encompasses various conditions. A dermal sinus tract (DST) consists of a duct communicating to the skin with deep structures that carries an important risk of infection. A different lesion consisting of a translucent skin opening and a fibrous tract that lacks a lumen can also be found in OSD. We termed this lesion pseudo-dermal sinus tract. PATIENTS AND METHODS: We reviewed clinical features of 20 patients with spinal skin orifices. The patients were classified into two categories: group 1 comprised children with true DST and group 2 included patients with skin dimples resembling a DST. AIMS: The aim of this study was to analyze differential features of patients in these two groups as they behaved dissimilarly in regard to clinical significance and outcomes, especially those concerning infectious risk. RESULTS: Children of group 1 (n = 8) presented with tiny skin orifices and with superficial or deep infection. In contrast, group 2 (n = 12) mostly manifested with neurological symptoms. No patient in group 2 developed an infection preoperatively. In both groups, magnetic resonance showed tracts that ended in different structures. Histopathology of the lesions were dissimilar, those of group 1 being hollow tubular structures lined by epithelium while those of group 2 being solid tracts of fibrous connective tissue. CONCLUSIONS: DST constitutes a clinicopathological diagnosis. Although sharing some common cutaneous and neuroimaging findings, both groups behaved differently. Patients of group 1 tended to show up with infection requiring urgent surgery. Patients of group 2 often presented with neurological manifestations and skin lesions, but needed no immediate operation.

Depressed skull fracture by a three-pin head holder: a case illustration.

UNLABELLED: BACKGROUND AND CASE REPORT: Many surgical procedures require a rigid immobilization of the patients' head, which is usually achieved by using a pin-type head holder. We briefly illustrate the case of a 4-year-old girl who sustained a depressed skull fracture by penetration of a pin of the head holder. The fracture was noted at the end of the surgery performed for treatment of a cerebellar astrocytoma and was managed conservatively. DISCUSSION: Several factors seem to be involved in the production of this complication as are faulty application of the pins, excessive pin pressure, skull thinning, young patient's age, and pathological conditions that evolve with long-standing raised intracranial pressure. Prevention and management measures are briefly discussed.

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.

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.

Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update.

BACKGROUND: Embryonal central nervous system (CNS) tumors are currently classified into three types: medulloblastoma, atypical rhabdoid/teratoid tumors, and primitive neuroectodermal tumor (PNET). A distinctive subtype of PNET called "embryonal tumor with abundant neuropil and true rosettes" (ETANTR) was reported in 2000. DISCUSSION: ETANTR is a recently described variety of PNET that combines microscopic features of neuroblastoma and ependymoblastoma, demonstrating areas of fine fibrillary neuropil intermingled with cellular zones and ependymoblastic rosettes. It has been suggested that this neoplasm should be considered as a separate entity. ETANTR is an eminently pediatric tumor that has been reported exclusively in children younger than 4 years. ILLUSTRATIVE CASES: A 9-month-old girl underwent subtotal resection of a brainstem neoplasm. A 23-month-old girl was submitted to surgery for a frontoparietal tumor. In both instances, the histopathological diagnosis confirmed ETANTR. Both children were treated with chemotherapy and one with radiotherapy. Death in both patients occurred after a mean period of 10 months. CONCLUSIONS: By reporting these two new instances of ETANTR, we want to contribute to the knowledge of this highly malignant CNS embryonal neoplasm that occurs only in young children, given its present lethal prognosis, the scarcity of reported cases, and the lack of treatment guidelines.

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.

Foreign body reactions causing spinal cord tethering: a case-based update.

BACKGROUND: Patients operated on for myelomeningocele (MMC) and lipomeningocele (LMC) can suffer from late functional worsening that, in many cases, is due to spinal cord tethering by the post-repair scarring process. ILLUSTRATIVE CASES: In this case-based update, we report two patients operated on for MMC and LMC, respectively, who presented the clinical manifestations of spinal cord tethering, which we attributed to severe foreign body reactions to the materials used at their primary back surgery. In the first case, the cause of the tethering was an intense fibrotic scar around the silk suture used at the initial MMC repair, while in the second one, it was due to a fibrotic mesh containing the lyophilized dural graft implanted for LMC surgery. After a search of the current literature, we were unable to find cases of spinal cord tethering related to excessive scarring after dural repair with foreign materials used for the surgical correction of spinal dysraphism. DISCUSSION AND CONCLUSIONS: The pathogenesis of spinal cord tethering in our instances closely relate with the histopathologically observed intense foreign body reaction around the materials used at the primary back surgery. We recommend avoiding as much as possible the use of foreign materials during the initial repair of spinal dysraphism.

Fibrocartilaginous mesenchymoma of the spine in a child: a case report.

BACKGROUND: Originally described by Dahlin et al. in 1984, fibrocartilaginous mesenchymoma (FCM) constitutes a rare bone tumor of children and adolescents that mainly affects the long bones. A spinal location of this tumor is exceptional. Only two previous instances of vertebral FCM have been reported in the current literature, and both occurred in young adults. REPORT OF THE CASE: We report the case of a 9-year-old boy with backache caused by a neoplasm that involved the Th12 vertebra treated with tumor excision. Histopathological diagnosis was confirmatory of FCM. During a follow-up period of 2 years, there was no evidence of disease progression. Our patient, thus, represents the first case of FCM occurring in a child. CONCLUSIONS: Given the rarity of spinal FCM, there are no guidelines about its management. However, treatment of this neoplasm seems to be mainly surgical aiming at total removal of the lesion, if feasible, as FCM may recur locally.

