Post-operative seizure after first time endoscopic third ventriculostomy in pediatric patients.

PURPOSE: Post-operative seizure rates after endoscopic third ventriculostomy (ETV) are not definitively known. We analyzed our institution's experience for all causes of hydrocephalus in pediatric patients undergoing ETV to determine rates of post-ETV seizure. METHODS: A retrospective review of institutional pediatric patients undergoing ETV from May 2014 to December 2018. Included were < 21 years, with 1-year follow-up. Exclusion criteria included ventriculoperitoneal shunts (VPS) prior to ETV, VPS within 7 days post-ETV, and prior seizure disorder. Data included age, gender, diagnosis, early post-operative seizure (within 7 days post-ETV), late post-operative seizures (after first 7 days and within first year post-ETV), concomitant choroid plexus cauterization (CPC), VPS conversion within 1 year, and administration of prophylactic antiepileptics. RESULTS: Sixty of 81 ETV cases were included; 41% underwent concomitant CPC. Of these, 53% (n = 32) were male, 46% (n = 28) female, averaging 5.8 years, with the most common diagnosis neoplasm-related obstructive hydrocephalus (38.3%, n = 23). Early post-operative seizure occurred in 6.7% (n = 4); late post-operative seizure occurred in 8.3% (n = 5). Late post-operative seizures were higher in patients experiencing early post-operative seizure versus those without (75% vs 3.7%, p = 0.003). Late post-operative seizure occurred in 13.6% (n = 3 patients) requiring VPS versus 5.3% (n = 2 patients) with successful ETV (p = 0.36). Rates did not correlate with pathology. No patients received prophylactic antiepileptics prior to surgery or exhibiting a seizure. CONCLUSIONS: Patients with early post-operative seizures have an increased likelihood of developing late post-operative seizures. Pediatric ETV patients may have a lower rate of both early and late post-operative seizure; underlying pathology may influence these rates.

The cause of infant and toddler subdural hemorrhage: a prospective study.

OBJECTIVE: To determine the frequency of child abuse and unintentional injury as a cause of infant and toddler subdural hemorrhage (SDH). METHODS: A prospective case series of a level I regional trauma center, regional children's hospital, and county medical examiner's office assessed consecutive children who were

Abnormal coagulation during pediatric craniofacial surgery.

INTRODUCTION: This prospective study of children undergoing major craniofacial surgery was undertaken to determine whether abnormal hemostasis occurred and to characterize any coagulopathy found. METHODS: Coagulation tests, blood loss and blood product transfusions were recorded perioperatively. Packed red blood cells (PRBC) were transfused to maintain target hematocrit. Patients with blood loss >100 ml/kg (group A, n = 5) were compared to patients with blood loss <100 ml/kg (group B, n = 22) using Mann-Whitney U test (p < 0.05). RESULTS: Twenty-seven children (age range 2.9--27.9 months) had median total blood loss of 64 ml/kg. At completion of surgery, median coagulation values differed significantly between groups for prothrombin time (A: 16.6 s; B: 13.8 s), partial thromboplastin time (A: 44 s; B: 29 s), thrombin time (A: 28 s; B: 23 s), thromboelastograph reaction time (A: 7 mm; B: 4 mm), prothrombin fragment F1.2 (A: 1.9 nmol/l; B: 3.3 nmol/l) and platelet count (A: 174 K/mm(-3); B: 239 K/mm(-3)). Fibrinolysis was not associated with blood loss. Median units transfused were in group A 3 units and group B 1 unit (p = 0.001). All patients received PRBC transfusions but only group A patients received other blood products (fresh frozen plasma, platelets). CONCLUSION: Children transfused with PRBC during craniosynostosis repair can become coagulopathic from coagulation factor depletion when hemorrhage approaches 1.5 times estimated blood volume.

Techniques for cerebral bypass: practical laboratory for microvascular anastomosis.

The techniques used for microvascular anastomosis are best mastered in the laboratory setting. A variety of techniques for end-to-end and end-to-side anastomoses are described and illustrated. An understanding of these different modalities allows the surgeon to choose the appropriate technique for each clinical setting.

Spontaneous cerebellar hemorrhage due to a juvenile pilocytic astrocytoma: case report and review of the literature.

A 13-year-old boy presented to the emergency room with headaches and ataxia. Imaging studies revealed a cerebellar hemorrhage within a posterior fossa tumor. The patient underwent complete resection of this lesion and made a full recovery. Microscopic examination of this lesion revealed a juvenile pilocytic astrocytoma.

