Frontoethmoidal encephalocele represents protrusion of meninges and brain in a sac through a defect in the anterior skull base, mostly as swelling over the nose. Rarely it is associated with facial dysmorphism and palatal cleft. There are various perioperative concerns like airway difficulties, leaking from the swelling causing fluid and electrolyte disturbances, risk of infection, compression of the swelling causing a rise in intracranial pressure, bleeding, hypothermia, etc., In neonates, these challenges rise exponentially because of the inherent difficulties in dealing with this group of patients. Frontoethmoidal encephaloceles are common in low socioeconomic strata and are often missed in the antenatal period. We are reporting a rare case of frontoethmoidal encephalocele, with a huge swelling protruding through the cleft palate and occupying more than 50% of the face and oral cavity, making mask ventilation impossible.

This critique evaluates a recent study on the management of cerebrospinal fluid-related intracranial abnormalities in frontoethmoidal encephalocele, as explored in a retrospective cohort study titled \"Shunt Algorithm for Frontoethmoidal Encephalocele (SAFE): A Retrospective Cohort Study of Published Cases.\" focusing on their strengths, weaknesses, and suggestions for future research. This highlights the positive impacts of the SAFE algorithm in standardizing treatment protocols, improving patient outcomes, and enhancing our understanding of the condition through collaborative data analysis. However, it also addresses limitations, such as potential biases in retrospective data analysis and the need for individualized patient care. This abstract emphasizes the significance of continued research and multidisciplinary collaboration in refining the treatment strategies for frontoethmoidal encephaloceles. Prospective, multicenter studies with standardized protocols, collaborative efforts among institutions, and innovative techniques are needed to advance our understanding and management.

A cerebrospinal-fluid-related (CSF-related) problem occurred in 25-30% of frontoethmoidal encephalocele (FEE) cases. Since there was no algorithm or guideline, the judgment to treat the CSF-related problem often relies upon the surgeon\'s experience. In our institution, the early shunt was preferable to treat the problem, but it added risks to the children. We developed an algorithm, \"Shunt Algorithm for Frontoethmoidal Encephalocele\" (SAFE), to guide the surgeon in making the most reasonable decision. To evaluate the SAFE\'s efficacy in reducing unnecessary early shunting for FEE with CSF-related intracranial abnormality. Medical records of FEE patients with CSF-related abnormalities treated from January 2007 to December 2019 were reviewed. The patients were divided into two groups: before the SAFE group as group 1 (2007 - 2011) and after the SAFE group as group 2 (2012 - 2019). We excluded FEE patients without CSF-related abnormalities. We compared the number of shunts and the complications between the two groups. One hundred and twenty-nine patient\'s medical records were reviewed. The males were predominating (79 versus 50 patients) with an average age of 58.2±7.1 months old (6 to 276 months old). Ventriculomegaly was found in 18 cases, arachnoid cysts in 46 cases, porencephalic cysts in 19 cases, and ventricular malformation in 46 cases. Group 1, with a score of 4 to 7 (19 cases), received an early shunt along with the FEE repair. Complications occurred in 7 patients of this group. Group 2, with a score of 4-7, received shunts only after the complication occurred in 3 cases (pseudomeningocele unresponsive with conservative treatment and re-operation in 2 cases; a sign of intracranial hypertension in 1 case). No complication occurred in this group. Groups 1 and 2, with scores of 8 or higher (6 and 8 cases, respectively), underwent direct shunt, with one complication (exposed shunt) in each group. The SAFE decision algorithm for FEE with CSF-related intracranial abnormalities has proven effective in reducing unnecessary shunting and the rate of shunt complications.

Encephalocele is a rare congenital anomaly characterized by the protrusion of intracranial contents through a defect in the skull base or calvarial. In Southeast Asia, frontoethmoidal encephaloceles are more frequently observed compared to their occurrence in Western countries. Typically, frontoethmoidal encephaloceles present as a visible mass adjacent to the nasal region. In this report, we delineate the case of a 9-year-old boy who presented with a palpable mass on the nasal bridge. Subsequent ultrasound and CT scan evaluations identified a nasofrontal defect with a communicating connection to the intracranial compartment.

Encephaloceles, also known as meningoencephaloceles, are a group of neurological disorders characterized by herniation of the cerebral parenchyma along with overlying meninges through openings in the skull bone. They can be seen following congenital defect in neural tube closure or acquired defect in the skull bone. We report a case of acquired frontoethmoidal encephalocele presenting with drug-refractory epilepsy. Interictal FDG PET-computed tomography done for lateralization and localization of seizure focus showed right-sided frontoethmoidal encephalocele associated with hypometabolism in the adjacent right frontopolar cortex, concordant with ictal onset on electroencephalogram.

Objective  Congenital frontoethmoidal encephaloceles are associated with a shallow sloping forehead. We (1) sought to determine if early repair reverses abnormal forehead slope, and (2) assessed a modification of the fetal profile (FP) line to assess results. Design  Study of two cases. Participants  Newborns with frontoethmoidal encephaloceles repaired prior to the age of 4 months with cranial base bone grafting. Main Outcome Measures  Forehead slope was assessed using a modification of the FP line, defined as the line that passes through the anterior border of the mandible and nasion, on pre and postoperative magnetic resonance imaging (MRI) in the midsagittal plane. A modified FP (mFP) line anterior to the forehead was \" - \", while a posterior (normal) mFP line was \" + .\" The largest distance from the mFP line to the forehead was measured. Results  Both infants underwent bifrontal craniotomy, excision of encephalocele, and repair of cribriform plate defect using full-thickness autologous parietal bone before the age of 4 months. Preoperatively, the mFP line was -20.6 mm in case 1, and -9.8 mm in case 2. In both cases, follow-up MRI showed excellent reversal of forehead slope and normal calvarium development. The mFP line improved to +7.4 (age = 16 months) in case 1, and +7.6 (age = 11 months) in case 2. The parietal bone donor site ossified completely within 3 months in both cases. Conclusion  Early repair with bone grafting can promote normal frontal bone development and improve forehead slope. The mFP line is a useful method to measure degree of forehead slope.

