Aplasia cutis congénita con encefalocele: a propósito de un caso clínico / Congenital aplasia cutis with encephalocele: clinical case study / Aplasia cútis congénita com encefalocele: relato de caso

La aplasia cutis es una rara alteración congénita caracterizada por la ausencia de piel, pudiendo llegar a estructuras más profundas: músculo, hueso y duramadre, como en el presente caso. Se localiza más frecuentemente en el cuero cabelludo, donde se asocia a un defecto óseo en el 20% de los casos. Recién nacido de sexo femenino, término, adecuado para la edad gestacional, vigoroso. Con diagnóstico prenatal a las 36 semanas de edad gestacional de encefalocele. Constatándose al nacimiento microcefalia, hipoplasia ósea y cutánea, encefalocele en línea media de cráneo. Se realizó manejo por equipo multidisciplinario, se practicaron varias intervenciones quirúrgicas, con buena evolución.

Aplasia Cutis is a rare congenital condition, defined by the absence of skin in a particular body region, it can also compromise muscle, bone and dura mater as shown in this case. It is mostly located on the scalp, where it is associated with a bone defect in 20% of cases. We will discuss the case of a female newborn, term gestation, vigorous at birth, with prenatal diagnosis of encephalocele at 36 weeks of gestational age. We observed microcephaly, bone and skin hypoplasia, encephalocele in the midline of the skull at birth. Several surgical interventions were carried out and the follow-up was made by a multidisciplinary team, with good evolution.

A Aplasia Cútis é uma alteração congênita rara, caracterizada pela ausência de pele, podendo atingir estruturas mais profundas: muscular, óssea e dura-máter, como neste caso. Localiza-se mais frequentemente no couro cabeludo, onde está associada a um defeito ósseo em 20% dos casos. É apresentado caso de recém-nascida do sexo feminino, a termo, adequada para idade gestacional, vigorosa. Com diagnóstico pré-natal às 36 semanas de idade gestacional de Encefalocele. Microcefalia, hipoplasia óssea e cutânea e encefalocele na linha média do crânio foram confirmadas ao nascimento. O manejo foi realizado por equipe multidisciplinar, diversas intervenções cirúrgicas, com boa evolução.

Delayed Posttraumatic Blepharocele: A Rare Case Report with Review of the Literature / Blefarocele Pós-traumática Tardia: Relato de caso raro com revisão da literatura

Though posttraumatic cerebrospinal fluid (CSF) rhinorrhea and otorrhea are fairly common, blepharocele and blepharoencephalocele are rare, with only 15 cases reported to date. A 29-year-old female patient presented with a complaint of swelling of the right eyelid that had begun three months before. The patient had sustained a head injury 24 years prior to presentation. Imaging studies revealed the presence of a craniopalpebral CSF fistula. The patient underwent successful surgical repair of the fistula with craniotomy and duroplasty by autologous fascia lata graft. Delayed development of blepharocele 24 years after trauma is unusual, and, to our knowledge, the case herein reported is the first one in the literature.

Embora a rinorreia e a otorreia pós-traumática do líquido cefalorraquidiano (LCR) sejam bastante comuns, a blefarocele e a blefaroencefalocele são raras, com apenas 15 casos relatados até o momento. Paciente do sexo feminino, 29 anos, apresentou queixa de edema em pálpebra direita com início há três meses. O paciente havia sofrido um traumatismo cranioencefálico 24 anos antes da apresentação. Os exames de imagem revelaram a presença de fístula liquórica craniopalpebral. O paciente foi submetido com sucesso ao reparo cirúrgico da fístula com craniotomia e duroplastia com enxerto autólogo de fáscia lata. O atraso no desenvolvimento de blefarocele 24 anos após o trauma é incomum e, até onde sabemos, o caso aqui relatado é o primeiro na literatura.

