Multiple secondary cauda equina non-Hodgkin's lymphoma: a case report and literature review.

BACKGROUND: Secondary central nervous system involvement of non-Hodgkin's lymphoma (NHL) is rare and with poor prognosis, the most common pathological type is diffuse large B cell lymphoma (DLBCL). Although it can occur in any part of central nervous system, it rarely directly infiltrates the spinal cord or cauda equina. CASE PRESENTATION: We present the case of 64-year-old immunocompetent man with a worsening pain of waist and left lower extremity, accompanied by numbness and paresis of bilateral lower extremity for 20 days. His previous medical history included a resection of painless mass in the left groin in another hospital 7 months ago, and the pathological diagnosis was non-Hodgkin small B cell lymphoma. Gd-enhanced MRI and F-18 FDG PET-CT scan demonstrated multiple infiltrations in the cauda equina. During the operation, we removed as many as 11 subdural-extramedullary bean-size lesions involving multiple nerve roots. The paralysis of his left leg recovered rapidly after the operation. During the follow-up period of more than one year, he underwent standard R-CHOP chemical therapy, no evidence of recurrence was noted until the 13th month, the patient died because of intracranial relapse. CONCLUSIONS: Imaging examination is important in the diagnosis of multiple secondary cauda equina non-Hodgkin's lymphoma, and we highlight the significance of gadolinium-enhanced MRI and F-18 FDG-PET/CT in preoperative diagnosis as well as the previous history.

Herniation despite Decompressive Hemicraniectomy in Large Hemispherical Ischemic Strokes.

BACKGROUND: Despite decompressive hemicraniectomy (DHC), progressive herniation resulting in death has been reported following middle cerebral artery (MCA) strokes. We aimed to determine the surgical parameters measured on brain computed tomography (CT) scan that are associated with progressive herniation despite DHC in large MCA strokes. METHODS: Retrospective chart review of medical records of patients with malignant hemispheric infarction who underwent DHC for cerebral edema was performed. Infarct volume was calculated on CT scans obtained within 24 hours of ictus. Radiological parameters of craniectomy bone flap size, brain volume protruding out of the skull, adequate centering of the craniectomy over the stroke bed, and the infarct volume outside the craniectomy bed (volume not centered [VNC]) were measured on the postoperative brain CT. RESULTS: Of 41 patients who underwent DHC, 7 had progressive herniation leading to death. Radiographic parameters significantly associated with progressive herniation included insufficient centering of craniectomy bed on the stroke bed (P = .03), VNC (P = .011), additional anterior cerebral artery infarction (P = .047), and smaller craniectomy length (P = .05). Multivariate logistic regression analysis for progressive herniation using craniectomy length and VNC as independent variables demonstrated that a higher VNC was significantly associated with progressive herniation despite surgery (P = .029). CONCLUSIONS: In large MCA strokes, identification of large infarct volume outside the craniectomy bed was associated with progressive herniation despite surgery. These results will need to be verified in larger prospective studies.

A Retrospective Analysis of Neonatal Encephalocele Predisposing Factors and Outcomes.

OBJECTIVE: This study evaluates the predisposing factors and outcomes of surgical management of encephaloceles at our institution. MATERIALS AND METHODS: A retrospective analysis of 32 occipital encephaloceles managed operatively at the Neurosurgery Department Clinics of the Faculty of Medicine, Adiyaman University, was performed between 2011 and 2015. RESULTS: Among the study population, 19 mothers had been exposed to TORCH infections (toxoplasma, rubella, cytomegalovirus, herpes simplex virus), 18 were in consanguineous marriages, and 3 had regular prenatal screening. Associated congenital anomalies were common. Eight infants required reoperation, and 9 died during follow-up. CONCLUSIONS: The study identified key areas for prevention. Knowledge of the intracranial and associated anomalies can guide management.

Large Size Hemicraniectomy Reduces Early Herniation in Malignant Middle Cerebral Artery Infarction.

