Frontoethmoidal Encephalomeningocele Research Articles

Three Years Follow-Up of Single-Stage Correction with Modified Chula Technique for Frontoethmoidal Encephalomeningocele: The Advantage of The Teamwork Approach (A Case Report)

Backgrounds: The main objective of Frontoethmoidal encephalomeningocele (FE) treatment are neural morbidities defect correction and aesthetically pleasing looks. Staged procedures are used to be performed in Indonesia. This article aimed to reveal the result of FE correction through the single-stage modified Chula technique (ST-MCT) procedure in collaboration with the neurosurgery team. Case Reports: A rare case of 5 years old girl diagnosed with FE was reported in this study. The FE was slowly increased in size, causing apparent facial deformity and the appearance of telecanthus. An ST-MCT procedure in collaboration with the neurosurgery team was conducted to correct the defects. The IOD values, IPD values, postoperative complications, and anesthetic improvements were evaluated in this study. Result: The patient was well after the surgery, with no complications and short length of stay. There were noted improvement of ICD, IOD and IPD postoperative follow up. At three years after the surgery ICD= 29mm; IOD= 26mm; IPD= 52 mm (normal range). Summary: ST-MCT procedure conducted in collaboration with neurosurgery team had shown excellent correction of ICD, IOD, and IPD values, no complication, shorter length of stay, and minimal scars. It considers as the most proper technique to reach a good result of correction and aesthetically pleasing looks in FE cases.

Frontoethmoidal Encephalomeningocele Correction by Medial Orbital Composite-Unit Translocation Technique: 13 Years of Siriraj Hospital Experience.

Frontoethmoidal encephalomeningocele (FEEM) is a congenital anomaly involving herniation of the glial tissues and the meninges. Our unit implemented single-stage repair in 1996, and this was followed by the introduction of the medial orbital composite-unit translocation (MOCUT) technique for encephalocele repair in 2001. This report describes the long-term outcomes of patients who underwent the MOCUT technique. Data were collected on 32 patients operated on between 2005 and 2018. A full analysis was subsequently made of the data from 4 selected patients who had been monitored for at least 5 years, and their age-related anterior interorbital distances were compared. Two of the patients, who had simple forms of FEEM, demonstrated improvements in their intercanthal and anterior interorbital distances, whereas telecanthus was experienced by the 2 other cases, both of whom had complex FEEM types. We recommend using the MOCUT technique for simple types of FEEM (especially Type IA) and patients whose medial canthal ligaments are still anatomically attached to the medial orbits.

CT-Based Measurements of Facial Parameters of Healthy Children and Adolescents in Thailand.

Facial parameters are used for evaluating normal growth patterns, diagnosing patients with craniofacial abnormalities, and planning surgical procedures. However, these parameters vary by ethnicity and race. This study aims to describe soft-tissue and bony facial parameters based on CT of healthy pediatric and adolescent patients in Thailand. CT imaging of the brain, orbit, facial bones, and neck was performed at Maharaj Nakorn Chiangmai Hospital, in patients from birth to 19 years old. Patients with known syndromic disease, craniofacial syndrome, facial trauma and/or infection, and previous surgery that deformed the study area were excluded. The key points of measurement were soft-tissue intercanthal, bony interorbital, and bony lateral orbital distances. There were 932 patients: 554 males (59.4%) and 378 females (40.6%). Facial parameters rapidly increased in the first 2 years of life. Significant differences in these parameters between the males and females were found at the age of ≥15 years. However, ratios of the interorbital to the lateral orbital distance were generally consistent among age groups in both sexes, at 0.25. This study, in Thailand, provides detailed age- and sex-specific normative data of the craniofacial measurements in children and adolescences based on CT imaging. These data can be used for evaluating individual patients with craniofacial abnormalities as well as determining the treatment in Thai and Asian populations, in whom craniofacial abnormalities, for example, frontoethmoidal encephalomeningocele, are common.

