Anterior fontanelle closure and diagnosis of non-syndromic craniosynostosis: a comparative study using computed tomography

Abstract Objective: Suspicion of early anterior fontanel (AF) closure is a common reason for referral to a pediatric neurosurgeon because of the suspected increased risk of developing craniosynostosis (CS) in spite of the absence of evidence in the literature. The aim of this study was to analyze the association between AF closure and the diagnosis of non-syndromic CS in Brazilian children. Methods: An observational and case-cohort study was conducted to compare the incidence of closed AF between healthy children (group 1) and children diagnosed with non-syndromic CS (group 2) at a pediatric neurosurgery referral center. The accuracies of completely closed AF and diagnosis of CS were assessed. Results: High-resolution three-dimensional reconstruction computed tomography scans were obtained for 140 children aged < 13 months, of whom 62.9% were boys and 37.1% were girls (p < 0.001). The most common types of non-syndromic CS were trigonocephaly (34, 48%) and scaphocephaly (25, 35.7%). Closed fontanel (27, 38.6%) was observed in both groups, and a sensitivity of 36.1%, specificity of 72%, the positive predictive value of 59%, and negative predictive value of 51% were observed in the patients diagnosed with CS when AF closure occurred before the age of 6 months. Conclusion: The results of this comparative study of AF closure and CS diagnosis suggest that early AF closure does not imply a diagnosis of CS. Pediatricians should be aware of the risk of misdiagnosis of CS in cases with a widely open AF in spite of the presence of CS.

Anterior fontanelle closure and diagnosis of non-syndromic craniosynostosis: a comparative study using computed tomography.

OBJECTIVE: Suspicion of early anterior fontanel (AF) closure is a common reason for referral to a pediatric neurosurgeon because of the suspected increased risk of developing craniosynostosis (CS) in spite of the absence of evidence in the literature. The aim of this study was to analyze the association between AF closure and the diagnosis of non-syndromic CS in Brazilian children. METHODS: An observational and case-cohort study was conducted to compare the incidence of closed AF between healthy children (group 1) and children diagnosed with non-syndromic CS (group 2) at a pediatric neurosurgery referral center. The accuracies of completely closed AF and diagnosis of CS were assessed. RESULTS: High-resolution three-dimensional reconstruction computed tomography scans were obtained for 140 children aged < 13 months, of whom 62.9% were boys and 37.1% were girls (p < 0.001). The most common types of non-syndromic CS were trigonocephaly (34, 48%) and scaphocephaly (25, 35.7%). Closed fontanel (27, 38.6%) was observed in both groups, and a sensitivity of 36.1%, specificity of 72%, the positive predictive value of 59%, and negative predictive value of 51% were observed in the patients diagnosed with CS when AF closure occurred before the age of 6 months. CONCLUSION: The results of this comparative study of AF closure and CS diagnosis suggest that early AF closure does not imply a diagnosis of CS. Pediatricians should be aware of the risk of misdiagnosis of CS in cases with a widely open AF in spite of the presence of CS.

The role of the Liliequist membrane in the third ventriculostomy.

Endoscopic third ventriculostomy (ETV) is a hydrocephalus treatment procedure that involves opening the Liliequist membrane (LM). However, LM anatomy has not been well-studied neuroendoscopically, because approach angles differ between descriptive and microsurgical anatomical explorations. Discrepancies in ETV efficacy, especially among children age 2 and younger, may be due to incomplete LM opening. The objective of this study was to characterize the LM anatomically from a neuroendoscopic perspective to better understand the impact of anatomical features during LM ostomy and the ETV success rate. Additionally, the ETV success score was tested to predict patient outcome after the intraoperatively difficult opening of LM. Fifty-four patients who underwent ETV were prospectively analyzed with a mean follow-up of 53.1 months (1-90 months). The ETV technical parameters of difficulty were validated by seven expert neurosurgeons. The pediatric population (44) of this study represents the majority of patients (81.4%). The overall ETV success rate was 68.5%. Anomalies on the IIIVT floor resulted in an increased rate of ETV failure. The IIIVT was anomalous, and LM was thick in 33.3% of cases. Fenestration of LM was difficult in 39% of cases, and the LM and TC were opened separately in 55.6% of cases. The endoscopic third ventriculostomy success score (ETVSS) accurately predicted the level of difficulty opening the LM (p = 0.012), and the group with easy opening presented greater durability in ETV success. Neurosurgeons should be aware of the difficulty level of the overture of LM during ETV and its impact on long-term ETV effectiveness.