O6-Methylguanine-DNA methyltransferase in pediatric primary brain tumors: relation to patient and tumor characteristics.

The DNA repair protein O6-methylguanine-DNA methyltransferase (MGMT) confers resistance to methylating and chloroethylating agents in pediatric medulloblastoma- and glioma-derived cell lines and xenografts. Here, we assayed MGMT activity in 110 pediatric brain tumors to establish correlates with patient and tumor characteristics. We also assayed MGMT in histologically normal brain adjacent to 22 tumors to characterize changes in activity accompanying neurocarcinogenesis. MGMT activity was detected in 94% of tumors, ranging ca. 1,500-fold from 0.34 to 498 fmol/10(6) cells (approximately 205-300,000 molecules/cell). Mean activity was 25 +/- 66 fmol/10(6) cells, including six specimens with undetectable activity (Mer- phenotype; <0.25 fmol/10(6) cells or 151 molecules/cell). MGMT content varied 10-fold among diagnostic groups and was associated with degree of malignancy, as evidenced by a 4-fold difference in activity between high- and low-grade tumors (P = 0.03). Tumor MGMT content was age dependent, being 5-fold higher in children 3-12 years old than in infants (P = 0.015) and adolescents (P = 0.015). Mean activity in tumors was 9-fold higher than in adjacent histologically normal brain (21 +/- 44 versus 2.4 +/- 4.0 fmol/10(6) cells; P = 0.05). By comparing tumor and adjacent normal tissue from the same patient, we found that 68% of cases exhibited an elevation of tumor activity that ranged from 2- to >590-fold. Moreover, 67% of Mer- normal tissue was accompanied by Mer+ tumor. These observations indicate that MGMT activity is frequently elevated during pediatric neurocarcinogenesis. Significantly, enhanced MGMT activity may heighten resistance to alkylating agents, suggesting a potential role for MGMT inhibitors in therapy.

Inhibition of apoptosis: a potential mechanism for syndromic craniosynostosis.

The biologic pathogenesis of syndromic craniosynostosis remains unknown. The purpose of this investigation was to determine whether specific biologic differences exist between normal calvarial osteoblasts and osteoblasts derived from patients with syndromic craniosynostosis. This study (1) examined the apoptotic rate and cell cycle of osteoblasts derived from patients with syndromic craniosynostosis, and (2) investigated for the presence of soluble factors released from syndrome-derived osteoblasts. Osteoblast cell lines were established from calvarial specimens of patients with clinically diagnosed syndromic synostosis and from normal controls. A co-culture technique was used to investigate for the presence of elaborated soluble factors. Apoptotic rate and cell cycle analyses were performed by using flow cytometry after staining with annexin V-fluorescein isothiocyanate and propidiumiodide, respectively. The apoptotic rate was significantly reduced in syndrome-derived osteoblasts as compared with control osteoblasts. Control osteoblasts co-cultured with syndromic osteoblasts demonstrated a dramatic reduction in their apoptotic rate as compared with those co-cultured with control osteoblasts. These results indicate that osteoblasts derived from patients with syndromic craniosynostosis display a lower apoptotic rate, a normal DNA synthetic rate, and the capability to reduce the apoptotic rate in normal calvarial osteoblasts through the elaboration of soluble factors.

Transcortical surgery for lateral ventricular tumors.

OBJECT: Tumors of the lateral ventricle can be removed via two major approaches: the transcallosal or the transcortical route. The purpose of this study is to discuss the techniques and outcomes in transcortical surgery of tumors located in the lateral ventricle. METHODS: An experience with 29 consecutive lateral ventricular tumors resected via the transcortical route, over a 5-year period, is presented. The risks, complications, and outcomes of this surgical series, as well as those reported in the literature, are discussed. Surgical approaches to all five regions of the lateral ventricle are described. Neuropsychological, functional, and neurological outcomes are evaluated. CONCLUSIONS: The transcortical technique makes it possible to resect lesions in each of the five regions of the lateral ventricle. It provides superior microsurgical working space and flexibility for maneuvering within the lateral ventricle. The key to a successful transcortical approach is an understanding of the functional anatomy of eloquent cortex to be broached, the location of the lesion, and its vascular supply. A clear understanding of the advantages and limitations of the transcortical approach makes performing this procedure for resection of large lesions in the ventricle both safe and effective. The majority of the patients in this series (86%) had a good outcome, returning to baseline functional status and suffering minimal morbidity. In the microsurgical era, transcortical surgery-related postoperative morbidity and outcome are dependent more on tumor histological type and site of origin than on approach.