Surgical effect of skull defect in children / 中华神经医学杂志

Objective:To explore the clinical features, surgical treatments and treatment effects of children with skull defect.Methods:Sixty children with skull defect, admitted to our hospital from January 2010 to December 2020, were chosen in our study. These children were divided into encephalocele group ( n=28) and non-encephalocele group ( n=32) according to the imaging results (whether brain tissues were 1.5 cm higher than the bone window plane or not). The time and area of skull defect were compared between the two groups. Titanium mesh or polyether ether ketone material were used to repair the skull defect; 24 children without nerve fiber bundle distribution from encephalocele group underwent resection of the encephalocele tissues additionally. All children were followed up for 3-10 years in the outpatient department, and the prognoses of children from the two groups were evaluated by Glasgow Outcome Scale (GOS) one year after surgery. Results:As compared with the non-encephalocele group, children in the encephalocele group had significantly younger age accepted skull removal, significantly longer skull defect course, significantly higher incidence of epilepsy, significantly more common secondary changes in the brain tissues around the defect, but statistically smaller skull defect area ( P<0.05). There was no bleeding, severe edema, wound infection or cerebrospinal fluid leakage after surgery in both groups, and primary healing was achieved. In the encephalocele group, 16 children were complicated with epilepsy; 10 got complete seizure control, and 6 got seizure improvement. In the non-encephalocele group, 8 children were complicated with epilepsy; 6 got complete seizure control, and 2 got seizure improvement. Postoperative follow-up showed that GOS scores in the non-encephalocele group were significantly higher than those in encephalocele group ( P<0.05). Conclusion:As ompared with skull defect children without encephalocele, skull defect children with encephalocele have earlier defect age, longer course of disease, higher incidences of ventricular perforation malformation and epilepsy, and a relatively poorer prognosis.

Encefalocele en paciente con síndrome de Hurler: descripción y revisión de alternativas terapéuticas / Encephalocele in a patient with Hurler syndrome: description and therapeutic alternatives review

Introducción: el síndrome de Hurler es la forma más grave de la mucopolisacaridosis I. El depósito de heparán y dermatán sulfato en las meninges favorece el desarrollo de hidrocefalia y, a su vez, de meningoencefaloceles. Caso clínico: se describe el caso de una paciente de 23 años con este síndrome y un encefalocele nasosinusal intervenido mediante cirugía endoscópica nasosinusal y posterior refuerzo mediante un colgajo pericraneal. Discusión: el abordaje endoscópico de los meningoencefaloceles nasales ha crecido notablemente en los últimos años debido a una tasa de éxito elevada tanto para la eliminación de dicha lesión, como para el adecuado control de la fístula de líquido cefalorraquídeo consecuente. Conclusiones: actualmente, la cirugía endoscópica nasosinusal es una herramienta muy útil para el tratamiento de patologías de base de cráneo y reduce la morbilidad causada por el abordaje transcraneal.

Introduction: Hurler syndrome is the most severe version of mucopolysaccharidosis I. The storage of dermatan and heparin sulfate in meninges allows the development of hydrocephalus and meningoencephaloceles. Case report: We report a 23-year-old female with this syndrome and a sinonasal encephalocele operated by endoscopic sinonasal surgery and subsequent pericranial flap as support. Discussion: Endoscopic sinonasal surgery has grown in last years in relation with treatment of sinonasal meningoencephaloceles due to a high rate of success removing the lesion and closing the subsequent cerebrospinal fluid fistula. Conclusion: Currently, the endoscopic sinonasal surgery has become a useful tool in the management of skull base pathologies, and reduces the morbidity due to a transcranial approach.