The relationship between external bony defects and widened lateral interorbital distance in frontoethmoidal encephalomeningocele

PurposeThe aim of this study was to identify the prevalence of the abnormal lateral interorbital distance (LIOD), micropthalmia or anopthalmia and incidence of hydrocephalus in frontoethmoidal encephalomeningocele (FEEM), and to identify the correlation between the external bony defects and a widened lateral orbital wall. Materials and methodsFEEM patients who were treated in the Faculty of Medicine, Chiang Mai University between January 1, 2006, and December 31, 2016, were retrospectively reviewed from the medical records. We included the patients who had undergone head computed tomography (CT) of the facial bones. Exclusion criteria were any patients who had undergone prior facial surgery or had maxillofacial bone trauma before they underwent a CT scan, craniofacial disease such as facial clefts, frontonasal dysplasia, craniosynostosis, and inter-frontal encephalocele. Measurements were taken from the CT images to establish the diameter of the external bony defect, medial interorbital distance and lateral interorbital distance. The relationships between the external bony defect and widened LIOD were analyzed. Patients were divided into two groups: those with normal LIOD and those with wide LIOD. ResultsA total of 94 FEEM patients were evaluated. Thirteen patients (13.83%) had a wider than normal LIOD. The incidence of micropthalmia or anopthalmia was 9.57% and hydrocephalus was 45.74%. From a univariable model, there were no statistically significant differences in terms of patient characteristics between the LIOD groups, except for sex. The proportion of wide LIOD was higher in male patients (male vs female; 20.34% vs 2.86%, p = 0.027). The proportion between the external bony defect and the LIOD (EL ratio) associated with a wide LIOD was shown in a cutoff point of 23% (AuROC = 73.27%, 95%CI = 59.87%–86.66%). The multivariable analysis indicated factors associated with abnormal LIOD were an EL ratio of 23% (adjusted RR = 1.98, 95% CI = 1.58–2.51) and male sex (adjusted RR = 6.85, 95% CI = 2.00–23.55). ConclusionThere is an association between the EL ratio and widening of the LIOD. An EL ratio greater than 23% could be used to moderately predict a wide LIOD. A large external bony defect may be associated with lateral displacement of the orbit causing true hypertelorism. Therefore, orbital translocation must be considered in patients with a wide LIOD.

Internasal: A New Type of Frontoethmoidal Encephalomeningocele.

Frontoethmoidal encephalomeningocele (FEEM) is a congenital anomaly with a skull base defect at the foramen caecum and a protrusion of meninges and brain tissue through an external defect on the face. The external defect was classified to nasofrontal, nasoethmoidal, naso-orbital, combined, and abortive type. The aim of our study was to present a newly discovered internasal type (IN) of FEEM. Ninety-six FEEM patients' preoperative 3-dimensional computer tomography scans were studied in the Department of Surgery, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand followed by treatment from 2006 to 2016. Sixteen patients had an external bony defect between the nasal bone that could not be classified in any previous classification. We suggest a new type of FEEM called "IN." The bony defect of this IN can be located on the upper nasal bone (4 cases), middle nasal bone (5 cases), or combined with naso-orbital type (7 cases) counted in the combined type. Our study presents a newly discovered IN of FEEM. This IN can cause hypertelorism when the nasofrontal type could not. Preoperative 3-dimensional computer tomography scans are very helpful for surgical planning.

Management of the Frontoethmoidal Encephalomeningocele.