BACKGROUND: Decompressive hemicraniectomy (DHC) reduces mortality and improves outcome after malignant middle cerebral artery infarction (MMI) but early in-hospital mortality remains high between 22 and 33%. Possibly, this circumstance is driven by cerebral herniation due to space-occupying brain swelling despite decompressive surgery. As the size of the removed bone flap may vary considerably between surgeons, a size too small could foster herniation. Here, we investigated the effect of the additional volume created by an extended DHC (eDHC) on early in-hospital mortality in patients suffering from MMI. METHODS: We performed a retrospective single-center cohort study of 97 patients with MMI that were treated either with eDHC (n = 40) or standard DHC (sDHC; n = 57) between January 2006 and June 2012. The primary study end point was defined as in-hospital mortality due to transtentorial herniation. RESULTS: In-hospital mortality due to transtentorial herniation was significantly lower after eDHC (0 vs. 11%; p = 0.04), which was paralleled by a significantly larger volume of the craniectomy (p < 0.001) and less cerebral swelling (eDHC 21% vs. sDHC 25%; p = 0.03). No statistically significant differences were found in surgical or non-surgical complications and postoperative intensive care treatment. CONCLUSION: Despite a more aggressive surgical approach, eDHC may reduce early in-hospital mortality and limit transtentorial herniation. Prospective studies are warranted to confirm our results and assess general safety of eDHC.

Shunting in acute cerebral venous thrombosis: a systematic review.

BACKGROUND AND PURPOSE: The efficacy of cerebrospinal fluid shunting to reduce intracranial hypertension and prevent fatal brain herniation in acute cerebral venous thrombosis (CVT) is unknown. METHOD: From the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT) and a systematic literature review, we retrieved acute CVT patients treated only with shunting (external ventricular drain, ventriculoperitoneal or ventriculojugular shunt). Outcome was classified at 6 months and final follow-up by the modified Rankin Scale (mRS). RESULTS: 15 patients were collected (9 from the ISCVT and 6 from the review) who were treated with a shunt (external ventricular drain in 6 patients, a ventriculoperitoneal shunt in 8 patients or an unspecified type of shunt in another one). Eight patients (53.3%) regained independence (mRS 0-2), while 2 patients (13.3%) were left with a severe handicap (mRS 4-6) and 4 (26.7%) died despite treatment. Five patients with parenchymal lesions were shunted within 48 h from admission deterioration, 4 with an external ventricular drain: 2 (40%) recovered to independence, 2 (40%) had a severe handicap and 1 (20%) died. In contrast, all 3 patients with intracranial hypertension and no parenchymal lesions receiving a ventriculoperitoneal shunt later than 48 h regained independence. CONCLUSION AND IMPLICATIONS: A quarter of acute CVT patients treated with a shunt died, and only half regained independence. With the limitation of the small number of subjects, this review suggests that shunting does not appear to be effective in preventing death from brain herniation in acute CVT. We cannot exclude that shunting may benefit patients with sustained intracranial hypertension and no parenchymal lesions.

Decompressive craniectomy for encephalitis with brain herniation: case report and review of the literature.

BACKGROUND: Decompressive craniectomy (DC) has been sporadically used in cases of infectious encephalitis with brain herniation. Like for other indications of DC, evidence is lacking regarding the beneficial or detrimental effects for this pathology. METHODS: We reviewed all the cases of viral and bacterial encephalitis treated with decompressive craniectomy reported in the literature. We also present one case from our institution. These data were analyzed to determine the relation between clinical and epidemiological variables and outcome in surgically treated patients. RESULTS: Of 48 patients, 39 (81.25 %) had a favorable functional recovery and 9 (18.75 %) had a negative course. Only two patients (4 %) died after surgical treatment. A statistically significant association was found between diagnosis (viral and bacterial encephalitis) and outcome (GOS) in surgically treated patients. Viral encephalitis, usually caused by herpes simplex virus (HSV), has a more favorable outcome (92.3 % with GOS 4 or 5) than bacterial encephalitis (56.2 % with GOS 4 or 5). CONCLUSIONS: Based on this literature review, we consider that, due to the specific characteristics of infectious encephalitis, especially in case of viral infection, decompressive craniectomy is probably an effective treatment when brain stem compression threatens the course of the disease. In patients with viral encephalitis, better prognosis can be expected when surgical decompression is used than when only medical treatment is provided.