Individual variation of tentorial notch morphometry in a series of neurocritical patients / Variación individual de la morfometría de la hendidura tentorial en una serie de pacientes neurocríticos

Background: Cadaveric studies on humans have shown anatomical variabilities in the morphometric characteristics of the tentorial notch. These anatomical variations could influence the worsening of neurocritical patients. Objectives: 1) To investigate the morphometric characteristics of the tentorial notch in neurocritical patients using computed tomography (CT); 2) To investigate the correlation between tentorial notch measurements by CT and by magnetic resonance imaging (MRI); and 3) To analyze the individual variability of the tentorial notch anatomy seen in neurocritical patients. Methods: Prospective series of neurocritical patients was examined. An imaging protocol for measurements was designed for CT and MRI. The level of the agreement of the measurements from CT and MR images was established. According to the measurements found, patients were divided into different types of tentorial notch. Results: We studied 34 neurocritical patients by CT and MRI. Measurements of the tentorial notch via CT and MRI showed significant agreement: concordance correlation coefficient of 0.96 for notch length and 0.85 for maximum width of tentorial notch. Classification of tentorial notch measurements according to the criteria established by Adler and Milhorat, we found the following: 15 patients (58%) corresponded to a "short" subtype; 7 (21%) to "small"; 3 (9%) to "narrow"; 2 (6%) to "wide"; 2 (6%) to "large"; 1 (3%) to "long"; and 4 (12%) to "typical". Conclusions: The anatomical variability of the tentorial notch could be detected in vivo by means of CT scan and MRI. Good agreement between the measurements made using these two imaging methods was found.

Antecedentes: Estudios cadavéricos en humanos han mostrado variabilidad anatómica en las características morfométricas de la hendidura tentorial (HT). Estas variaciones anatómicas podrían influir en el neurodeterioro agudo de los pacientes neurocríticos. Objetivos: 1) Investigar las características morfométricas de la HT en pacientes neurocríticos mediante tomografía computarizada (TC); 2) Investigar la correlación de las mediciones de la HT realizadas por TC y resonancia magnética (RM); 3) Analizar la variabilidad individual de la anatomía de la HT observada en pacientes neurocríticos. Métodos: Se examinó una serie prospectiva de pacientes neurocríticos. Se diseñó un protocolo de imágenes para mediciones por TC y RM. Se estableció la concordancia de las mediciones realizadas mediante TC y RM. Según las mediciones encontradas, los pacientes se dividieron en diferentes tipos de HT. Resultados: Estudiamos 34 pacientes neurocríticos por TC y RM. Las mediciones de la HT por TC y RM mostraron una concordancia significativa: coeficiente de correlación de concordancia de 0,96 para la longitud de la HT y 0,85 para el ancho máximo de la HT. Clasificando las medidas de la HT de acuerdo con los criterios establecidos por Adler y Milhorat, encontramos: 15 pacientes (58%) correspondieron al subtipo "corto", 7 (21%) al "pequeño", 3 (9%) al "estrecho" ", 2 (6%) a "ancho ", 2 (6%) al "grande ", 1 (3%) al "largo" y 4 (12%) al "típico". Conclusiones: Se pudo detectar variabilidad anatómica de la HT in vivo, mediante TC y RM. Se encontró una buena concordancia en las medidas obtenidas con ambos métodos imagenológicos.

Encefalocele occipital gigante sin complicaciones neonatales inmediatas / Giant occipital encephalocele without immediate neonatal complications

RESUMEN El encefalocele se produce por un defecto del tubo neural debido a una insuficiencia de la superficie ectodérmica de separarse del neuroectodermo. Este tipo de defecto incluye una etiología multifactorial. Se presentó el caso de una paciente de 43 años, raza indígena, con historia obstétrica de 14 embarazos (13 partos y 1 aborto espontáneo). La paciente acudió con dolor al servicio de urgencia del Centro de atención integral materno-infantil de Ixchiguán, San Marcos, Guatemala (fue atendida como parte de la colaboración médica cubana). En la cesárea se obtuvo un recién nacido con encefalocele occipital, sin complicaciones neonatales inmediatas. El pronóstico para los pacientes con este tipo de malformación congénita es variable, pues depende, en primer lugar, de la localización y el tamaño, del tipo de tejido cerebral herniado, y por otro lado, del número, tipo y gravedad de las malformaciones asociadas.