Introduction:The Frontoethmoidal encephalomeningocele (FEEM) is a congenital neural tube anomaly, with herniation of intracranial material such as the brain and leptomeninges through a defect of the dura and anterior skull base at the junction of the frontal and ethmoidal bones. It may result not only in neural defects, sensorimotor deficits, neurological morbidities, visual impairment, impaired nasal function, and a potential risk of intracranial infection, but also in significant craniofacial disfigurement with complex deformities in the frontal, orbital, and nasal regions.Materials and Method:The standard two-staged surgical protocol comprises of the first stage performed by a neurosurgeon, which aims at correcting the neural defect by a formal craniotomy; then the second stage performed by a craniomaxillofacial or plastic and reconstructive surgeon, to correct craniofacial hard and soft tissue deformities. The case discussed was managed using a modified intracranial-transcranial single stage approach, achieving both the desired objectives.Results and Conclusion:This protocol elucidates the importance and value of teamwork between the Neurosurgeon and Craniomaxillofacial surgeon, in comprehensively and efficiently managing small to moderately sized FEEMs, assuring their complete elimination, satisfactory defect closure, effective functional treatment as well as esthetic correction and reconstruction of the attendant craniofacial deformities by means of a single-stage definitive surgical approach.

Incidence of Associated Brain and Ophthalmic Anomalies in Frontoethmoidal Encephalomeningocele Evaluated by Multidetector Computed Tomography Facial Bone Imaging.

Frontoethmoidal encephalomeningocele (FEEM) is a congenital disorder characterized by herniation of brain and meninges through an anterior skull defect. The main pathological changes are found internally at the foramen cecum and externally at the frontonaso-orbital region. The aim of this study was to determine the incidence of developmental anomalies found in FEEM using multidetector computed tomography images of the facial bone. A total of 78 patients who underwent multidetector computed tomography scan of the facial bone during the January 1, 2003 to June 31, 2012 study period were retrospectively reviewed. Demographic data, size of internal defect, and intracranial anomalies were recorded. Associated brain and ophthalmic anomalies were identified in 53 patients (67.9%), and all of those had brain anomalies. The most common brain anomalies were absent/undetermined septum pellucidum (75.5%), ventricular dilatation (71.7%), abnormal frontal horn (67.9%), and dysgenesis of the corpus callosum (58.5%). Eight patients (10.3%) had ophthalmic anomalies. Patients with brain and ophthalmic anomalies tended to have a higher incidence of large-size internal defects. The authors found a high prevalence of developmental anomalies in FEEM patients. Absent/undetermined septum pellucidum was the most commonly found anomaly in this study. Brain and ophthalmic anomalies tended to have a higher incidence of large-size internal bone defects. Consistent with the authors' acknowledged limitation, further studies using dedicated brain MRI and magnetic resonance angiography to evaluate parenchymal abnormalities and vascular anomalies may be beneficial for surgical planning, prognosis, and the identification of clinical correlations.

Inconspicuous Nasoethmoidal Encephalocele Might Be Wrongly Diagnosed.

Dear Editor-in-Chief, We have read the paper of Song et al. [1] on frontonasal dysplasia deformity. It was an interesting discussion of an image that depicts a boy with a nose deformity, and it was reported that no basal encephalocele was observed. We would like to point out that we had a similar case of a girl with the same nose deformity. As in the report by Song et al., our patient also presented with mild hypertelorism, a broad nasal root and a bifid nasal tip (Fig. 1). The defect at the nasal dorsum had never increased in size and was not cystic. Her parents were anxious about the cosmetic appearance of their child and requested an early excision of the nasal defect and rhinoplasty. Fig. 1 (A, B) A 6 months old, Malay girl with features of nasoethmoidal encephalomeningocele, including telecanthus, broadening of the nasal root with irregular nasal dorsal skin and lack of a nasal tip. Intraoperatively, we noted a very small stalk passing between the nasal bone and the upper lateral cartilage (Fig. 2A, B). The stalk was faintly seen on the magnetic resonance imaging due to its very small size. This small stalk passed from an internal location between the frontal and ethmoidal bone to an external location between the nasal bone and the upper lateral cartilage (Fig. 2C). Fig. 2 (A, B) Stalk of the lesion passing in between the nasal bone and upper lateral cartilage. (C) Small fine stalk in a T2-weighted magnetic resonance imaging (shown with a red arrow). Due to this inconspicuous finding, a frontoethmoidal encephalocele may be missed and wrongly diagnosed. Frontoethmoidal encephalomeningocele is associated with hypertelorism, a broad nasal root, bifid nasal tip, widow's peak and encephalocele, all of which were present in our patient. Based on the clinical review by Rojvachiranonda et al. [2], our patient did have a small herniation mass with a very small stalk of 2 mm passing through a long narrow exit pathway. Due to the small diameter of the stalk and mass, there were no external bone defects or a huge facial deformity. Only skin thickening was present, without a secondary pressure effect, which correlates with the classification on the basis of the soft tissue aspect by Rojvachiranonda et al. [2]. Based on the proposed classification by Rojvachiranonda et al. [2], which describes a facial deformity, external bone defect, exit pathway and malformation of the brain, this patient could be classified as F1c E1NE E1 M0. Thank you. Yours sincerely,