Twenty-five-year survival of children with birth defects in New York State: a population-based study.

BACKGROUND: Few studies have been conducted on long-term survival of children with major birth defects because of a lack of longitudinal birth defects surveillance data. The objective of this study was to conduct a 25-year survival analysis among children in New York born with major defects by survival age, birth defect category, and other possible contributing factors. METHODS: A cohort was constructed containing children born in 1983 to 2006 with selected major birth defects. Deaths among the study cohort were identified by matching the children to their death certificates. The survival probability was estimated by Kaplan-Meier methods. Cox proportional hazards regression was used to examine the effect of the risk factors on survival. RESULTS: A total of 9112 deaths were identified among 57,002 live births with selected birth defects between 1983 and 2006. The overall 25-year survival probability of the study cohort was 82.51% (95% confidence interval, 82.11-82.89%). The estimated survival probability was comparable to that reported from previous studies regarding individual defects including spina bifida, encephalocele, atrioventricular septal defects, tracheoesophageal fistula and esophageal atresia or stenosis, renal agenesis or dysgenesis, lower limb reduction, diaphragmatic hernia, abdominal wall defects, and Down syndrome. Sex, low birth weight for gestational age, existence of multiple birth defects (nonisolated), and maternal age and nativity were identified as risk factors. CONCLUSION: Using the statewide, population-based birth defects surveillance data in New York State, the survival experience of the study cohort was examined across all survival time periods by individual birth defect of interest. Several risk factors that affect survival were identified.

[Impact of the fortification of food with folic acid on neural tube defects in Costa Rica]. / Impacto de la fortificación de alimentos con ácido fólico en los defectos del tubo neural en Costa Rica.

OBJECTIVE: Evaluate the impact of the fortification of food with folic acid on prevalence trends for neural tube defects (NTD) and the infant mortality rate (IMR) associated with this disorder in Costa Rica. METHODS: The surveillance data from the Congenital Disease Registry Center and the Central American Population Center were analyzed. The neural tube defects considered were anencephaly, spina bifida, and encephalocele. The trends from 1987-2009, as well as the differences in prevalence and mortality rates prior to and up to 12 years after food fortification with folic acid, were examined (95% confidence interval [CI]). The contribution of fortification to the decrease in the overall IMR was determined. RESULTS: During 1987-1997, prior to the period of food fortification with folic acid, NTD prevalence was 12/10 000 births (95% CI: 11.1-12.8), whereas in 2009 prevalence was 5.1/10 000 births (3.3-6.5). The IMR associated with NTD was 0.64/1 000 births (46-0.82) in 1997 and 0.19/1 000 births (0.09-9.3) in 2009. There were significant decreases in the IMR associated with NTD and the prevalence of NTD: 71%, and 58%, respectively (P < 0.05). The overall IMR decreased from 14.2/1 000 births in 1997 to 8.84/1 000 births in 2009 (P < 0.05). The decrease in the IMR associated with NTD contributed to an 8.8% decrease in the overall IMR from 1997 to 2009. CONCLUSIONS: Food fortification with folic acid caused a decrease in NTD at birth and the IMR associated with this malformation during the 1997-2009 period. It also led to a decrease in the overall IMR. There is a temporal relationship between the introduction of fortification policies and the decrease in prevalence and mortality associated with NTD. This intervention should be promoted in Latin American and Caribbean countries where it has not yet been implemented.

