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.

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.

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.

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.

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.

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.

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.

Hernias encefálicas. Clasificación, neuropatología y problemas medicolegales / Brain herniations. Classification, Neuropathology and medico-legal problems

Las hernias cerebrales son la causa inmediata del fallecimiento en muchas lesiones neurológicas. Algunos protocolos recientes de actuación neuroquirúrgica han originado nuevas modalidades, como por ejemplo en el llamado síndrome del trefinado. Se requiere, pues, una revisión actualizada del tema, máxime cuando tiene importantes implicaciones medicolegales. Nuestro objetivo es revisar las clasificaciones, adaptarlas a los avances en Neurorradiología y Neurocirugía, y analizar su problemática medicolegal. La actualización de la clasificación y un conocimiento detallado de la fisiopatología de las hernias cerebrales constituyen la mejor orientación para la resolución de problemas forenses en lo relativo a etiología, causalidad y secuelas de las lesiones encefálicas (AU)

Brain herniation is the immediate cause of death in most of neurological lesions. Some recent neurosurgical protocols have promoted new kinds of herniation, like those that occur in the so-called syndrome of the threphined. Therefore, an up-to-date revision of this subject is required in regard of its important medico-legal implications. Our aim is to review the present classifications, adapting them to Neuroimaging and Neurosurgical advances, and analyzing the medical-legal issues of brain herniation. The problems extend not only to Pathology, but also to Clinical Forensic subjects. An up-to-date classification and a more detailed knowledge of the brain herniations physiology and pathology are the best tools to address their forensic problems, in particular those related with etiology, causation and sequels of brain lesions (AU)

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.

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.

Condroma intracraneal: un caso de muerte súbita / Intracranial chondroma and sudden death: a case report

Las muertes súbitas por tumores intracraneales primarios no diagnosticados en vida son excepcionales. Presentamos un caso de muerte súbita en un adulto joven, sin antecedentes relevantes de interés, en el que la autopsia evidencia como causa de muerte un condroma intracraneal. De acuerdo a la revisión bibliográfica realizada en todos los casos publicados se había efectuado un diagnóstico en vida, por lo que es excepcional que este tumor debute con una muerte súbita. El condroma intracraneal es un tumor benigno, poco frecuente y con características histopatológicas y radiológicas bien definidas. El tratamiento de elección es la resección completa y el pronóstico a largo plazo es bueno. Este caso demuestra que a todas las personas jóvenes que fallecen de forma súbita e inesperada se les debería practicar una necropsia a fin de agotar todos los medios diagnósticos para averiguar la causa de la muerte(AU)

Sudden deaths due to primary intracranial tumours undiagnosed during life are rare. We present a case of sudden death in a young adult with no clinical history of interest. The forensic autopsy discovered that the cause of death was an intracranial chondroma. According to the literature, intracranial chondromas are usually diagnosed in life, so the onset of this tumour as sudden death is exceptional. Intracranial chondroma is a rare benign tumour. Histopathological and radiological characteristics are well defined. The treatment of choice is complete resection, and the long-term prognosis is good. This case shows that it would be convenient to perform an autopsy of all unexpected sudden deaths in young people, in order to determine the cause of death(AU)

Raised intracranial pressure (ICP): management in emergency department.

Raised intracranial pressure is a life threatening condition; unless recognized and treated early, it may progress into herniation syndrome and death. Symptoms and signs are neither sufficiently sensitive nor specific, hence a high index of suspicion and vigilance are needed for early recognition. Immediate goal of management is to prevent / reverse herniation and to maintain good cerebral perfusion pressure. The therapeutic measures include stabilization of airway, breathing and circulation, along with neutral neck position, head end elevation by 30°, adequate sedation and analgesia, minimal stimulation, and hyperosmolar therapy (mannitol or 3% saline). Short-term hyperventilation (to achieve PCO(2) ≈ 30 mm Hg) using bag ventilation can be resorted to if signs of impending herniation are present.

[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.

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.

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

OBJETIVO: Evaluar el impacto de la fortificación de alimentos con ácido fólico en las tendencias de las prevalencias de los defectos del tubo neural (DTN) y la tasa de mortalidad infantil (TMI) por este trastorno en Costa Rica. MÉTODOS: Se analizaron los datos de vigilancia del Centro de Registro de Enfermedades Congénitas y el Centro Centroamericano de Población. Se consideraron defectos del tubo neural la anencefalia, la espina bífida y el encefalocele. Se examinaron las tendencias durante 1987-2009, así como las diferencias de tasas (intervalo de confianza [IC] 95 por ciento) de prevalencia y mortalidad antes de la fortificación de alimentos con ácido fólico y hasta 12 años después de su implementación. Se determinó el aporte de la fortificación al descenso en la TMI general. RESULTADOS: En 1987-1997, previo al período de fortificación de alimentos con ácido fólico, la prevalencia de DTN fue de 12/10 000 nacidos (IC95 por ciento: 11,1-12,8), mientras que en 2009 fue de 5,1/10 000 nacidos (3,3-6,5). La TMI por DTN en 1997 fue de 0,64/1 000 nacimientos (46-0,82) y en 2009 de 0,19/1 000 (0,09-0,3). La TMI por DTN y su prevalencia disminuyeron en forma significativa, 71 por ciento y 58 por ciento respectivamente (P < 0,05). La TMI general disminuyó de 14,2/1 000 nacidos en 1997 a 8,84/1 000 en 2009 (P < 0,05). El descenso en la TMI por DTN contribuyó a una caída de 8,8 por ciento en la TMI general entre 1997 y 2009. CONCLUSIONES: La fortificación de alimentos con ácido fólico provocó una reducción de DTN al nacimiento y de la TMI por esta malformación durante el período 1997-2009, así como también el descenso de la TMI general. Existe relación de temporalidad entre el inicio de las políticas de fortificación y el descenso de la prevalencia y mortalidad por DTN. Se debe pro-mover esta intervención en los países de América Latina y el Caribe donde todavía no ha sido implementada.