Carbapenem-resistant Acinetobacter baumannii septicemia and meningitis in a neonate treated with colistin and netilmycin

A term, male infant with a large frontoethmoidal encephalomeningocele underwent a cranial defect repair with excision of nasoencephalocele after the mass had ruptured. Two days after operation, the wound became infected and the infant developed sepsis and meningitis due to carbapenem-resistant Acinetobacter baumannii. Ultrasonographic study of the brain revealed severe obstructive hydrocephalus and evidence of ventriculitis. He was treated with intravenous netilmycin, ampicillin/sulbactam and oral rifampicin. Four days later, the blood and cerebrospinal fluid cultures grew no organism but the infant still had persistent thrombocytopenia with evidence of disseminated intravascular coagulation, so colistin 5 mg/kg/day given intravenously every 8 hr was added. His condition gradually improved. He was treated with intravenous amphotericin B concomitantly due to nosocomial fungal infection. Colistin was given for 6 weeks; netilmycin, ampicillin/sulbactam and rifampicin were given for 4 weeks. There was no rising of serum creatinine even though he also received other nephrotoxic agents. Intravenous colistin in combination with netilmycin and rifampicin may be a potentially effective and safe therapy for meningitis caused by carbapenem-resistant A. baumannii in infant.

Outcome of anesthetic management for children with craniofacial deformities

The challenge for anesthetic management for children with craniofacial deformities is to develop comprehensive anesthetic care. The purposes of this study were to review the authors' experience with airway management and ventilator support and evaluate outcome during the perioperative period among these patients at the Tawanchai Center, Srinagarind Hospital. A retrospective (2005-2009), descriptive study was conducted to analyze the anesthetic management and perioperative outcomes among children with craniofacial deformities at the Tawanchai Center. Techniques for airway management included direct laryngoscope (DL), DL with stylet, fiber-optic bronchoscope (FOB), laryngeal mask airway, retrograde intubation, a combination of these techniques and more than one anesthesiologist. Forty-five children with craniofacial deformities were classified as having the following: craniosynostosis; craniofacial microsomia; Treacher-Collins syndrome (TCS); coloboma of the eye, heart defects, atresia choanae, retarded growth and/or development, genital and/or urinary abnormalities, ear abnormalities and deafness (CHARGE) syndrome; frontoethmoidal encephalomeningocele; Pierre Robin sequence; and craniofacial cleft. Most of the patients underwent induction of anesthesia by inhalation of an anesthetic agent. The children with TCS were the most difficult group with regard to airway management with DL view (13%), and multiple airway accessories and intubation techniques were needed. The two most common methods for management of difficult airway obstructions were the use of stylet and FOB. No complications occurred during intubation and minor complications were observed during the perioperative period. A successful outcome depended on the provision of interdisciplinary craniofacial team management, comprehensive anesthetic evaluation and management, well-informed and cooperative children and supportive family members.