Prognostic factors in patients with occipital encephalocele.

BACKGROUND: An encephalocele is a herniation of the brain and the meninges through a skull defect protruding towards the exterior. The condition is not rare when compared to spinal dysraphisms, but the worldwide incidence is not precisely known. The cases involving occipital encephaloceles which we have diagnosed in our clinic and the surgical approaches for this rare condition are presented herein. METHODS: Thirty patients who were diagnosed with occipital encephaloceles and referred to our Neurosurgery Clinic at the Yuzuncu Yil University, Faculty of Medicine Research Hospital between 2000 and 2009 were enrolled in this study. The age of the patient, size of the sac, pathologies that accompanied the condition, and treatments applied were assessed. RESULTS: In the present study, 30 patients (22 girls and 8 boys), whose ages varied between newborn and 14 months, were evaluated. The encephalocele sac was located in the occipital region in 27 patients (90%) and in the occipitocervical region in 3 patients (3%). Nine (30%) of the 30 patients died; 2 in the preoperative period, 2 in the postoperative early period (0-7 days) and 5 in the late postoperative period (first week to 3 months). With the exception of the 2 patients who died preoperatively, surgery was performed on all of the patients. The mortality rate in our study was 29%. CONCLUSIONS: Our study demonstrated that factors which determine the prognosis of patients diagnosed with occipital encephaloceles include the size of the sac, the contents of the neural tissue, hydrocephaly, infections, and pathologies that accompany the condition. An occipital encephalocele is a congenital neurologic condition with an extremely high morbidity and mortality in spite of the treatments rendered pre- and postoperatively.

Second-look strokectomy of cerebral infarction areas in patients with severe herniation.

OBJECTIVE: Decompressive craniectomies (DCs) are performed on patients suffering large cerebral infarctions. The efficacy of this procedure has been demonstrated in several trials. In some cases, however, this procedure alone is not sufficient and patients still suffer refractory elevations of intracranial pressure (ICP). The goal of this study was to determine whether resection of infarcted tissue, termed strokectomy, performed as a second-look procedure after DC, improves outcome in selected cases. METHODS: The authors retrospectively evaluated data of patients who underwent a DC due to a cerebral infarction at their institution from 2009 to 2016, including patients who underwent a strokectomy procedure after DC. Clinical records, imaging data, outcome scores, and neurological symptoms were analyzed, and clinical outcomes and mortality rates in the strokectomy group were compared to those for similar patients in recently published randomized controlled trials. RESULTS: Of 198 patients who underwent DC due to cerebral infarction, 12 patients underwent strokectomy as a second surgical procedure, with a median National Institutes of Health Stroke Scale (NIHSS) score of 19 for patients with versus 16 for those without secondary strokectomy (p = 0.029). Either refractory increases of ICP > 20 mm Hg or dilated pupils in addition to herniation visible on CT images were triggers for strokectomy surgery. Ten of 12 (83%) patients had infarctions in more than one territory (p < 0.001). After 12 months, 43% of patients had a good outcome according to the modified Rankin Scale (mRS) score (≤ 3). In the subgroup of patients suffering infarctions in more than one vascular territory, functional outcome after 12 months was better (mRS ≤ 3 in 40% of patients in comparison to 9%; p = 0.027). A 1:3 case-control analysis matched to age, side of infarction, sex, and vascular territory confirmed these results (mRS ≤ 3, 42% in comparison to 11%; p = 0.032). Age, NIHSS score on admission, and number of vascular territories involved were identified as risk factors in multivariate analysis (p < 0.05). Patients in the strokectomy group had more infections (p < 0.001). According to these results, the authors developed a scale (Münster Stroke Score, 0-6 points) to predict whether patients might benefit from additional strokectomy. Receiver-operating characteristic (ROC) curve analysis revealed an area under the curve (AUC) of 0.86 (p < 0.001). The authors recommend a Münster Stroke Score of ≥ 3 as a cutoff, with a sensitivity of 92% and specificity of 66%, for predicting benefit from strokectomy. CONCLUSIONS: In this study in comparison to former studies, mortality rates were lower and clinical outcome was comparable to that of previously published trials regarding large cerebral infarctions. Second surgery including strokectomy may help achieve better outcomes, especially in cases of infarction of more than one vascular territory.

