Current management of congenital anterior cranial base encephaloceles.

OBJECTIVES: Congenital encephaloceles provide unique diagnostic and reconstructive challenges for the pediatric rhinologist. The objectives of the current study were to evaluate contemporary treatment strategies for congenital encephaloceles focusing on presentation, surgical technique, and outcomes. METHODS: Multi-institutional retrospective chart review of congenital encephaloceles (2003-2019). Data regarding demographics, presenting symptoms, associated abnormalities, surgical technique, size, location, and complications were collected. RESULTS: Fourteen patients with 15 congenital encephaloceles were treated using endoscopic techniques (avg 6.0 years, range 2 months-22 years) with mean follow up of 23 months. The majority presented with nasal obstruction (n = 13); only one child had cerebrospinal fluid (CSF) rhinorrhea. Associated anomalies included nasal deformities, congenital hypopituitarism, and Morning Glory syndrome. Average encephalocele size was 2.44 cm (range 0.5-3.6 cm) with mean skull base defect size of 8.6 x 7.7 mm. Locations included the foramen cecum (n = 9), central sphenoid (n = 3), midline anterior cranial fossa (n = 1), orbital plate of frontal bone (n = 1), and ethmoid roof (n = 1). Because of favorable expansion from encephaloceles, it was unnecessary to postpone surgeries to allow nasal cavity growth. Three individuals had prior operations, including surgeries for "nasal polyp" or "adenoid cyst". Two patients had post-operative complications (meningitis and CSF leak) effectively treated with no further sequelae. CONCLUSIONS: In the current study, congenital encephaloceles in children as young as 2 months were successfully repaired using endoscopic techniques. Endoscopic approaches remain a safe and effective intervention for management of these lesions.

Meckel-Gruber Syndrome: A Case Who Lived for 5 Months.

The Meckel-Gruber syndrome is a rare, congenital, and lethal malformation characterized by typical manifestations such as encephalocele, polycystic kidneys, and polydactyly. Herein, we present a case of a patient with the typical triad as well as facial, ocular, liver, and genital abnormalities who lived for almost 5 months.

Frontoethmoidal encephalocele. Report of a case / Encefalocele frontoetmoidal. A propósito de un caso

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

Los encefaloceles son infrecuentes en los países occidentales y su localización más frecuente es occipital. Los encefaloceles frontales pueden afectar hueso etmoidal, frontal y/o órbitas. La reparación quirúrgica es compleja y habitualmente precisa de un abordaje multidisciplinar. El objetivo de la cirugía es reconstruir la anatomía del paciente con un buen resultado estético, y evitar la fístula de líquido cefalorraquídeo. Se presenta un caso de un gran encefalocele frontoetmoidal. El encefalocele fue reparado y la reconstrucción ósea se realizó con hueso autólogo de la capota craneal. El cierre y el resultado cosmético fueron buenos y la única complicación fue una infección posquirúrgica. Se describe la técnica y se revisa la literatura publicada al respecto

Sublabial transsphenoidal microsurgical technique to treat congenital transsphenoidal encephalocele: a technical note.

Encephalocele is a rare malformation consisting in herniation of cranial contents through a cranial defect. A transsphenoidal location is uncommon, representing 5% of all basal encephaloceles. The surgical treatment of transsphenoidal encephaloceles is challenging. An optimal approach has not yet been determined, and it varies according to the surgical experience. We report the surgical management of a transsphenoidal encephalocele. The encephalocele and the sellar defect were repaired through a sublabial transsphenoidal microsurgical approach (TSM). Preoperative magnetic resonance imaging (MRI) and computed tomography (CT) scans were crucial for surgical planning. The sublabial transsphenoidal microsurgical approach offered a good and complete exposure of both the sac and the bone defect. Therefore, the congenital defect was successfully repaired with complete resolution of the encephalocele without any surgical or medical complications. Postoperative CT scan and MRI showed the restoration of the bone defect and the recovery of a normal anatomy with herniated structures pushed back into the sella. The described sublabial transsphenoidal microsurgical approach represents a minimally invasive, safe, and effective treatment strategy for transsphenoidal encephalocele.

Endoscopic management of paediatric meningoencephaloceles: a case series.

