Comparing Outcomes and Billing Costs of Middle Cranial Fossa and Transmastoid Approaches for Otogenic Encephalocele and Cerebrospinal Fluid Leak Repair.

OBJECTIVE: Comparison of outcomes and billing costs of patients treated at our institution using transmastoid (TM), middle cranial fossa (MCF), and combined approaches for repair of otogenic cerebrospinal fluid (CSF) leaks and encephaloceles. STUDY DESIGN: Retrospective cohort review. SETTING: Tertiary-care hospital. PATIENTS: Seventy-seven cases of otogenic CSF leaks or encephaloceles. INTERVENTIONS: Surgical repair of an otogenic encephalocele or CSF leak using either a TM, MCF, or combined approach. MAIN OUTCOME MEASURES: Success of repair, length of operation, cost of operating room materials, postoperative need for intensive care, and postoperative length of stay. RESULTS: Forty cases (52%) were performed by the TM approach, 27 (35%) by MCF, and 10 (13%) by combined TM/MCF. Mean length of stay was not statistically different amongst TM patients (2.1 d), MCF patients (3.3 d), and combined TM/MCF patients (3.70; p = 0.112). Only 3/40 TM cases required intensive care during their admission while all MCF and combined TM/MCF approach cases were admitted to the intensive care unit for at least one night ( p < 0.001). On follow-up, CSF leak recurred in 3/77 (4%) cases: 3/27 (11%) MCF, 0/40 TM, and 0/10 combined TM/MCF patients ( p = 0.056). The mean cost of operating room materials charged to the patient was significantly greater in the MCF group ($9,883) than the TM group ($3,748; p = 0.001). CONCLUSIONS: In carefully selected patients, the TM approach is an effective and less costly alternative to MCF approaches for repair of otogenic CSF leaks and encephaloceles.

Understanding the Mothers of Children with Spina Bifida and Hydrocephalus in Tanzania.

OBJECTIVE: To identify if there are cultural, medical, educational, economic, nutritional and geographic barriers to the prevention and treatment of spina bifida and hydrocephalus. METHODS: The mothers of infants with spina bifida and hydrocephalus admitted to Muhimbilli Orthopaedic Institute, Dar Es Salaam, Tanzania, between 2013 and 2014 were asked to complete a questionnaire. A total of 299 infants were identified: 65 with myelomeningoceles, 19 with encephaloceles, and 215 with isolated hydrocephalus. The questionnaire was completed by 294 of the mothers. RESULTS: There was a high variation in the geographic origin of the mothers. Approximately 85% traveled from outside of Dar Es Salaam. The mean age was 29 (15-45) years old with a parity of 3 (1-10). The rates of consanguinity, obesity, antiepileptic medication, HIV seropositivity, and family history were 2%, 13%, 0%, 2%, and 2%, respectively. A maize-based diet was found in 84%, and only 3% of woman took folic acid supplementation, despite 61% of mothers stating that they wished to conceive another baby. Unemployment was high (77%), a low level of education was common (76% not attended any school or obtaining a primary level only), and 20% were single mothers. Hospital only was the preferred method of treatment for 94% of the mothers, and 85% of the babies were born in a hospital. CONCLUSIONS: Our study highlights some of the cultural, educational, geographic, nutritional, and economic difficulties in the prevention and management of spina bifida and hydrocephalus in Tanzania.

Occipital Encephalocele: Cause, Incidence, Neuroimaging and Surgical Management.