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.

Citrobacter koseri meningitis: a neurosurgical condition?

A 2-month-old girl developed meningitis, ventriculitis and brain abscess in the course of Citrobacter koseri infection. She was successfully treated with the combined use of antibiotics, intra-cavitary urokinase and surgery, thus avoiding the development of hydrocephalus and of ventricular loculation. C. koseri is a Gram-negative pathogen with a strong predilection for the neonatal brain. Brain abscesses develop in roughly 77% of cases, causing severe neurological sequels in one-half and death in one-third of patients. The authors aim to report the role of neurosurgical treatment for managing the severe complications that may arise in the course of C. koseri brain infection and the use of urokinase for preventing the development of loculated hydrocephalus.

Staphylococcus warneri ventriculoperitoneal shunt infection: failure of diagnosis by ventricular CSF sampling.

BACKGROUND: The definite diagnosis of hydrocephalus valve infection is generally made by cerebrospinal fluid (CSF) sampling via the valve reservoir, which is considered to be more dependable than that of the CSF obtained by lumbar puncture. CASE REPORT: We treated a 17-year-old boy with an intra-abdominal pseudocyst due to ventriculoperitoneal shunt infection caused by Staphylococcus warneri whose ventricular CSF, obtained via the valve reservoir, was repeatedly sterile thus causing a considerable delay in the management of the complication. DISCUSSION AND CONCLUSIONS: S. warneri constitutes an emergent contaminant of catheters and prostheses. We found only a detailed report of S. warneri infection of a ventriculoatrial shunt. If manifestations of peritoneal involvement in shunted patients would occur, the attention should be shifted to the distal component of the shunt hardware, even in the presence of a normal ventricular CSF as happened in our case to avoid unnecessary delay in diagnosis and management.

Cervical spinal cord infarction after posterior fossa surgery: a case-based update.

BACKGROUND: Several positions are currently utilized for operating patients with posterior fossa lesions. Each individual position has its own risks and benefits, and none has demonstrated its superiority. A dreaded, and probably underreported, complication of these procedures is cervical cord infarction with quadriplegia. DISCUSSION: We reviewed eight previous reported instances of this devastating complication aimed at ascertaining its pathogenesis to suggest preventive strategies. Several hypotheses have been put forward to explain the occurrence of this complication. Some factors involved in the production of cervical cord infarction include patient's position (seated or prone), hyperflexion of the neck, excessive spinal cord traction, canal stenosis, and systemic arterial hypotension. We hypothesize that spinal cord infarction in our patient might have resulted from compromised blood supply to the midcervical cord caused by tumor infiltration of the cervical leptomeninges in addition to a brief episode of arterial hypotension during venous air embolism. CASE REPORT: We treated an 8-year-old girl who developed quadriplegia after surgery for a fourth ventricular ependymoma. Postoperative magnetic resonance imaging demonstrated cervical cord infarction. Evoked potentials confirmed the diagnosis. CONCLUSIONS: With this report, we want to draw the attention of neurosurgeons to the possibility of the occurrence of this dreadful complication during posterior fossa procedures. Retrospectively, the only measures that might have helped to avoid this complication in our patient would have been using the prone position and intraoperative monitoring of evoked potentials.

CSF overdrainage in shunted intracranial arachnoid cysts: a series and review.

BACKGROUND: Cerebrospinal fluid (CSF) overdrainage in hydrocephalus is well recognized. Overshunting following cysto-peritoneal (CP) drainage in patients with arachnoid cysts (AC) is rarely documented. AIM: We report five patients with acquired Chiari malformation I and three with posterior fossa overcrowding due to excessive CSF drainage in shunted intracranial ACs. We review our observations and discuss the current knowledge on the pathogenesis and management of this complication. PATIENTS AND METHODS: The medical records of the eight patients were analyzed in regard to clinical manifestation, cyst and shunt characteristics, management, and outcomes. RESULTS: Mean age of the patients was 5.5 years. After an average interval of 5 years, five patients developed symptoms related to hindbrain herniation and three to severe shunt overdrainage following CP shunting. Several management modalities were utilized that achieved a good result in seven instances. DISCUSSION: Some shunted ACs may evolve with overdrainage syndromes. Posterior fossa overcrowding and tonsillar herniation constitute their most severe forms. CSF hypotension, bone changes, venous engorgement, and probably cerebral chronic edema at the posterior fossa constitute the main factors involved in the pathogenesis of this entity. We also review previous instances of acquired Chiari malformation originating after AC shunting. CONCLUSIONS: Posterior fossa overcrowding and acquired Chiari I malformation can develop after excessive CSF drainage of intracranial ACs. Overshunting manifestations require prompt recognition and management. Preventive measures consist of making a stringent selection of cases being considered for surgery, avoiding CP drainage, and placing of a programmable valve as initial treatment of intracranial ACs if shunting is considered.