INTRODUCTION: Congenital meningoencephaloceles represent a rare clinical entity, with its frequency being around 1 out of 4000-5000 live births. They usually present as a midline mass and the differential diagnosis includes dermoids, encephaloceles, and gliomas. Although the standard coronal approach with frontal craniotomy and pericranial flap has been considered the preferred method for several years, it is associated with risks and prolonged hospitalization. The endoscopic procedure is gaining ground and we are aiming to present our experience from five cases that were treated endoscopically. MATERIALS AND METHODS: Five cases of paediatric meningoencephaloceles (age ranging from 2.5 to 10 years) were treated in our department between the years 2007-2017. Four children had a congenital and one child a post-traumatic meningoencephalocele. The presenting symptoms included nasal obstruction, meningitis and CSF leak. All patients had pre-operative imaging with CT and MRI scans and the preferred method of repair was multi-layered closure. RESULTS: All our patients had uneventful recovery, without any post-operative complications. The follow-up period ranges from 39 to 98 months. All five patients remain asymptomatic and recurrence-free. CONCLUSION: Endoscopic management of paediatric meningoencephaloceles is a reliable and safe approach and it is known to have no adverse effects on facial growth. It carries a satisfactory success rate, reduces the in-hospital stay with lower cost of treatment, better cosmesis and lower morbidity than external procedures. It requires multidisciplinary team setting in a tertiary centre with experienced endoscopic anterior skull base surgeons. Pre-operative assessment and post-operative care are of paramount importance and larger series need to be studied to reach safe conclusions and establish management guidelines.

Internasal: A New Type of Frontoethmoidal Encephalomeningocele.

IMPORTANCE: 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. OBJECTIVE: The aim of our study was to present a newly discovered internasal type (IN) of FEEM. DESIGN, SETTING, PARTICIPANTS: 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. FINDINGS: 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. CONCLUSIONS AND RELEVANCE: 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.

Tumores nasales congénitos de la linea media / Congenital nasal tumors of the midline in ENT

Las masas nasales congénitas de la línea media se presentan con una frecuencia muy baja ­1/20.000 a 1/40.000 nacidos vivos­. Se trata de hallazgos asintomáticos en el recién nacido y son resultado de anomalías congénitas del desarrollo embrionario, que suelen aparecer como masas en la línea media nasal en un punto cualquiera entre glabela y columela. Estas tumoraciones presentan un riesgo elevado de extensión al sistema nervioso central, lo que es especialmente importante tener en cuenta para prevenir consecuencias tales como la fístula de líquido cefalorraquídeo y/o la aparición de meningitis recidivante. Existen gran cantidad de tumores nasales de la línea media que aparecen en el recién nacido o en el lactante y que constituyen diagnósticos diferenciales de las lesiones congénitas antes descriptas. Describiremos brevemente los más frecuentes según nuestra experiencia. AU

Congenital nasal masses of the midline are very rare ­ 1/20,000 to 1/40,000 live births ­. Nasal tumors are asymptomatic findings in the neonate and are caused by congenital abnormalities during fetal development, usually appearing at the nasal midline between the glabella and columella. These tumors are associated with a high risk of extension to the central nervous system; therefore, it is especially important to prevent the development of a cerebrospinal fluid fistula and/or recurrent meningitis. There is a large number of nasal tumors of the midline in neonates or infants in the differential diagnosis of the above-described congenital lesion. Here we briefly describe the most common nasal tumors seen at our department. AU

[Modern diagnosis and treatment in children with congenital basal encephalocele]. / Sovremennye metody diagnostiki i lecheniia detei s vrozhdennymi bazal'nymi cherepno-mozgovymi gryzhami.

Basal encephalocele is a rare disease that predominantly occurs in children. Its most common symptoms include nasal liquorrhea, difficulty in nasal breathing, and deformity of the naso-orbital region. MATERIAL AND METHODS: The study group included 19 patients with basal encephalocele, aged 2 months to 18 years. Ten (59%) patients were operated on through a transnasal endoscopic approach; 3 (17.5%) patients were operated on through a transcranial approach; 4 (23.5%) patients were operated on using a combined approach: the patients underwent simultaneous elimination of a cranio-orbital region deformity using the basal transcranial approach as well as hernial sac resection and hernioplasty using the transnasal endoscopic approach. Two children had no surgery due to minimal symptoms and a lack of cerebrospinal fluid leak. RESULTS: Application of the algorithms for diagnosis and treatment of encephalocele, suggested by the authors, enabled making the timely diagnose, defining the optimal surgical tactics, and achieving good treatment results. CONCLUSION: A differentiated approach to the choice of a surgical technique for basal encephalocele, the use of auto-tissues for skull base reconstruction, intraoperative and postoperative lumbar drainage, and simultaneous elimination of deformity of the fronto-naso-orbital region enable avoiding complications and achieving good functional and aesthetic results.