External validation of the ETV success score in 313 pediatric patients: a Brazilian single-center study.

The endoscopic third ventriculostomy (ETV) success score (ETVSS) was developed to predict the success rate of ETV at 6 months. In this study, the authors assessed the performance of this score for > 6 months, i.e., at 12 months, and provided external validation in Brazilian children. All children undergoing first ETV (without choroid plexus cauterization) at a Brazilian single institution for > 20 years were included in the study. The ETVSS was retrospectively calculated for each patient and compared with the actual success of the procedure observed at 6 and 12 months after the procedure. A total of 313 eligible children underwent initial ETV, 34.18% of whom had undergone shunt placement before ETV. The most common etiologies were aqueductal stenosis (45%) and non-tectal brain tumors (20.8%). ETV was successful at 6 months in 229 patients (73.16%) compared with the 61.3% predicted by the ETVSS. The overall actual success rate observed at 1 year after ETV was 65.1% (204 patients). The area under the receiver operating characteristic curve was 0.660 at 6 months and 0.668 at 1 year, which suggested a tendency for the ETVSS to underestimate the actual success rate of ETV at both timepoints. The ETVSS showed good success prediction in accordance with the actual ETV success rate and proved to be useful during the decision-making process of ETV.

Proposed radiological score for the evaluation of isolated fourth ventricle treated by endoscopic aqueductoplasty.

PURPOSE: Evidence supporting the effectiveness of endoscopic aqueductoplasty (EA) for the treatment of isolated fourth ventricle (IFV) is limited to small surgical series of cases. Additionally, studies adopted different radiological outcome criteria, which makes it difficult to compare outcomes accurately. Thus, we aimed to develop a radiological score (RS) as an alternative assessment method for EA. METHODS: The cases of 20 consecutive pediatric patients harboring IFV and treated by EA were retrospectively reviewed. Clinical data and pre- and 1-year postoperative brain images were analyzed. The RS was based on the enlargement of the fourth ventricle and deformation of the cerebellum and brainstem. After randomization, three experts, blinded to patient outcomes, analyzed the brain images and established a consensus for the values of the score. Outcomes were validated by comparing the maximum anteroposterior distance of the fourth ventricle using the RS, pediatric functional status score, and clinical symptoms. RESULTS: The RS was strongly correlated with the anteroposterior distance of the fourth ventricle (Pearson's coefficient = 0.78), and the mean RS dropped from 6.15 to 3.90 (p < 0.001) 1 year after EA. Upward extension (p = 0.021) and brainstem deformation (p = 0.010) were the most significant improved features. There was agreement among RS and symptom improvement in 16 children (80%) and the pediatric functional status score in 14 children (70%). CONCLUSION: In this study, the proposed radiological score proved to be an accurate tool for the evaluation of IFV treatment with EA.

A Technique for Minimizing the Need for Hemotransfusion in Non-Syndromic Craniosynostosis Surgery.