AIMS: To review and present the current knowledge of incidence, signs and symptoms, diagnosis and treatment of the occipital encephalocele. BACKGROUND: Encephalocele (E) is a defect of the neural tube that refers to congenital malformations featured by skull defect and dura with extracranial spread of intracranial structures. Occipital encephalocele (OE) are the most common form of this congenital disorder and are manifested as a swelling of different sizes over the occipital bone in the midline. Proper diagnosis and treatment is highly important in the management of this congenital malformation of brain. OBJECTIVE: To review and present the current knowledge of incidence, signs and symptoms, diagnosis and treatment of the occipital encephalocele. METHODS: We conducted a search of case reports or case-series of patients by the use of electronic databases: Pub Med, Medline, Index Medicus, Scorpus. The key words were: encephalocele, occipital encephalocele, neural tube defect, congenital malformation. The search was updated to December 31, 2018. Papers published in English were the only source of information. RESULTS: Occipital encephalocelle are more frequent in females than in males. The incidence is between 1 in 3000 to 1 in 10,000 live births; approximately 90% of them involve the midline. Magnetic resonance imaging is the method of choice in diagnosis and surgery is the best option for the treatment of OE. Overall morbidity and mortality is still high in spite of advenced surgical management, but have been significantly improved in recent years thanks to sophisticated highresolution imaging, adequate and proper surgical treatment and decent post-operative care. CONCLUSION: Occipital encephalocele is the most common form of encephalocele. The diagnosis is mostly based by the use of neuroimaging techniques. Operation is the best option for treatment. Overall morbidity and mortality is still high, but have been significantly improved in recent years thanks to sophisticated high-resolution imaging, adequate and proper surgical treatment and decent post-operative care.

Posttraumatic refractory intracranial hypertension and brain herniation syndrome: cerebral hemodynamic assessment before decompressive craniectomy.

BACKGROUND: The pathophysiology of traumatic brain swelling remains little understood. An improved understanding of intracranial circulatory process related to brain herniation may have treatment implications. OBJECTIVE: To investigate the cerebral hemodynamic changes associated with brain herniation syndrome due to traumatic brain swelling. METHODS: Nineteen head-injured patients with evidence of refractory intracranial hypertension and transtentorial herniation were prospectively studied. Cerebral hemodynamic assessment by transcranial Doppler (TCD) ultrasonography was performed prior to decompressive craniectomy. Patients and their cerebral hemispheres were classified according to TCD-hemodynamic patterns, and the data correlated with neurological status, midline shift on CT scan, and Glasgow outcome scale scores at 6 months after injury. RESULTS: A wide variety of cerebral hemodynamic findings were observed. Ten patients (52.7%) presented with cerebral oligoemia, 3 patients (15.8%) with cerebral hyperemia, and 6 patients with nonspecific circulatory pattern. Circulatory disturbances were more frequently found in the side of maximal cerebral swelling than in the opposite side. Pulsatility index (PI) values suggested that ICP varied from acceptable to considerably high; patients with increased PI, indicating higher microvascular resistance. No correlation was found between cerebral hemodynamic findings and outcome. CONCLUSIONS: There is a marked heterogeneity of cerebral hemodynamic disturbances among patients with brain herniation syndrome.

Reliability of preoperative assessment of cerebrospinal fluid pressure in the management of spontaneous cerebrospinal fluid leaks and encephaloceles.

BACKGROUND: The association of spontaneous cerebrospinal fluid (CSF) leaks with increased intracranial pressure (ICP) is well-documented. Accurate assessment of CSF pressure is paramount to optimal long-term outcomes, as failure of surgical repair or recurrent leaks may be associated with untreated intracranial hypertension. Many surgeons utilize a single opening pressure measured at the onset of the surgical procedure to determine if long-term acetazolamide or shunt placement will be necessary. However, preoperative measurement of CSF pressure may be inaccurate secondary to active drainage. The purpose of this study is to determine the accuracy of preoperative CSF pressure measurement in the setting of active CSF rhinorrhea. METHODS: Retrospective review of 65 cases of endoscopic repair of active spontaneous CSF rhinorrhea performed at a tertiary care institution from 2002 to 2009. A total of 16 cases in which reliable preoperative opening pressure and 48-hour to 72-hour postoperative CSF pressures were recorded were included in the analysis. Cases in which measurements were potentially unreliable or in which acetazolamide therapy was used were excluded from analysis. RESULTS: The average preoperative measurement was 26 ± 11 cm H20, and the average postoperative measurement was 15 ± 6 cm H20. The average change in CSF pressure from preoperative to postoperative was -10 ± 11 cm H20. Student paired t test was used to confirm statistical difference between the 2 sets of measurements. CONCLUSION: Our results suggest that a single preoperative measurement of CSF pressure in patients with active CSF rhinorrhea may not be sufficiently reliable to make subsequent long-term clinical decisions.