Atypical hemifacial microsomia associated with Chiari I malformation and syrinx: further evidence indicating that chiari I malformation is a disorder of the paraaxial mesoderm. Case report and review of the literature.

The authors present the first known reported case of hemifacial microsomia associated with a Chiari I malformation and syrinx. A 14-year-old girl presented with progressive torticollis of 3 years' duration and headaches exacerbated by exercise. Computerized tomography scanning and magnetic resonance imaging revealed extensive craniofacial and vertebral abnormalities, including aplasia of the floor of the left middle fossa and posterior fossa cranium, articulation of the left mandibular condyle with the left temporal lobe, and progressive development of a Chiari I malformation with associated syringomyelia. The patient first underwent posterior fossa decompression, duraplasty, and occipitocervical fusion. This procedure was later followed by reconstruction of the floor of the left middle fossa and temporomandibular joint. The patient's outcome was excellent. In this case report the authors review the complex embryological development of craniofacial and craniovertebral structures, and emphasize the use of a staged approach to treat pathophysiological consequences of this congenital anomaly.

Cognitive rehabilitation for traumatic brain injury: A randomized trial. Defense and Veterans Head Injury Program (DVHIP) Study Group.

CONTEXT: Traumatic brain injury (TBI) is a principal cause of death and disability in young adults. Rehabilitation for TBI has not received the same level of scientific scrutiny for efficacy and cost-efficiency that is expected in other medical fields. OBJECTIVE: To evaluate the efficacy of inpatient cognitive rehabilitation for patients with TBI. DESIGN AND SETTING: Single-center, parallel-group, randomized trial conducted from January 1992 through February 1997 at a US military medical referral center. PATIENTS: One hundred twenty active-duty military personnel who had sustained a moderate-to-severe closed head injury, manifested by a Glasgow Coma Scale score of 13 or less, or posttraumatic amnesia lasting at least 24 hours, or focal cerebral contusion or hemorrhage on computed tomography or magnetic resonance imaging. INTERVENTIONS: Patients were randomly assigned to an intensive, standardized, 8-week, in-hospital cognitive rehabilitation program (n=67) or a limited home rehabilitation program with weekly telephone support from a psychiatric nurse (n=53). MAIN OUTCOME MEASURES: Return to gainful employment and fitness for military duty at 1-year follow-up, compared by intervention group. RESULTS: At 1-year follow-up, there was no significant difference between patients who had received the intensive in-hospital cognitive rehabilitation program vs the limited home rehabilitation program in return to employment (90% vs 94%, respectively; P=.51; difference, 4% [95% confidence interval ¿CI¿, -5% to 14%]) or fitness for duty (73% vs 66%, respectively; P=. 43; difference, 7% [95% CI, -10% to 24%]). There also were no significant differences in cognitive, behavioral, or quality-of-life measures. In a post-hoc subset analysis of patients who were unconscious for more than 1 hour (n = 75) following TBI, the in-hospital group had a greater return-to-duty rate (80% vs 58%; P=. 05). CONCLUSIONS: In this study, the overall benefit of in-hospital cognitive rehabilitation for patients with moderate-to-severe TBI was similar to that of home rehabilitation. These findings emphasize the importance of conducting randomized trials to evaluate TBI rehabilitation interventions. JAMA. 2000;283:3075-3081

Occult intraspinal anomalies in congenital scoliosis.

Thirty consecutive patients with congenital spinal deformity underwent magnetic resonance imaging (MRI) to determine the incidence of occult intraspinal anomaly. These congenital spinal deformities included 29 cases of congenital scoliosis and one case of congenital kyphosis. Physical examination findings and plain radiographs were reviewed in an attempt to correlate these findings with subsequent intraspinal pathology. Nine patients had intraspinal anomalies identified on MRI consisting of five with tethered cord, four with syringomyelia, three with lipoma, and one with diastematomyelia. One patient required surgery for diastematomyelia; another underwent release of his tethered cord. Only one patient, with diastematomyelia associated with a syrinx and bifocal tethering, had his anomaly suggested by physical examination and plain radiographs. Two other patients had findings on plain radiographs previously associated with high prevalence of occult intraspinal anomalies; one patient with congenital kyphosis had a tethered cord, and one patient with a unilateral hemivertebrae associated with a contralateral bar had a tethered cord. Two of nine patients with occult intraspinal anomalies required surgery for their anomaly. In patients with a congenital spinal deformity, we found nine (30%) of 30 to have an associated anomaly within the spinal canal. Only three of these nine had plain radiographs and physical examination findings suggestive of their subsequent MRI findings. Given the poor correlation between findings on physical examination, plain radiographs, and subsequent occult intraspinal anomalies on MRI, we believe that MRI is helpful in evaluating patients with congenital spinal anomalies.