ABSTRACT Encephalocele occurs from a neural tube defect due to a failure of the ectodermal surface to separate from the neuroectoderm. This type of defect includes a multifactorial etiology. We present a 43-year-old female indigenous patient, with an obstetric history of 14 pregnancies (13 deliveries and 1 spontaneous abortion). She came with pain to the emergency service of the Comprehensive Maternal and Child Care Center of Ixchiguán, San Marcos, Guatemala and was treated as part of the Cuban medical collaboration. A neonate with occipital encephalocele, without immediate neonatal complications, was born by caesarean section. Patient prognosis with this type of congenital malformation is variable, since it depends, firstly, on its location and size, type of herniated brain tissue, and on the other hand, on the number, type and severity of the associated malformations.

Caso Clínico: Meningohidroencefalocele Gigante / Case Report: Giant Meningohydroencephalocele

INTRODUCCIÓN: Los encefaloceles son una anomalía congénita rara, que afecta a 1 de cada 5 000 nacidos vivos. Un meningohidroencefalocele es la herniación de meninges, ventrículos y parte del encéfalo a través de un defecto congénito en el cráneo. El pronóstico de los recién nacidos con encefalocele depende de varios factores. La corta edad y las anomalías congénitas asociadas en estos pacientes plantean desafíos en el diagnóstico y manejo. CASO CLÍNICO: Recién nacido masculino, nacido a las 37 semanas de gestación, por cesárea de emergencia por parto en fase latente, presentado una masa de gran tamaño, sobre la región parieto-occipital, de bordes lobulados, irregulares, cubierta en parte por cuero cabelludo, y en otras zonas de un tono rojo violáceo. El neonato fue poco reactivo, con tono disminuido, llanto ausente. EVOLUCIÓN: Tras valoración del caso por especialistas, debido al importante defecto de la calota craneal y las anomalías estructurales cerebrales, el paciente no fue candidato para tratamiento quirúrgico. Neonato fue dado de alta con expectativa de vida reducida, para recibir cuidados en el hogar. CONCLUSIÓN: El meningohidroencefalocele gigante es un defecto del tubo neural poco frecuente. El manejo de las encefaloceles puede ser complicado y debe ser individualizado y dependerá de las estructuras anatómicas comprometidas. Si bien en este caso el pronóstico fue malo, siempre es necesario el trabajo multidisciplinario para un adecuado manejo.

BACKGROUND: Encephaloceles are a rare congenital anomaly, affecting 1 in 5 000 live births.A meningohydroencephalocele is the herniation of the meninges, ventricles, and part of the brain through a congenital defect in the skull. The prognosis of newborns depends on several factors. The young age and the associated congenital anomalies in these patients contribute to the diagnostic and treatment challenge. CASE REPORT: Male newborn, born at 37 weeks of gestation, by emergency C-section due to latent labor, presenting a large mass over the parieto-occipital region, with lobed, irregular edges, partially covered by the scalp, and in other areas a purplish red tone. The newborn was no very reactive, had decreased tone, absent crying. EVOLUTION: After specialist's evaluation, due to the significant defect in the cranial shell and the structural brain abnormalities, the patient was not a candidate for surgical treatment. The newborn was discharged with a reduced life expectancy, to receive care at home. CONCLUSION: giant meningohydroencephalocele is a rare neural tube defect. The management of encephaloceles can be complicated and must be individualized, and will depend on the anatomical structures involved. Although in this case the prognosis was bad, multidisciplinary work is always necessary for proper management.