Frontoethmoidal meningoencephalocoele repair in Cambodia: outcomes and cost comparisons.

In Cambodia, spina bifida is rare, but frontoethmoidal meningoencephalocoeles (MECs) are common. Mean life expectancy for patients with congenital MECs may be <20 years, but the complex treatment required has not been available in the country until recently. During visits by combined neurosurgical/craniofacial teams from both Germany and France, a method of repair has been developed that is suitable for the local conditions, affordable and has allowed Cambodian surgeons to learn how to successfully treat MECs. The surgical technique and initial results with 30 patients have been described in a previous publication. This paper presents the outcomes of 128 cases and illustrates that it is cost-effective for these patients to be treated in Cambodia.

Extra-arachnoidal cranio-cervical decompression for syringomyelia associated with Chiari I malformation in adults: technique assessment.

BACKGROUND: The osteo-dural decompression of the cerebellar tonsils at the cranio-cervical junction is generally considered the most effective treatment for syringomyelia-Chiari I complex. However much controversy concerning a great number of surgical adjuvants to the standard bony decompression is still present. In this work an extra-arachnoidal cranio-cervical decompression (CCD) without duroplasty is described and the surgical results are reported. METHOD: Between 2000 and 2005, 24 adult patients underwent surgery for symptomatic syringomyelia-Chiari I complex not associated with hydrocephalus. In all cases, the surgical procedure consisted of a limited suboccipital craniectomy and laminectomy of C1 (when necessary C2 as well) followed by dural opening leaving the arachnoid membrane intact. The dura mater is left open and stitched laterally to the muscles. FINDINGS: With a mean clinical long term follow-up of 44 months (range, 12-78 mo), neurological disturbances improved in 21 of 24 patients (87.5%) as result of extra-arachnoidal CCD. The postoperative complications occurred when the arachnoid was accidentally violated (4 cases, 16.6%). The complications included aseptic meningitis (one patient), nucal pseudomeningocele (two patients) and postoperative hydrocephalus requiring a ventriculoperitoneal shunt (one patient). Finally, one patient received an additional C2 laminectomy in order to obtain symptoms improvement and syrinx shrinkage. Postoperative MRI studies demonstrated that the syrinx decreased in size or collapsed in 20 patients (83.3%) and stabilized in 4 (16.7%). CONCLUSIONS: The extra-arachnoidal CCD is a safe and effective treatment for syringomyelia associated with Chiari I malformation in adults without intraoperative evidence of adhesive arachnoiditis. However a larger number of patients and longer follow-up will be necessary to determine the efficacy of extra-arachnoidal CCD.

Treatment of frontoethmoidal meningoencephalocele in Cambodia: a low-cost procedure for developing countries.

OBJECT: Frontoethmoidal meningoencephaloceles (MECs) are a relatively common abnormality in southeast Asia, with disastrous consequences for the sufferer. In Cambodia, a lack of skilled neuro- and craniofacial surgeons, and the cost of surgery limit the possibilities for appropriate treatment of patients with these lesions. The authors developed a low-cost humanitarian program with the goals of treating frontoethmoidal MECs, ensuring careful postoperative follow-up, and teaching Khmer surgeons how to treat these malformations. METHODS: This program was facilitated by two nongovernmental organizations: Rose Charities Cambodia provided the facilities, patients, and local staff, and "Médecins du Monde" provided visiting surgeons and anesthesiologists. All operations were free of charge for all patients. A strict follow-up program was organized to evaluate the surgical results, the social impact of the surgery, and the satisfaction levels of the children and their parents. RESULTS: Forty-five children and seven young adults with MECs were treated using a rather simple surgical technique. Of the three types of MECs encountered, the most frequent was the nasoethmoidal type (43 cases). The most common postoperative issue was a temporary CSF leak (in 16 patients). Cosmetic results were considered excellent or good in 40 patients, average in nine, and poor in one; two patients were lost to follow-up. The overall cost of each operation was estimated to be $380 (US dollars), far less than a standard MEC operation would cost in a more developed country. At the end of this humanitarian program, Khmer surgeons were able to treat standard cases of frontoethmoidal MECs without the help of foreigners. CONCLUSIONS: Patients in developing nations who have limited access to standard neurosurgical care can be treated for frontoethmoidal MECs with few complications and a satisfactory cost-to-benefit ratio.