Fatal abusive head trauma cases: consequence of medical staff missing milder forms of physical abuse.

OBJECTIVES: Missed diagnosis of child abuse may lead to chronic abuse with potential for death. This paper reports 3 such cases. METHODS: This is a retrospective chart review of 38 cases diagnosed as abusive head trauma between January 1, 2004 and December 31, 2006 at a university hospital. We sought to identify fatal cases with a past medical history of physical abuse that was missed by the medical staff. RESULTS: Three cases (7.9%) had a past medical history of physical abuse that was missed by the medical staff. Infants were 2 males and 1 female. Their ages were 2.5, 3.5, and 6 months, respectively. Missed abuse episodes involved rib fractures, a metaphyseal tibial fracture, and a shaking episode, respectively. The intervals that had elapsed between the missed and the fatal abuse episodes were 24 hours, 12 days, and 6 weeks, respectively. Perpetrators of fatal head trauma were all biological fathers. One plead guilty, and 2 were convicted of involuntary manslaughter. The infants were in the care of the perpetrators of the fatal abuse episodes at the time the missed abuse episodes occurred. CONCLUSIONS: Physicians assessing children, especially infants, should be alert to indicators of abusive trauma to recognize abuse early on. Including abusive trauma in the differential diagnostic list and taking appropriate steps to rule out or confirm the diagnosis are of paramount importance in establishing child protective services and preventing further abuse and neglect that may at times be fatal.

Procalcitonin as a Biomarker for Malignant Cerebral Edema in Massive Cerebral Infarction.

The objective of this study is to explore whether procalcitonin (PCT) can serve as an early biomarker of malignant cerebral edema in patients with massive cerebral infarction (MCI). Ninety-three patients with acute MCI were divided into death or survival groups based on whether they died or survived within 1 week of cerebral herniation. Differences in laboratory parameters between these two groups were analyzed by univariate analysis, followed by multivariate logistic regression analyses if the influencing factors were significantly different. Compared with the survival group, the patients in the death group had a larger cerebral infarct area, higher body temperature, neutrophil counts, PCT level, and neuron-specific enolase (NSE) level within 48 h of onset. Multivariate logistic regression analyses revealed an odds ratio (OR) of 1.830 or 1.235 for PCT and neutrophil counts respectively, suggesting that PCT and neutrophil counts are two independent risk factors for death in MCI. The area under receiver operating characteristic (ROC) curve was 0.754 for PCT, larger than that for neutrophil counts. Thus, both serum PCT levels and neutrophil counts can be used as biomarkers to predict malignant cerebral edema at the early stages after MCI, but PCT levels are superior predictors of malignant cerebral edema.

Perioperative Management of Children With Giant Encephalocele: A Clinical Report of 29 Cases.