Role of pre-suture craniotomy combined with controlled decompression under intracranial pressure monitoring in surgery for posttraumatic acute diffuse brain swelling / 中华神经医学杂志

Objective:To investigate the application value of pre-suture craniotomy combined with intracranial pressure monitoring in surgery for posttraumatic acute diffuse brain swelling (PADBS).Methods:One hundred and fifty-seven patients with PADBS admitted to our hospital from February 2015 to December 2019 were chosen in our study; 68 patients (control group), admitted to our hospital from February 2015 to June 2017, underwent controlled decompression under intracranial pressure monitoring; and 89 patients (treatment group), admitted to our hospital from June 2017 to December 2019, were performed pre-suture craniotomy combined with controlled decompression under intracranial pressure monitoring. The craniotomy time, brain tissue exposure time, cranial closure time, incidence of acute encephalocele, and Glasgow outcome scale (GOS) scores at 6 months after injury were retrospectively analyzed and compared between the two groups.Results:As compared with those in the control group, the patients in the treatment group had significantly longer intraoperative craniotomy time ([19.2±1.6] min vs. [15.4±1.4] min), significantly shorter exposure time of brain tissues ([18.5±2.4] min vs. [26.3±2.2] min), significantly shorter time of cranial closure ([11.2±1.5] min vs. [18.3±2.1] min), and statistically lower incidence of acute encephalocele (22.5% vs. 38.2%), P<0.05). The good prognosis rate of the treatment group (70.8%) was significantly higher than that of the control group (50.0%), and the mortality rate (6.7%) was statistically lower than that of the control group (17.6%, P<0.05). Conclusion:Pre-suture craniotomy combined with controlled decompression under intracranial pressure monitoring can shorten the time of cranial closure and brain tissue exposure, reduce the incidence of acute encephalocele, and ultimately improve the prognosis of patients with posttraumatic acute diffuse brain swelling.

Clinical value of ventricular intracranial pressure monitoring in gradient decompression for patients with traumatic cerebral hernia / 中华神经医学杂志

Objective:To investigate the clinical value of ventricular intracranial pressure monitoring (V-ICPM) in gradient decompression for patients with traumatic cerebral hernia.Methods:The clinical data of 103 patients with traumatic cerebral hernia admitted to our hospital from October 2016 to October 2020 were retrospectively analyzed. These patients were divided into observation group ( n=49) and control group ( n=54) according to whether V-ICPM was applied. Patients in the observation group accepted V-ICPM before gradient decompression, and patients in the control group accepted gradient decompression directly. Incidence of malignant encephalocele, clinical short-term and long-term efficacies, and complications were compared between the two groups. According to intracranial pressure (ICP), the patients were divided into normal or slightly increased ICP (≤22 mmHg), moderate increased ICP (23-40 mmHg) and severe increased ICP (>40 mmHg); the relationship between ICP and prognoses was analyzed in the observation group. Results:(1) The incidences of intraoperative malignant encephalocele in the observation group (16.33%) were slightly lower than that in the control group (29.63%), without significant difference ( P>0.05). Twenty four h after gradient decompression, pupils recovered in 35 patients (71.43%) from the observation group and 28 patients (51.85%) from the control group, significant difference in the pupils recovery rate was noted between the two groups ( χ2=4.145, P=0.042); the Glasgow Coma Scale (GCS) scores between the observation group (8.43±2.56) and control group (7.39±2.47) showed statistical differences ( t=-2.095, P=0.039). Three months after gradient decompression, there were 7 patients with Glasgow Outcome Scale (GOS) scores of 5, 18 patients with scores of 4, 10 patients with scores of 3, 8 patients with scores of 2, and 6 patients with score of 1 in the observation group; there were 12 patients with GOS scores of 5, 17 patients with scores of 4, 12 patients with scores of 3, 7 patients with scores of 2, and 6 patients with score of 1 in the control group; the difference was not statistically significant ( Z=-0.681, P=0.496). (2) The higher the ICP in the observation group (initially and when the dura mater is cut), the worse the prognosis. Conclusion:The application of V-ICPM before gradient decompression cannot further improve the long-term prognosis of the patients, but it can provide intraoperative reference and prognosis prediction for the operators.