The debate between the extracranial and the intracranial approach for frontoethmoidal (FEE) encephalocele corrective surgery was not summarized yet. The extracranial approach is traditionally believed being inferior to the intracranial approach, but convincing evidence was missing. To provide robust evidence, we conducted a meta-analysis on the incidence of cerebrospinal fluid (CSF) leakage, its progression to infection, the reoperation to treat the leakage, and the recurrence rate between the two techniques. We performed a meta-proportion pooled analysis and meta-analysis on eligible literature following the recommendation of PRISMA guidelines. The outcome of interest was the incidence of CSF leakage, the CSF leakage that progressed into an infection, the reoperation rate to treat the leakage, and the recurrence rate. We included 28 studies comprising 1793 patients in the pooled prevalence calculations. Of the 28 studies, nine studies describing 730 patients were eligible for meta-analysis. The prevalence of CSF leakage was 8% (95% CI, 0.04-0.12) in the intracranial approach and 10% (95% CI, 0.01-0.23) in the extracranial approach The subgroup analysis of the intracranial approach showed higher CSF leakage prevalence in the frontal craniotomy approach (9%; 95% CI, 0.03-0.16) than the subfrontal osteotomy (6%; 95% CI, 0.03-0.12). Meta-analysis study revealed a significantly higher risk of CSF leakage (OR 2.82; 95% CI, 1.03-7.72), a higher reoperation rate (OR 5.38; 95% CI: 1.13 - 25.76), and the recurrence rate (RR 4.63; 95% CI, 1.51-14.20) for the extracranial approach. The event of infected CSF leakage (OR 3.69; 95% CI, 0.52-26.37) was higher in the extracranial than intracranial approach without any statistical significance. The extracranial approach was associated with a higher risk of CSF leakage, reoperation rate to treat the CSF leakage, and the recurrence rates. The infected CSF leakage between the extracranial and intracranial approaches showed no significant difference.

BACKGROUND: Primary encephalocele is a rare deformity that is challenging for the neurosurgeon. It requires a multidisciplinary team for adequate reconstructive surgery.
METHODS: We report the case of a 6-month-old African boy who presented with a frontoethmoidal encephalocele; we present a technical description of the surgical procedure, using no implant.
CONCLUSIONS: The postoperative evolution of the boy was uneventful, with a good clinical result at the follow-up.

Encephaloceles are uncommon in western countries and most cases are located in the occipital bone. Frontal encephaloceles may involve the ethmoid bone, nasal bones and/or the orbits. Surgical repair is complex and usually requires a multidisciplinary approach. The goal of the surgery is to reconstruct the normal anatomy, to achieve a good cosmetic repair and to avoid a cerebrospinal fluid leak. We present a case of a patient with a large congenital frontoethmoidal encephalocele. Autologous calvarian bone grafts were used to repair of encephalocele defect and for the reconstruction of the frontonasal area. The defect closure and the cosmetic result were satisfactory, and the only complication detected was the infection of a previously performed ventriculoperitoneal shunt. A description of the technique and a review of the literature are presented.

BACKGROUND: Frontoethmoidal encephalocele is a congenital abnormality of the anterior skull base involving herniation of cranial contents through a midline skull defect. Patency of the foramen cecum, along with other multifactorial variables, contributes to the development of frontoethmoidal encephaloceles. Because of limited resources, financial constraints, and lack of surgical expertise, repair of frontoethmoidal encephaloceles is limited in developing countries.
METHODS: Between 2008 and 2013 an interdisciplinary team composed of neurosurgeons, craniofacial surgeons, otolaryngologists, plastic surgeons, and nursing personnel, conducted surgical mission trips to Davao City in Mindanao, Philippines. All patients underwent a combined extracranial/intracranial surgical approach, performed in tandem by a neurosurgeon and a craniofacial surgeon, to detach and remove the encephalocele. This procedure was followed by reconstruction of the craniofacial defects.
RESULTS: A total of 30 cases of frontoethmoidal encephalocele were repaired between 2008 and 2013 (20 male; 10 female). The average age at operation was 8.7 years, with 7 patients older than 17 years. Of the 3 subtypes, the following breakdown was observed in patients: 18 nasoethmoidal; 9 nasofrontal; and 3 naso-orbital. Several patients showed concurrent including enlarged ventricles, arachnoid cysts (both unilateral and bilateral), and gliotic changes, as well as orbit and bulbus oculi (globe) deformities. There were no operative-associated mortalities or neurologic deficits, infections, or hydrocephalus on follow-up during subsequent trips.
CONCLUSIONS: Despite the limitations of performing advanced surgery in a developing country, the combined interdisciplinary surgical approach has offered effective treatment to improve physical appearance and psychological well-being in afflicted patients.