Purse-String Closure for Frontoethmoidal Encephalomeningocele

Skin incision over the nose is routinely made for accessing the nasal structures, removing the mass, as well as resecting the redundant skin in patients with frontoethmoidal encephalomeningocele (FEEM). Unfortunately, the conventional elliptical excision leaves a long, straight-line scar that becomes a stigma of the disease. The author describes a purse-string closure technique for closure of the skin defect over the nasal dorsum in a patient with FEEM, which results in the reduction of a surgical scar. The skin overlying the encephalocele is pinched and marked around the mass. The skin is cut, and the encephalocele is dissected deep down the bony opening. Then a bicoronal scalp flap and frontal craniotomy and bilateral medial orbital walls osteotomies are performed. The encephalocele is removed at the neck, and the dural defect is repaired. The bony defect is repaired and bone-grafted as necessary. The skin defect is closed with double layers with the purse-string closure technique and crisscross mattress sutures. : Between January 2004 and July 2009, a total of 7 FEEM patients underwent a 1-stage combined intracranial and extracranial repair and reconstruction of the deformity using the purse-string closure technique. The purse-string closure technique as described provides an alternative skin closure for the repair and reconstruction of FEEM.

A New Approach on Reconstruction of Frontonasal Encephalomeningocele Assisted With Medpor

The frontoethmoidal encephalomeningocele, which is a congenital brain herniation through skull defect at the frontoethmoidal region, is a common problem in Southeast Asia. Patients usually present with perinasal mass(es), and its pressure affects facial structures resulting to conditions such as telecanthus, medial canthal dystopia, long nose, and elevated medial eyebrows. Currently 1-stage surgical correction is widely accepted. Facial incision is routinely made for accessing the facial structures, removing the mass as well as resecting the covering skin which is usually expanded and redundant. Resultant facial scar is usually extensive and becomes a stigma of the disease even after a surgical correction. This study demonstrates the possibility of doing definitive surgery with minimal facial incision and bone defect reconstruction with the alloplastic material. In this study, we used the porous polyethylene implant for bone defect. This can be an alternative approach to autogenous reconstruction of frontoethmoidal encephalomeningocele.

Secondary Craniofacial Reconstruction of Huge Frontoethmoidal Encephalomeningocele After Primary Neurosurgical Repair

Frontoethmoidal encephalomeningocele is a congenital herniation of intracranial contents, including meninges, brain and part of the ventricle, through a bony defect in the skull at the junction of the frontal and ethmoid bones. Management involves meticulous preoperative assessment using computed tomography scans and magnetic resonance imaging, and surgical repair of the central nervous system, skeletal deformities of the orbit, downward displacement of the medial canthi, upward displacement of the eyebrows, and nasal deformities. Frontoethmoidal encephaloceles are best operated on via a craniofacial approach which enables repair of the central nervous system and skeletal deformities in one stage. However, a two-stage reconstruction must be considered when a prolonged operative time is expected or the patient's general condition increases the risks. There have only been a few reports of two-stage reconstructions. We performed a two-stage reconstruction of a huge frontoethmoidal encephalomeningocele, with neurosurgical repair during the first procedure and craniofacial reconstruction during the second procedure. We report on the surgical procedures and the problems encountered.

Risk factors associated with the occurrence of frontoethmoidal encephalomeningocele

Objectives To determine factors associated with the occurrence of frontoethmoidal encephalomeningocele (FEEM), a congenital defect with unique geographical distribution. Methods The subjects of this study were 160 unrelated cases of FEEM. Subjects were recruited between 1999 and 2006 from 15 medical centers throughout Thailand. Data obtained from FEEM cases were analyzed and compared with data from 349 cases of oral clefts studied in the same centers and during the same time and those from the general population (GP) taken in 2003. Results About 52% of FEEM cases had brain anomalies which were not different among types of FEEM. We found familial aggregation reflected by an increased risk to siblings. All of the FEEM cases were of Thai nationality and came from low socioeconomic status. Seven FEEM cases had amniotic rupture sequences. Compared with oral clefts, advanced maternal age (OR: 1.08, 95% CI: 1.02–1.15) was found to be associated with FEEM. In addition, the interpregnancy interval between the FEEM cases and their previous siblings was significantly longer than that of the oral cleft patients and unaffected sibs (OR: 1.17, 95% CI: 1.06–1.28). Conclusions Low socioeconomic status, advanced maternal age, and a long interpregnancy interval may lead to an unfavorable intrauterine environment which, with a certain genetic background such as Thai ethnicity, could contribute to the occurrence of FEEM.