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 percent 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 percent 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 percent, and 58 percent, 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 percent 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.

Encephalocele in Uganda: ethnic distinctions in lesion location, endoscopic management of hydrocephalus, and survival in 110 consecutive children.

OBJECT: This study characterizes the first clinical series of encephalocele (EC) from East or Central Africa, and is the largest reported from the African continent. The authors explored survival, the efficacy of primary endoscopic management of associated hydrocephalus, and ethnic differences in EC location. METHODS: One hundred ten consecutive children presented to CURE Children's Hospital of Uganda for treatment of EC over a 9-year period. Clinical data, including patient demographic information, birth date, lesion type (sincipital, parietal, or occipital), operative data, and subsequent course had been entered prospectively into a clinical database. Home visits to update the status of those lost to follow-up were done when possible. With appropriate institutional approvals, the database was reviewed for this retrospective study. Two-tailed probability values calculated using the Fisher exact test were used to assess the significance of differences among groups, with p < 0.05 being considered significant. The Kaplan-Meier method was used for analysis of survival and treatment success probabilities. RESULTS: There were 53 (48%) occipital, 33 (30%) sincipital, and 24 (22%) parietal lesions. Occipital lesions were significantly more common among children of Bantu origin (p = 0.02). Nilotes demonstrated a roughly equal distribution among sincipital, parietal, and occipital locations. The female/male ratio was 1.2, with no difference between EC types (range 1.0-1.4, p = 0.6-0.8). Of 110 patients, 108 (98%) underwent surgical repair at a median age of 1 month (mean 15.7 months), whereas 2 had treatment for hydrocephalus only. Wound revision was required in 13% of cases. Surgery-related mortality was 3%. One-year and 5-year survival rates were 87% (95% CI 0.79-0.93) and 61% (95% CI 0.51-0.70), respectively. Hydrocephalus required treatment in 32%, and was equally common among the 3 EC types. Thirteen patients were treated with combined endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC) and 2 with ETV alone, whereas 18 patients received primary shunt placement. Predicted treatment success at 1 year was 79% for ETV or ETV/CPC (95% CI 0.50-0.94) and 47% for shunt placement (95% CI 0.24-0.71). CONCLUSIONS: Analysis of this first EC series from this region suggests that sincipital lesions are 3 times more common in East than in West Africa. Occipital lesions predominate in patients of Bantu origin, but not among those of Nilotic descent. Hydrocephalus incidence was equally common among different EC types, and endoscopic treatment was more successful (79%) than shunting (47%) at 1 year. The 5-year mortality rate was similar to that for infants with myelomeningocele in Uganda, and more than twice that for their unaffected peers.

Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases.

OBJECT: Frontoethmoidal mengingoencephaloceles (fMECs) are frequently observed in Cambodia, especially in poor families. The authors describe issues related to the surgical treatment of fMECs in Cambodia at the end of a humanitarian program that provided surgery free of charge to patients and their families. METHODS: The authors reviewed 257 cases of fMEC involving patients who presented to their institution, the Children's Surgical Center in Phnom Penh, between 2004 and 2009. They treated 200 of these patients surgically (108 males, 92 females; 89% younger than 18 years) using a "low-cost" management plan with no routine pre- or postoperative investigations. Initially, surgery was performed by visiting foreign surgeons who taught the procedures to resident surgeons. Patients were not charged for consultations or treatment and received at least 1 follow-up examination 6 months postoperatively. RESULTS: The nasoethmoidal type was the most frequent fMEC encountered (69%). Many patients had associated ophthalmological issues (46% of cases). Only 1 familial case was detected. Combined neurosurgical and facial procedures were successfully standardized and learned by surgeons initially unfamiliar with fMEC management. A neurosurgical approach avoided the need for a facial incision in 42 cases, improving cosmetic results. The most common postoperative issues were a temporary CSF leak (24 cases [12%]) and/or infection (28 cases [14%]). There were 3 deaths directly related to the operations. Cosmetic results were good in 145 cases, average in 27, poor in 7, and worse than preoperative appearance in 6 patients. Fifteen patients were lost to follow-up. The parents of 87% of the children were rice farmers. Questionnaire results confirmed that fMEC has important social and educational consequences for the affected children and that these consequences can be partially improved by fMEC correction. CONCLUSIONS: This experience in fMEC management demonstrates that local surgeons can treat these malformations with limited surgical materials and in a nonspecialized infrastructure after principles of treatment have been learned and if they are carefully respected. Surgery for fMEC can thus be more accessible to a larger number of patients in developing countries. Moreover, local treatment facilitates better postoperative and follow-up care.