Adult Nasoethmoidal Encephalocele Corrected by Supraorbitary Approach: Case Report and Literature Review

Encephalocele is a protrusion of the central nervous system elements through a defect in the dura mater and in the cranium. The prevalence of encephalocele ranges from 0.08 to 0.5 per 1,000 births. The posterior encephaloceles are more common in North America and Europe, while frontal defect is frequently found in Asia. The present paper describes a 26-year-old male patient presenting with cerebrospinal fluid leak and meningitis symptoms. He was diagnosed with congenital nasoethmoidal encephalocele and treated surgically using a supraorbital approach without complications.

Meckel-Gruber syndrome: a rare and lethal foetal anomaly

Meckel-Gruber syndrome (MGS) is a rare and lethal autosomal recessive disorder characterized by occipital encephalocele, postaxial polydactyly and bilateral dysplastic cystic kidneys. It can be associated with many other congenital malformations. The incidence of Meckel-Gruber syndrome ranges between 1 in 13,000 to 4,00,000 live births. Antenatal ultrasound examination establishes the diagnosis by identifying at least two of the major features. A case is presented that describes a baby with ambiguous genitalia, who presented with the triad of Meckel-Gruber syndrome. The baby died shortly after birth.

Recent advance in interaction between acute subdural hematoma and disorder of intracranial superficial venous circulation / 中华神经医学杂志

Traumatic acute subdural hematoma (ASDH) is often severe,and bridging vein rupture is one of the case mechanisms of ASDH.After traumatic ASDH,venous reflux disorder,cerebral ischemia,delayed bleeding in other parts of the brain,and intraoperative encephalocele are prone to occur.This article reviews the occurrence and development of ASDH and its relation with intracranial venous circulation.

Case report of anaesthetic management of neonate with large occipital encephalocele

In the present case report, endotracheal intubation in a case of large occipital encephalocele presented for surgical excision was performed. As the management of airway even in a neonate is different and complex as compared to airway of old child and that of adult. It was challenging because of the difficulty in securing airway because of prone position in occipital encephalocele. The major concerns during the operation were to avoid premature rupture of the encephalocoele and to manage a possible difficult airway due to restricted neck movement and inability to achieve optimal position for intubation of the trachea. Continuous monitoring of patient was done during the surgery.

Dermoid cyst with cerebellar meningoencephalocele at different locations accompanied by posterior fossa abnormalities: case report

ABSTRACT CONTEXT: Dermoid cysts are well-defined cysts containing sebaceous glands and dermal structures. In the literature, dermoid cysts and associated closure defects have been described in the same locations. CASE REPORT: In this case, a dermoid cyst was found at the base of the mouth with a coexisting closure defect in the occipital calvarium. Additional abnormalities were also observed, including posterior myeloschisis, right cerebellar dysgenesis, vermian hypogenesis and posterior fusion of the second and third vertebrae. The finding of a dermoid cyst located at the base of the mouth is discussed here, with additional imaging findings. CONCLUSION: Dermoid cysts in the head and neck region may be accompanied by posterior fossa abnormalities.

Advances in diagnosis and treatment of syndrome of trephined and paradoxical herniation after decompressive craniectomy / 中华创伤杂志

The efficacy of decompressive craniectomy (DC) in reducing traumatic brain injury mortality has been affirmed,but there are also many serious complications.Syndrome of the trephined (ST) and paradoxical herniation (PH) are rare complications.ST is characterized by a series of neurological deterioration due to skin flaps subsidence from weeks to months after DC.These neurological impairments are closely related to the subsequent repair of skull defects.PH shows progressive decrease in consciousness on the basis of ST,changes in pupils on the side of skull defects,low touch pressure at the defect window,and obvious midline shift and brain stem compression on CT.ST and PH have common inducements in pathophysiology,including cerebrospinal fluid dynamics,atmospheric pressure,cerebral blood flow and brain material metabolism.There is no consensus on the diagnosis of ST and PH,and early cranioplasty is suggested in terms of treatment.This article reviews the clinical manifestations,pathophysiological changes,diagnosis and treatment of ST and PH after DC operation,so as to provide references for clinicians to further understand ST and PH.