Perioperative management for surgical correction of frontoethmoidal encephalomeningocele in children: a review of 102 cases

Frontoethmoidal encephalomeningocele (FEEM) is a congenital neural tube defect characterized by herniation of brain and meninges through an anterior skull defect. The extruding mass results in a cutaneous expanding lesion and facial deformity. The objective of this study was to review perioperative management for surgical correction of this condition. We reviewed the charts of FEEM children who were treated by surgical correction in King Chulalongkorn Memorial Hospital during 1995-2005. The collected data were categorized into preoperative, intraoperative and postoperative data. In addition, average blood loss was calculated. A total of 102 charts were reviewed. Preoperative data: The mean age of the patients was 4.62 years. Forty-six patients (45%) were smaller than average weight or height. Preoperative-associated abnormal conditions were found in 69 cases (68%). Intraoperative data: The mean duration of the anesthesia and surgery were 6.35 h and 5.51 h, respectively. The estimated mean red cell mass loss was 13.04 ml.kg(-1). There is one case of difficult intubation. No major anesthetic complication was found. Postoperative data: The average intensive care unit stay and hospital stay were 1.55 days and 11.51 days, respectively. The main postoperative complications were tonic-clonic seizure and serious infection including septic shock from wound infection and meningitis. We reported perioperative management in 102 FEEM children. Comprehensive care during preoperative, intraoperative and postoperative period is essential for successful outcome.

Correction of the Frontoethmoidal Encephalomeningocele with Minimal Facial Incision

At present all surgical techniques to correct the frontoethmoidal encephalomeningocele require extensive incisions over the mass and perinasal area, thus adding scars to the already-disfigured faces. This study demonstrates a possibility of doing definitive surgery with minimal facial incision. The technique follows the principles of the "Chula technique," which is the one-stage definitive technique without formal frontal craniotomy. However facial incision was kept to minimum, or even avoided, while amputation of the herniation, dural repair, skull defect closure, and repositioning of the medial canthal ligaments were performed mainly via the coronal incision. There were 20 patients operated on using this modified Chula technique. No perinasal incision was needed at all in three patients (15%) with F1 masses (small- and medium-sized masses according to the "FEEM classification"). Three patients with F1 masses had only small stab incisions just medial to the medial canthus for medial canthopexy. The rest (70%) consisting of two F2 (large-sized) masses and twelve F1 masses had limited nasal incisions just to help removing the facial masses and correcting facial deformity. With an average of 287 days of follow-up period (14-997 days), there had been no cerebrospinal fluid leakage or disease recurrence. Correction of the frontoethmoidal encephalomeningocele can be done safely via the coronal incision alone while facial incision can be omitted or, if necessary, kept to minimum.

One-Stage Operation for a Frontoethmoidal Encephalomeningocele Associated with Ventriculomegaly-A Case Report

Encephalomeningoceles, especially in the frontoethmoidal region, are a form of neural tube defect which affect patients in Southeast Asia more commonly than those in Western countries. We performed a 1-stage reconstruction and ventriculoperitoneal (V-P) shunt placement for a frontoethmoidal encephalomeningocele in an 18-year-old male. We used the 3D reformation computed tomography (CT) and magnetic resonance imaging (MRI) to delineate the skull defect and the exit pathway of the herniated brain tissue and to determine the surgical strategy. The frontoethmoidal encephalomeningocele was completely resected via a 1-stage operation. Neither hydrocephalus nor cerebrospinal fluid (CSF) rhinorrhea was noted after the operation. However, Klebsiella pneumoniae meningitis was diagnosed 2 weeks after the operation. The infection was cured by the use of meropenem. A good aesthetic result was achieved. A 1-stage operation is necessary for large frontoethmoidal encephalomeningoceles and should be performed as early as possible in order to resect the mass, to reconstruct the defect, and to restore the aesthetic appearance with assistance of 3D reformation CT and MRI. A V-P shunt or extraventricular drainage (EVD) catheter placement needs to be considered during the operation for large encephalomeningoceles coexisting with hydrocephalus.