ABSTRACT: Surgery for craniosynostosis is associated with excessive blood loss, as well as morbidity and mortality risks. This study investigated the effectiveness of a surgical technique for nonsyndromic craniosynostosis intervention in controlling bleeding, assessed based on the volume of blood transfused. The cohort included 73 children who underwent nonsyndromic craniosynostosis surgery during a 3-year period. Retrospective evaluation of patient parameters included sex, weight, and age at the time of surgery; type of craniosynostosis; duration of surgery; hemoglobin concentration before and after surgery; rate of transfusion; and volume of transfused blood (mL/kg). The surgical technique involved skin incision and subgaleal dissection using electrocautery with a Colorado needle tip. The pericranium was not removed but instead kept in situ, and orbiectomy was performed using piezosurgery. Of the 73 children in the cohort, 75.3% underwent fronto-orbital advancement and were included in the analysis. The average age was 10.9 months (range: 4-96 months), with 68.5% boys and 31.5% girls (P < 0.001). The most common type of craniosynostosis was trigonocephaly (57.5%), followed by scaphocephaly (19.2%). The mean duration of the surgery was 2 hours and 55 minutes. Blood transfusion was needed in 56.2% of patients, with a mean volume of 8.7 mL/kg body weight transfused intraoperatively. No complications or fatalities were observed. These results suggested that meticulous, continuous control of homeostasis is paramount in minimizing blood loss during surgical repair of nonsyndromic craniosynostosis.

Technical Strategy Using Piezosurgery to Correct Flattened Supraorbital Rim in Unilateral Coronal Craniosynostosis.

ABSTRACT: The surgical correction of orbital deformities in patients with unilateral coronal craniosynostosis is challenging. Traditional techniques have shown the persistence of orbital flattening. This study presents a new strategy for remodeling the compromised orbit, using the piezosurgery technique, which improves the orbital curvature.

Pediatric minor head trauma in Brazil and external validation of PECARN rules with a cost-effectiveness analysis.

BACKGROUND: Pediatric minor head trauma approaches aim to ensure the absence of traumatic brain lesions, minimize ionizing radiation, and enhance cost control. We evaluated the applicability and cost-effectiveness of the Pediatric Emergency Care Applied Research Network (PECARN) rules after head trauma and rationalize the use of head computed tomography (CT) scans. METHODS: We retrospectively divided patients <18 years old who presented at a single institution in Brazil with minor head trauma into four groups: Group I (skull X-ray only), Group II (head CT only), Group III (X-ray and CT), and Group IV (observation only). Direct costs were calculated based on examination and length of hospitalization. The PECARN rules were applied retrospectively in each patient to determine who required a CT scan, and the costs were re-calculated. RESULTS: Of the 1328 patients, CT scans were performed in 36.4% and X-rays in 52.6%. The mean cost was USD 5.88, 34.58, 41.85, and 4.04 for Groups I-IV, respectively. After applying the PECARN rules, 77.6% of patients no longer required a CT scan, and overall costs were reduced from USD 16.71 to 7.88 (p < .001). Conclusion: The PECARN rules demonstrated a meaningful cost-effectiveness and should be applied to the Brazilian pediatric population.

Endoscopic third ventriculostomy in patients with myelomeningocele after shunt failure.

PURPOSE: Myelomeningocele (MMC) is the most frequent form of spina bifida. Moreover, 90% of patients with MMC have hydrocephalus and require shunt placement. However, shunt failure management in such patients typically involves several shunt revisions, increasing the risk for morbidity and mortality. In this study, we report our experience with endoscopic third ventriculostomy (ETV) in MMC patients with shunts in a Brazilian institution. METHODS: Patients with MMC who presented with shunt failure and underwent ETV alone between January 1996 and December 2016 were included in this study. Patients who had undergone endoscopic choroid plexus cauterization (CPC) or had non-shunted MMC hydrocephalus were excluded. Various data related to the ETV procedure were collected for each patient, including features, operative characteristics, complications, and ETV success. The follow-up period for the patients was 6-12 months. ETV success scores (ETVSSs) were calculated retrospectively for each patient to compare the predicted success of the procedure with the actual success at 6 months after ETV. RESULTS: Forty-three MMC patients with shunt failure were included in this study. Of them, 16 (37.2%) had a previous central nervous system (CNS) infection and 4 (9.3%) had complications. Two neuroendoscopies were interrupted, one because of anatomic impossibility due to thickening of the interthalamic adhesion, obliterating the floor of the third ventricle, and another because of intraoperative hemorrhage. One patient developed hyponatremia during the postoperative period, whereas another developed reversible paresis of the oculomotor nerve. The ETVSS predicted a success rate of 49.6% compared with the actual ETV success rate of 53.48% (23 patients) at 6 months. At 12 months, the actual success rate was 41.9% (18 patients). All patients survived the procedure. CONCLUSION: Our findings indicate that ETV is an effective procedure for managing MMC-related hydrocephalus after shunt failure. The expertise of the neurosurgeon in neuroendoscopy procedures is often the deciding factor when choosing a management option. Accordingly, less experienced neurosurgeons should be warned of the risks of this procedure.