Mechanism and management strategy of intraoperative acute encephalocele in traumatic brain injury / 中华神经医学杂志

The treatment of brain swelling in acute traumatic brain injury is extremely difficult, and its prognosis is closely related to early diagnosis and treatment. This paper reviews the research on related mechanisms and strategies of preventing disposals to provide guidance and references for clinical workers.

Encephalocele presenting as lower lid swelling: A rare case report

Encephalocele is a rare congenital abnormality characterized by abnormal protrusion of brain and meninges through an opening in the skull. We report an 8-year-old girl who presented with a swelling in the right lower lid for the last 6 years. In her infancy, she had undergone surgery for a very small swelling located in the right nasolacrimal area. On further clinicoradiological evaluation, anterior encephalocele was diagnosed. This case highlights the uncommon site of anterior encephalocele; misdiagnosis and mismanagement of which could result in dreaded complications such as meningitis and cerebrospinal fluid leaking fistula formation.

Modified two flap palatoplasty in asymptomatic transsphenoidal encephalocele: a case report

About one-third of patients with transsphenoidal basal encephaloceles have associated congenital anomalies, including cleft palate. Moreover, they are often plagued by symptomatic exacerbations in the form of upper respiratory obstructions, cerebrospinal fluid leaks, meningitis, etc., with few patients being asymptomatic. We herein present a rare asymptomatic case of transsphenoidal basal encephalocele in an 18-month-old child with cleft palate and highlight a modified version of two-flap palatoplasty.

Transpalatine Route for the Correction of Transsphenoidal Encephalocele in a Child: A Case Report and Literature Review

Introduction Transsphenoidal encephalocele is a rare neural tube defect characterized by the herniation of meninges and eventually of parts of the brain through a bony defect in the sphenoid bone. The clinical presentation is variable, and surgical treatment is controversial. Case Report This report describes the case of an 8-month-old female child diagnosed with transsphenoidal encephalocele. The child presented with obstruction of the upper airways and was fed via a nasogastric tube but did not present changes in the hypothalamic-pituitary-axis. The patient underwent surgery with the transsphenoidal­transpalatine route, with an excellent outcome and without fistulas or infections. Conclusions Although transsphenoidal encephalocele is a rare congenital anomaly, the transsphenoidal­transpalatine route for the correction of this type of encephalocele is a safe option and produces a favorable outcome in pediatric patients.

Introdução A encefalocele transesfenoidal é um defeito do tubo neural raro, caracterizado por herniação de meninges e eventualmente partes do cérebro através de uma falha óssea no osso esfenoide. A apresentação clínica é variável e o tratamento cirúrgico é controverso. Relato de Caso No presente trabalho descrevemos o caso de uma criança de 8 meses, sexo feminino, com diagnóstico de encefalocele transesfenoidal. Apresentava-se com obstrução de vias aéreas superiores, alimentando-se por sonda nasoenteral e sem alterações do eixo hipotálamo-hipofisário. Tratada com cirurgia via transesfenoidal transpalatal, evoluiu com excelente resultado, sem fístulas e sem infecções. Conclusões Embora a encefalocele transesfonoidal seja uma anomalia congênita rara, a via transpalatina-transesfenoidal para correção deste tipo de encefalocele é uma opção segura e produz um resultado favorável no paciente pediátrico.

Surgical correction of grade III hypertelorism

Orbital hypertelorism is an increased distance between the bony orbits and can be caused by frontonasal malformations, craniofacial clefts, frontoethmoidal encephaloceles, glial tumors or dermoid cysts of the root of the nose, and various syndromic or chromosomal disorders. We report a series of 7 cases of hypertelorism that were treated in our hospital. The underlying causes in our series were craniofacial clefts 0 to 14 (4 cases), craniofacial clefts 1 to 12 (1 case), and frontonasal encephalocele (2 cases), all congenital. Surgical techniques used to correct the deformity were box osteotomy and medial wall osteotomy with or without calvarial and rib grafts. A few of our cases were reoperations with specific challenges.