Endocrine Abnormalities in Patients with Frontoethmoidal Encephalomeningocele

Background: Frontoethmoidal encephalomeningocele (FEEM) is a congenital neural tube defect which is more common in Southeast Asia. Its etiology is unknown. No one has ever reported endocrine abnormalities in this condition. Method: We retrospectively reviewed medical records of 84 patients with FEEM, primarily focused on the clinical data suggestive of endocrine disorders including growth parameter, thyroid function test, serum electrolyte and serum osmolality. Results: Nasoethmoidal was the most common subtype found in this study. Sixty-four percent of our patients had their heights below the mean height of normal Thai children. The incidence of hypothyroidism (1:28), central DI (1:42) and GHD (1:42) were higher than that in the general population. Conclusion: A significant number of patients with FEEM had clinical evidences suggestive of hypothalamopituitary insufficiency. Appropriate replacement therapy is mandatory in order to prevent untoward complications.

Frontoethmoidal encephalomeningocele: new morphological findings and a new classification.

Given a lack of a comprehensive classification for the frontoethmoidal encephalomeningocele (FEEM), clinical, photographic, and computed tomography (CT) data of 23 nonoperated patients were reviewed. Extracranial pathological findings of interest included herniation masses, facial deformities, and frontonasal bone morphology. Intracranial pathological findings of interest included morphology of the anterior cranial floor and brain malformations. Stereographic software processed data from a new-generation CT scanner into three-dimensional pictures that revealed some interesting morphological findings not often appreciated (eg, herniation mass without underlying external bone defect; mass at location far from external bone defect ["sequestrated cephalocele"]; new type of external bone defect characterized by a combination of nasoethmoidal and naso-orbital defects; correlation between mass, external bone defect, and exit pathway of herniation). Given these observations plus current knowledge available in the medical literature, a new classification system was developed that covers phenotypes and severity of the disease. The "FEEM classification" is an alphanumeric system based on facial deformities, external bone defect, exit pathway of herniation, and malformation of brain. It was tested in 42 patients for usability and validity. When combined with a newly designed "FEEM diagram," relevant pathological findings can be recorded in an objective manner so that diagnosis becomes more precise and uniform and comparison of outcome is possible. It also emphasizes the fact that FEEM has a range of manifestations governed by dynamic interaction between structural defects and herniation. Each clinical entity is a final result of its own disease course (stable, progressive, or regressive FEEM), with a varying degree of communication between the external mass and the central nervous system.

Frontoethmoidal Encephalomeningocele: Surgical Correction by the Chula Technique

This study reevaluates a surgical technique known as the Chula technique, previously reported in 1991 for correction of frontoethmoidal encephalomeningocele. From 1986 to 1999, 108 patients were operated on with this technique, which could remove the herniation mass, repair dural and bone defects, reconstruct the naso-orbital area, and restore aesthetic facial appearance in a single stage. Formal frontal craniotomy was not necessary. The result has been very satisfying in terms of safety, cure rate, and aesthetic outcome. Spontaneous improvement of lacrimal passage obstruction occurred in 85.2 percent of cases, and dacryocystorhinostomy was required in the rest. There was no mortality. Complications (e.g., wound infection, 6.5 percent; wire extrusion, 3.7 percent; meningitis, 2.8 percent; cerebrospinal fluid leakage, 2.8 percent; and postoperative increased intracranial pressure, 2.8 percent) were much less frequent than in other reports. With a mean follow-up period of 439 days (maximum, 12 years), there has been no recurrence.