BACKGROUND: Giant encephalocele, a rare entity, makes anesthesiologists wary of challenging anesthetic course. Apart from inherent challenges of pediatric anesthesia, the anesthesiologist has to deal with unusual positioning, difficult tracheal intubation, and associated anomalies during the perioperative course. MATERIALS AND METHODS: Medical records of 29 children with giant encephalocele, who underwent excision and repair, during a period of 13 years, were retrospectively analyzed. Data pertaining to anesthetic management, perioperative complications, and outcome at discharge were reviewed. RESULTS: The average age at admission was 164 days. Hydrocephalus and delayed milestones were present in 19 (65.5%) and 7 (24.1%) children, respectively. Difficulty in tracheal intubation was encountered, in 15 (51.7%) children. Tracheal intubation was attempted with direct laryngoscopy, most often, in lateral position (24 [82.8%]). Intraoperative hemodynamic and respiratory complications were observed in 9 (31.0%) and 5 (17.2%) children, respectively. Intraoperative hypothermia was observed in 4 (13.8%) children. The average stay in the intensive care unit was 2.7 days and average hospital stay was 11.5 days. The condition at discharge remained same as the preoperative period in 24 children (82.7%), deteriorated in 2 (6.9%), and 3 children (10.3%) died. CONCLUSIONS: Management of children with giant encephalocele requires the updated knowledge on possible difficulties encountered during the perioperative period. They need specialized anesthetic care for dealing with difficult tracheal intubation, associated congenital anomalies, unusual positioning, electrolyte abnormalities, hypothermia, and cardiorespiratory disturbances. For securing the airway, we suggest the practice of direct laryngoscopy in lateral position after inhalational induction. Muscle relaxant should be administered only after visualization of the glottis.

Outcome following decompressive craniectomy for malignant swelling due to severe head injury.

OBJECT: The aim of this study was to assess outcome following decompressive craniectomy for malignant brain swelling due to closed traumatic brain injury (TBI). METHODS: During a 48-month period (March 2000-March 2004), 50 of 967 consecutive patients with closed TBI experienced diffuse brain swelling and underwent decompressive craniectomy, without removal of clots or contusion, to control intracranial pressure (ICP) or to reverse dangerous brain shifts. Diffuse injury was demonstrated in 44 patients, an evacuated mass lesion in four in whom decompressive craniectomy had been performed as a separate procedure, and a nonevacuated mass lesion in two. Decompressive craniectomy was performed urgently in 10 patients before ICP monitoring; in 40 patients the procedure was performed after ICP had become unresponsive to conventional medical management as outlined in the American Association of Neurological Surgeons guidelines. Survivors were followed up for at least 3 months posttreatment to determine their Glasgow Outcome Scale (GOS) score. Decompressive craniectomy lowered ICP to less than 20 mm Hg in 85% of patients. In the 40 patients who had undergone ICP monitoring before decompression, ICP decreased from a mean of 23.9 to 14.4 mm Hg (p < 0.001). Fourteen of 50 patients died, and 16 either remained in a vegetative state (seven patients) or were severely disabled (nine patients). Twenty patients had a good outcome (GOS Score 4-5). Among 30-day survivors, good outcome occurred in 17, 67, and 67% of patients with postresuscitation Glasgow Coma Scale scores of 3 to 5, 6 to 8, and 9 to 15, respectively (p < 0.05). Outcome was unaffected by abnormal pupillary response to light, timing of decompressive craniectomy, brain shift as demonstrated on computerized tomography scanning, and patient age, possibly because of the small number of patients in each of the subsets. Complications included hydrocephalus (five patients), hemorrhagic swelling ipsilateral to the craniectomy site (eight patients), and subdural hygroma (25 patients). CONCLUSIONS: Decompressive craniectomy was associated with a better-than-expected functional outcome in patients with medically uncontrollable ICP and/or brain herniation, compared with outcomes in other control cohorts reported on in the literature.

Acute Traumatic Subdural Hematoma: Surgical Management in the Presence of Cerebral Herniation-A Single-Center Series and Multivariate Analysis.