Posterior fossa reconstruction: a surgical technique for the treatment of Chiari I malformation and Chiari I/syringomyelia complex--preliminary results and magnetic resonance imaging quantitative assessment of hindbrain migration.

Experimental models have shown that Chiari I malformation is a primary paraaxial mesodermal insufficiency occurring after the closure of the neural folds takes place. According to these hypotheses, a small posterior fossa caused by an underdeveloped occipital bone would be the primary factor in the formation of the hindbrain hernia. The main objective in the surgical treatment of Chiari I malformation and related syringomyelia is directed to restore normal cerebrospinal fluid dynamics at the craniovertebral junction. The most widely accepted surgical approach is to perform a craniovertebral decompression of the posterior fossa contents with or without a dural graft. It has been emphasized that suboccipital craniectomy should be small enough to avoid downward migration of the hindbrain into the craniectomy. This slump of the hindbrain has been verified by studies using postoperative assessment by magnetic resonance imaging. Our aim in this study is to present a modification of the conventional surgical technique, which we have called posterior fossa reconstruction (PFR). Ten patients were operated on using this technique and compared with a historical control group operated on with the classic approach of making a small suboccipital craniectomy, opening the arachnoid, and closing the dura with a graft. To evaluate the morphological results in both groups objectively, preoperative and postoperative measurements of the relative positions of the fastigium and upper pons above a basal line in the midsagittal T1-weighted magnetic resonance images were obtained. In those cases with syringomyelia, syringo-to-cord ratios were calculated. The mean age of the PFR group was 35 +/- 16 years (mean +/- SD); in the control group it was 35.2 +/- 12 years. In the PFR group, the formation of an artificial cisterna magna was observed in every case; it was observed in only one case in the control group. An upward migration of the cerebellum was seen in all cases in the PFR group, with a mean ascent of the fastigium of 6.2 mm. A significant downward migration of the cerebellum was observed in seven cases in the control group. No significant differences were found in both groups when comparing syringo-to-cord ratios. This leads us to conclude that PFR is more effective than conventional surgical approaches in restoring the normal morphology of the craniovertebral junction. This allows cranial ascent of the hindbrain verified by magnetic resonance imaging and good short-term clinical results. Because PFR is mainly an extraarachnoidal approach, complications related to surgery using this technique can be kept to a minimum.

[The value of CT and MRI in the assessment of basal encephaloceles in children]. / Apport du CT et de l'IRM dans l'évaluation des céphalocèles basales de l'enfant.

Basal cephaloceles of the child are rare pathologies which require accurate preoperative imaging work-up. The CT and MR studies of six children with surgically proven basal cephalocele were retrospectively reviewed to evaluate the role of CT and MR in the preoperative work-up of a basal cephalocele of the child. In five patients, MR allowed to define the nature and topography of the cephalocele, and allowed an accurate depiction of the optic tract, ante- and post-hypophysis and associated agenesis of corpus callosum when present. 3-D CT allowed in one case a more precise depiction of the basal bony defect. MRI allows in a non invasive and non ionising way the best depiction of herniating meninges, brain or ventricles as well as associated cerebral anomalies.

Hindbrain migration after decompression for hindbrain hernia: a quantitative assessment using MRI.

Pre- and post-operative assessments are presented in 17 adult patients who have been treated with craniovertebral decompression for hindbrain herniation, 11 of whom had syringomyelia. Objective improvement in the size of the syrinx was seen in all 11 cases; contrary to expectation the hindbrain more frequently moved downwards than upwards after decompression of the tonsils and creation of an artificial cisterna magna. A method is reported for the quantitative assessment of hindbrain migration using magnetic resonance imaging (MRI). The degree of 'slump', further downward displacement of the hindbrain, was compared with the clinical outcome and the MRI appearances. Slump was more common than expected, although the severity was not usually great enough to produce symptoms. Slump was less marked where the artificial cisterna magna was generous (p less than 0.02). This quantitative method may be useful in assessing patients with unexplained post-operative symptoms and in comparing different surgical techniques.