Intracranial arachnoid cyst rupture after mild TBI in children: have we underestimated this risk?

The association between intracranial arachnoid cyst rupture and mild brain trauma is infrequently reported. The purpose of this case report is to describe the case of a child with a left temporal arachnoid cyst who suffered rupture with haemorrhage after mild trauma during a football match. The child presented with chronic headache of mild intensity that progressed to a more intense headache after a traumatic event. He underwent surgical intervention after diagnosis of chronic haemorrhage in an arachnoid cyst in the ipsilateral subdural space. The risk of intracranial arachnoid cyst rupture should be considered during the evaluation of oligosymptomatic patients because it is a potentially catastrophic event.

Bridge-therapy with enoxaparin in the preoperative period of endarterectomy.

Cervical clot is one of the complications of endarterectomy. This risk may be higher in patients using aspirin or clopidogrel. On the other hand, stroke may occur if the medication is interrupted before surgery. We carried out a prospective study of 124 endarterectomies in 119 patients in which aspirin or clopidogrel was stopped and a bridge-therapy with enoxaparin was administered preoperatively. There was no case of stroke during the period of the bridge-therapy. One patient developed cervical clot (0.8%) in the fifth postoperative day. Mortality rate in this series was 0.8%. There was no complication directly related to the use of enoxaparin. Bridge-therapy with low molecular weight heparin is a safe strategy for patients elected for endarterectomy.

Bridge-therapy with enoxaparin in the preoperative period of endarterectomy / Terapia-ponte com enoxaparina no período pré-operatório da endarterectomia

Cervical clot is one of the complications of endarterectomy. This risk may be higher in patients using aspirin or clopidogrel. On the other hand, stroke may occur if the medication is interrupted before surgery. We carried out a prospective study of 124 endarterectomies in 119 patients in which aspirin or clopidogrel was stopped and a bridge-therapy with enoxaparin was administered preoperatively. There was no case of stroke during the period of the bridge-therapy. One patient developed cervical clot (0.8 percent) in the fifth postoperative day. Mortality rate in this series was 0.8 percent. There was no complication directly related to the use of enoxaparin. Bridge-therapy with low molecular weight heparin is a safe strategy for patients elected for endarterectomy.

Hematoma cervical é uma das complicações graves de endarterectomia. O risco dessa complicação pode ser maior em pacientes em uso de antiagregante plaquetário. Por outro lado, a suspensão de antiagregante plaquetário no período pré-operatório de endarterectomia eleva o risco de acidente vascular cerebral (AVC). Realizamos estudo prospectivo de 119 pacientes submetidos a endarterectomia (124 procedimentos), nos quais foi suspenso antiagregante plaquetário (aspirina ou clopidogrel) e foi administrada terapia-ponte com enoxaparina subcutânea no período pré-operatório. Nessa série, não houve ocorrência de AVC no período pré-operatório. Um paciente (0,8 por cento) desenvolveu hematoma cervical no quinto dia pós-operatório. A mortalidade nessa série foi de 0,8 por cento. Não houve nenhuma complicação atribuída diretamente ao uso de enoxaparina. A terapia-ponte com heparina de baixo peso molecular demonstrou ser estratégia segura no preparo de pacientes para endarterectomia.