BACKGROUND: Traumatic acute subdural hematoma (aSDH) is a severe disease. Surgical treatment is still controversially discussed, especially in patients with additional signs of cerebral herniation. However, previously investigated patient populations were heterogeneous. We therefore performed an analysis of our institutional data in a large homogenous selection of patients with traumatic aSDH to analyze factors determining clinical outcome. METHODS: Between 2010 and 2014, 196 patients with aSDH underwent surgical treatment in our department. Information including patient characteristics, treatment modality, radiologic features, and functional outcome were analyzed. Outcome was assessed according to the Glasgow Outcome Scale (GOS) at 6 months and was dichotomized into favorable (GOS score, 1-3) and unfavorable (GOS score 4-5) outcome. Furthermore, a multivariate analysis was performed to identify independent predictors of functional outcome. RESULTS: Overall, 26% of patients with aSDH achieved favorable outcome. In further analysis, unilateral or bilateral dilated pupils as a sign of cerebral herniation were present in 47% of the included patients. In the multivariate analysis, age >70 years and the presence of cerebral herniation were significant prognostic predictors for unfavorable outcome in patients with aSDH. However, 15% of patients with aSDH and signs of cerebral herniation achieved favorable outcome during follow-up. CONCLUSIONS: We provide detailed data on patients with aSDH and signs of cerebral herniation. Despite mydriasis, favorable outcome may be achieved in many patients. Nevertheless, careful individual decision making is necessary for each patient, especially when signs of cerebral herniation have persisted for a long time.

Paradoxical Herniation After Unilateral Decompressive Craniectomy Predicts Better Patient Survival: A Retrospective Analysis of 429 Cases.

Paradoxical herniation (PH) is a life-threatening emergency after decompressive craniectomy. In the current study, we examined patient survival in patients who developed PH after decompressive craniectomy versus those who did not. Risk factors for, and management of, PH were also analyzed. This retrospective analysis included 429 consecutive patients receiving decompressive craniectomy during a period from January 2007 to December 2012. Mortality rate and Glasgow Outcome Scale (GOS) were compared between those who developed PH (n = 13) versus those who did not (n = 416). A stepwise multivariate logistic regression analysis was carried out to examine the risk factors for PH. The overall mortality in the entire sample was 22.8%, with a median follow-up of 6 months. Oddly enough, all 13 patients who developed PH survived beyond 6 months. Glasgow Coma Scale did not differ between the 2 groups upon admission, but GOS was significantly higher in subjects who developed PH. Both the disease type and coma degree were comparable between the 13 PH patients and the remaining 416 patients. In all PH episodes, patients responded to emergency treatments that included intravenous hydration, cerebral spinal fluid drainage discontinuation, and Trendelenburg position. A regression analysis indicated the following independent risk factors for PH: external ventriculostomy, lumbar puncture, and continuous external lumbar drainage. The rate of PH is approximately 3% after decompressive craniectomy. The most intriguing findings of the current study were the 0% mortality in those who developed PH versus 23.6% mortality in those who did not develop PH and significant difference of GOS score at 6-month follow-up between the 2 groups, suggesting that PH after decompressive craniectomy should be managed aggressively. The risk factors for PH include external ventriculostomy, ventriculoperitoneal shunt, lumbar puncture, and continuous external lumbar drainage.

Meckel-Gruber Syndrome: a population-based study on prevalence, prenatal diagnosis, clinical features, and survival in Europe.

Meckel-Gruber Syndrome is a rare autosomal recessive lethal ciliopathy characterized by the triad of cystic renal dysplasia, occipital encephalocele and postaxial polydactyly. We present the largest population-based epidemiological study to date using data provided by the European Surveillance of Congenital Anomalies (EUROCAT) network. The study population consisted of 191 cases of MKS identified between January 1990 and December 2011 in 34 European registries. The mean prevalence was 2.6 per 100,000 births in a subset of registries with good ascertainment. The prevalence was stable over time, but regional differences were observed. There were 145 (75.9%) terminations of pregnancy after prenatal diagnosis, 13 (6.8%) fetal deaths, 33 (17.3%) live births. In addition to cystic kidneys (97.7%), encephalocele (83.8%) and polydactyly (87.3%), frequent features include other central nervous system anomalies (51.4%), fibrotic/cystic changes of the liver (65.5% of cases with post mortem examination) and orofacial clefts (31.8%). Various other anomalies were present in 64 (37%) patients. As nowadays most patients are detected very early in pregnancy when liver or kidney changes may not yet be developed or may be difficult to assess, none of the anomalies should be considered obligatory for the diagnosis. Most cases (90.2%) are diagnosed prenatally at 14.3 ± 2.6 (range 11-36) gestational weeks and pregnancies are mainly terminated, reducing the number of LB to one-fifth of the total prevalence rate. Early diagnosis is important for timely counseling of affected couples regarding the option of pregnancy termination and prenatal genetic testing in future pregnancies.