Giant Parietal Encephalocele with Massive Brain Herniation and Suboccipital Encephalocele in a Neonate: An Unusual Form of Double Encephalocele.

Double encephalocele is extremely rare. We present an unusual form of double encephalocele including giant supratentorial and small infratentorial encephalocele in a neonate.

Encefalocele frontoetmoidal / Frontoethmoidal encephalocele

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Encefalocele frontonasal: reporte de un caso con diagnóstico prenatal / Frontonasal encephalocele: report of a case with prenatal diagnosis

El encefalocele es un defecto congénito mayor que se caracteriza por la herniación del tejido nervioso y las meninges a través de un defecto de la bóveda craneal. Esto se debe a alteraciones del cierre del tubo neural ocurridas en la cuarta semana de gestación. El diagnóstico prenatal es posible realizarlo a través de ecografía 2D y 3D. Se presenta el caso de un neonato con encefalocele anterior, entidad poco frecuente dentro de los defectos del cierre del tubo neural. Se realizó el diagnóstico presuntivo prenatal por ecografía 2D y 3D, y se confirmó al nacimiento por los hallazgos clínicos y escanografía(AU)

The encephalocele is a major birth defect characterized by herniation of neural tissue and meninges through a defect in the skull, due to alterations of neural tube closure occurring in fourth week of pregnancy. Prenatal diagnosis is possible through 2D and 3D ultrasound. We report a case of anterior encephalocele, a rare disease among the defects of neural tube closure, with diagnosis by 3D and 2D ultrasonography, which was confirmed at birth by clinical findings and scan(AU)

Endoscopic surgery for congenital basal meningoencephaloceles in children.

Conclusions Endoscopic surgery is safe and effective for children with congenital basal meningoencephaloceles (CBMs); it provides an acceptable operative outcome with a short recovery time and fewer complications and may be considered as a primary approach. Objectives To explore the safety and effectiveness of using transnasal or transoral endoscopic surgery on children with CBMs. Methods The clinical data of eight CBMs children who underwent transnasal or transoral endoscopic surgery in a hospital from January 2011 to January 2015 were collected. The presenting symptoms, lesion locations, surgical outcomes, and complications were examined retrospectively. Results Of the eight children, five (62.5%) patients were male, and their ages ranged from 1 year and 6 months to 14 years (median of 9 years). Six patients presented with the transethmoidal sub-type, and two presented with the transsphenoidal sub-type. The average hospital stay of all patients was 8.6 ± 2.6 days. There were neither intra-operative nor post-operative complications observed in any of the cases during the follow-ups that occurred between 6-54 months (mean of 15.5 months).

Congenital nasal neuroglial heterotopia and encephaloceles: An update on current evaluation and management.

OBJECTIVES/HYPOTHESIS: To describe our experience and current management approach for congenital nasal neuroglial heterotopia (NGH) and encephaloceles. STUDY DESIGN: Retrospective chart review at a tertiary pediatric hospital from 1970 to 2013. METHODS: Thirty patients met inclusion criteria: 21 NGH and nine encephaloceles. Data including demographics, pathology, imaging modality, surgical approach, resection extent, outcomes, and complications were analyzed. RESULTS: Fourteen NGH patients (67%) presented with an internal nasal mass and nasal obstruction. Three patients (14%) presented with an external nasal mass and four (19%) had a mixed lesion. Median age at surgery was 0.51 years (interquartile range 1.32 years). Thirteen (62%) had an intranasal endoscopic approach. Median operative time was 1.6 hours (interquartile range 1.2 hours), and there were no major complications. Nine patients with encephalocele were identified: six (67%) presented with transethmoidal encephaloceles, two (22%) presented with nasoethmoidal encephaloceles, and one (11%) presented with a nasofrontal lesion. The median age at surgery was 1.25 years (interquartile range 1.4 years). All patients required a craniotomy for intracranial extension. Median operative time was 5 hours (interquartile range 1.9 hours), and eight patients (88%) had a total resection. Length of stay ranged from 3 to 14 days. CONCLUSION: Nasal neuroglial heterotopia and encephaloceles are very rare lesions that require multidisciplinary evaluation and management. At our institution, there has been a shift to magnetic resonance imaging alone for the evaluation of NGH to avoid radiation exposure. Endoscopic extracranial resection is feasible for most intranasal and mixed NGH without an increase in operative time, residual disease, or complications. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:2161-2167, 2016.