Update on craniofacial surgery: the differential diagnosis of lambdoid synostosis/posterior plagiocephaly.

There has been an evolution of thought on the diagnosis and treatment of posterior plagiocephaly. Synostotic posterior plagiocephaly (lambdoid synostosis) can be diagnosed and differentiated from non-synostotic posterior plagiocephaly (positional molding) based on specific cosmetic and radiologic criteria. The advent of high-resolution three-dimensional CT analysis of cranial morphology combined with meticulous clinical studies of the two major causes of posterior plagiocephaly has added much to our understanding of this skull deformity. Children with lambdoid synostosis have a trapezoid-shaped skull with posterior displacement of their ipsilateral ear, ipsilateral mastoid bossing, contralateral occipital bossing, and a fused lambdoid suture that appears as a ridge. Children with posterior plagiocephaly without lambdoid synostosis have a characteristic parallelogram-shaped skull with anterior displacement of the ipsilateral ear and ipsilateral frontal bossing. This subject is surrounded in controversy because many of the children in the past thought to have lambdoid synostosis probably did not, based in part on the aforementioned specific criteria. This is an important point, as most patients with posterior plagiocephaly without synostosis will improve without surgery. One should thus be appropriately conservative in the selection of patients for surgery. The majority of infants evaluated at craniofacial clinics presumably have posterior plagiocephaly without synostosis and can be successfully treated with frequent head turning, helmet, or band therapy. Patients with clinically and radiologically proven synostotic posterior plagiocephaly and a severe deformity should undergo craniofacial surgery. The technique of biparieto-occipital craniotomy is safe, simple, and delivers a good cosmetic result. Our postoperative photographic evaluations have shown an immediate, aesthetically pleasing change in the contour of the occiput, which tends to improve with time.

Toward a rational treatment of Chiari I malformation and syringomyelia.

In patients with Chiari I malformation with and without associated syringomyelia, aberrant cerebrospinal fluid (CSF) dynamics and a spectrum of posterior fossa pathological findings are demonstrated. In this study, the authors test the validity of using prospective cardiac-gated phase-contrast cine-mode magnetic resonance (MR) imaging to define the malformation, delineate its pathophysiology, and assist in implementing a rational treatment plan. Eighty-five cases were prospectively analyzed using cine MR imaging. Sixty-five patients, adults and children, with symptomatic Chiari malformation, with and without syringomyelia, were surgically treated from 1990 to 1999. All patients underwent pre- and postoperative cine MR evaluation. Ten patients were treated after a previous surgical procedure had failed. To establish CSF flow characteristics and normative CSF profiles, 20 healthy volunteers were examined. Compared with normal volunteers, in Chiari I malformation patients with and without syringomyelia, uniformly abnormal craniocervical junction CSF flow profiles were revealed. After intradural exploration, nearly all patients with Chiari I malformation experienced clinical improvement and CSF flow profiles, paralleling those of normal volunteers, were shown. In all patients in whom treatment had failed, abnormal preoperative CSF flow profiles, which correlated with suspected physiological abnormalities and the pathological findings noted at reoperation, were demonstrated. Symptomatic Chiari I malformation is a dynamic process characterized by the impaction of the hindbrain in an abnormal posterior fossa. This compression obstructs the normal venting of CSF in and out of the craniocervical subarachnoid space, throughout the cardiac cycle. Therefore, decompression or enlargement of the posterior fossa to establish normal CSF pathways should be the primary goal of surgical intervention. Aberrant CSF flow appears to be only one aspect of the pathological condition found in patients with Chiari I malformation. Arachnoid scarring in the posterior fossa and selective vulnerability of the spinal cord may also be factors in the pathogenesis and maintenance of associated syringomyelia. Phase-contrast cine MR imaging is a useful tool in defining physiological and anatomical problems in patients with Chiari I and syringomyelia, and it can help guide an appropriate primary or salvage surgical therapy.