Outlook for the child with a cephalocele.

Specific information on the outcome for a child with a cephalocele can be difficult to find and interpret. To update outcome information for the child with a cephalocele, the investigators compared outcome of 34 infants from their institution with that of those in previously published series. For the infants from the investigators' institution, overall mortality was 29% and was confined to infants with posterior defects, which was consistent with other published series. Additional major congenital abnormalities were present in nearly half the infants, and these were an important factor in contributing to a poorer prognosis as well as whether the defect could be operatively reduced. Seizures and hydrocephalus were often secondary problems in those infants who did worse. In addressing outlook for the infant with the cephalocele, primary factors to be considered are operability and the presence of additional major abnormalities, both intracranial and extracranial.

The use of lobectomy in the management of severe closed-head trauma.

A retrospective review is presented of 20 patients with traumatic brain injury who were treated during the course of their illness by lobectomies either after a herniation or other significant deterioration or to reduce elevated intracranial pressure. All the patients suffered from blunt head trauma. Patient ages ranged from 19 to 59 years (average, 34 yr). The initial Glasgow Coma Scale score ranged from 3 to 15 (average, 8.2). There were 14 frontal lobectomies, 2 temporal, 3 frontal and temporal, and 1 occipital. Surgery was performed between 0 and 8 days after injury (average, 2.8). Outcome was favorable (good or moderately disabled) in 11 patients and unfavorable (severely disabled, persistently vegetative, or dead) in 9. No patients survived in a persistently vegetative state. A higher initial Glasgow Coma Scale score was positively correlated with a more favorable outcome (P < 0.03). Younger patients also showed a significant positive relationship to outcome (P < 0.0005). Better pupillary reactivity showed a significant trend toward a more favorable outcome (P < 0.04). The type of lesions identified on computed tomographic scans had no association with outcome. A lobectomy can be a useful adjuvant in the management of severe brain injury, especially in younger patients with relatively higher initial Glasgow Coma Scale scores who subsequently deteriorate or develop elevated intracranial pressure.

Functional recovery after traumatic transtentorial herniation.

To elucidate the factors associated with functional recovery after traumatic transtentorial herniation, we reviewed the records of 153 consecutive patients admitted with clinical signs of transtentorial herniation (altered level of consciousness, anisocoria or pupillary unresponsiveness, and abnormal motor findings). Overall, 28 patients (18%) had a functional outcome: 14 patients (9%) made a good recovery and 14 were moderately disabled. Sixteen patients (10.5%) were severely disabled or vegetative, and 104 (60%) died. Compared with patients who died or were left severely disabled or vegetative, patients who had a good recovery were younger (21 versus 38 years), were significantly more likely to be children (less than or equal to 17 years old) and have anisocoria and a deteriorating Glasgow Coma Score (GCS), and were significantly less likely to be flaccid or have bilaterally fixed pupils; moderately disabled patients also had a lower median age and a higher frequency of anisocoria. There was no difference in the incidence of significant intracranial hematomas between patients with a functional outcome and those with a nonfunctional outcome. Twenty-seven percent of the 95 patients with anisocoria had a good outcome or moderate disability, whereas only 3.5% of the 58 patients with bilaterally fixed and dilated pupils at admission had a functional recovery (P less than 0.05). Age, level of consciousness, and the degree of residual upper brain stem function at admission appear to be the most important determinants of functional outcome after traumatic transtentorial herniation.