Lessons learnt from the management of 1500 complex facial fractures.

The lessons learnt from the management of 1500 consecutive patients with complex facial fractures have been analysed. This analysis and the major changes in treatment principles have resulted in dramatic improvement in results. The application of craniofacial surgical principles, extended exposure of the craniofacial skeleton, accurate fracture reduction with rigid internal bony fixation and primary bone graft reconstruction has revolutionised the care of these patients. The reattachment of the soft tissue to the reconstructed skeleton provides the final link in this comprehensive one stage reconstruction. Analysis of results has demonstrated the majority of complications and failures to be due to inadequate exposure and reduction of fractures with bone segments rigidly plated in their unreduced position. Inadequate bone grafting and failure to re-suspend the soft tissue and canthi results in post treatment deformity even if the bony reconstruction is adequate. Adherence to the principles of reconstruction will almost always result in a one-stage correction no matter how severe the initial injury.

State of the art in craniofacial surgery: nonsyndromic craniosynostosis.

Craniosynostosis refers to the premature fusion of one of the six major sutures of the cranial vault. Functionally, craniosynostosis may be defined as the premature conversion of the dynamic region of growth and resorption between two adjacent bones of the cranium into a static region of bony union. Molecular analysis has blurred the traditional categories of nonsyndromic and syndromic synostosis to some extent, but, in general, the distinctions between the two groups still hold true. The complexity of the congenital anomalies may be limited with the former, whereas the latter usually requires reoperations and correction of the facial skeleton. This article briefly outlines the characteristic deformities produced from nonsyndromic craniosynostosis. Various approaches to surgical correction of the deformities are described. Finally, new biomaterials that are used in the correction of nonsyndromic craniosynostosis are reviewed.

Cerebral abscess after presumed superficial periorbital wound.

Penetrating wounds in the periorbital region may appear superficial and minor at first glance. The unique shape and thin bony roof of the orbit give these injuries a significant risk of associated intracranial penetration. This can initially be asymptomatic, and a high index of suspicion is essential to properly diagnose and treat these injuries. We report a case of an 8-year-old female who presented with delayed seizures from a frontal abscess resulting from such an injury. This article reviews the literature and discusses the appropriate management that should be used by emergency room and military physicians.

Spontaneous resolution of a cervicothoracic syrinx in a child. Case report and review of the literature.

A child with near complete spontaneous resolution of a cervicothoracic syrinx and improvement in a Chiari type I malformation without surgical intervention is presented. The child was followed clinically with serial magnetic resonance (MR) imaging and has remained neurologically stable over an 11-year period. To our knowledge, only 3 pediatric cases of spontaneous resolution of a spinal cord syrinx as documented by MR imaging without surgical intervention have been reported. This case contributes to the literature on the natural history of syringes.

19-year follow-up of fetal myelomeningocele brought to term.

We conducted a 19-year follow-up study of 223 infants with myelomeningocele born by cephalic presentation, 68 born by pre-rupture of amniotic membranes cesarean section (PRAM C/S) and 155 born vaginally to determine outcome. We recorded radiographic level as the last intact vertebral arch at the cephalad end of the spinal defect from standard AP films of the spine. Protrusions of the lesions dorsal to the back were obtained from medical records or parental recall. Subsequent spinal cord abnormalities (hydromyelia, tethered cord and hypoplasia) were determined by patient's symptoms and verified by MRI. Data were from our Patient Data Management System and analyzed using Microsoft Excel and Epi Info 6. Flat lesions protruding less than 1.0 cm beyond the plane of the back and those associated with breech position or kyphus were not benefited by PRAM CIS (Fisher's Exact, p = 0.58 between the two types of delivery for motor level). Flat lesions were more common in the vaginally delivered group (p = 0.01). Lesions protruding equal to 1.0 cm or beyond were associated with less paralysis after PRAM C/S (p = 0.01). Although tethered cord syndrome was more common in the PRAM C/S group (p = 0.02), there was no difference in the muscle strength loss between the PRAM C/S and the vaginally delivered groups. Symptomatic hydromyelia was equally distributed but spinal cord hypoplasia was more common in the PRAM C/S group (p = 0.03). Due to the limited numbers of patients in each group available for analysis, we recommend further study to ascertain the appropriate management of fetal myelomeningocele diagnosed in utero and brought to term.