Introdução
O que você precisa saber de cara
Encefalocele é um defeito do tubo neural caracterizado por protrusões em forma de saco do cérebro e das membranas que o recobrem através de aberturas no crânio. Esses defeitos são causados pela falha do fechamento completo do tubo neural durante o desenvolvimento fetal. As encefaloceles causam uma fenda no meio do crânio, ou entre a testa e o nariz, ou na parte posterior do crânio. A gravidade da encefalocele varia, dependendo de sua localização.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
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Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
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🇧🇷 Atendimento SUS — Encefalocelo nasal
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Publicações mais relevantes
CSF Oculorrhea Presenting as "Tearing" following the Repair of Fronto-Ethmoidal Encephalocele.
Cerebrospinal fluid (CSF) oculorrhea is extremely rare, and very few cases have been reported mostly following trauma. There is only 1 case in the published literature where oculorrhea occurred following the repair of fronto-nasal encephalocele. A six-year-old girl presented with gradually increasing fronto-ethmoidal encephalocele with secondary papulo-nodular changes. She underwent bi-frontal craniotomy with excision of encephalocele sac and herniated gliotic brain followed by dural closure using peri-cranial graft. One month later, she presented again with swelling over the operative site and "tearing" from both her eyes. She was diagnosed with CSF oculorrhea. After failing conservative management, lumbar drain was inserted and kept on continuous drainage. Oculorrhea stopped with lumbar drain but restarted with its removal. Therefore, theco-peritoneal shunt was placed, following which oculorrhea stopped. She is doing well at 5 months' follow-up. CSF oculorrhea must be considered by the pediatric neurosurgeons in any patient who presents with "tearing" following the repair of an anterior encephalocele.
[Progress of the diagnosis and treatment of congenital nasal dermoid sinus cysts].
Congenital midline nasal masses are rare. Nasal dermoid sinus cysts(NDSC) are the most common type of the congenital midline nasal masses in childhood. Clinical manifestations are midline nasal cysts, fistula and intracranial attachments. Nasal encephalocele and glioma should be included in the differential diagnosis. Radiologic images are instructive. NDSC are easily misdiagnosed, leading to recurrence and surgical trauma affecting the face. Early appropriate surgical excision is recommended. This article reviews the embryology pathogenesis, progress of diagnosis and treatment of congenital NDSC.
Delayed Cerebrospinal Fluid Rhinorrhea Associated With Ethmoidal Encephalocele After Resection of Remote Meningioma.
Diagnosis and treatment of neurosurgical pathology present unique challenges in underserved areas, and many conditions may go undiagnosed, misdiagnosed, or untreated for prolonged periods. The development of an unusual complication, seemingly unrelated to an area of neurosurgical intervention, may be particularly perplexing to non-neurosurgical providers, particularly in areas where neurosurgical procedures have not historically been available. A 44-year-old male presented with a giant meningioma which was successfully resected. A nasal encephalocele was noted preoperatively but was not addressed due to lack of associated symptoms and distance from the tumor. The patient lived on a remote island and was lost to follow-up. He developed delayed cerebral spinal fluid (CSF) rhinorrhea three months after surgery, which was diagnosed and treated by local providers as allergic rhinitis for 11 months until he presented with new-onset seizure. Imaging demonstrated descent of the lateral ventricle into the encephalocele. The encephalocele was amputated and the skull base defect was repaired successfully. The alteration of ventricular anatomy and CSF fluid dynamics following tumor resection appears to have created an environment where a non-traumatic CSF leak could develop where it had previously shown no signs of developing. It may be prudent to treat skull base defects prophylactically to prevent this type of complication, particularly in patients of remote regions where regular follow-up is difficult.
Endoscopic management of congenital nasal meningoencephaloceles: a short series of 15 paediatric cases.
To retrospectively analyse surgical management of clinico-radiologically proven nasal meningoencephalocele amongst children and results of repair with single-layer septo-mucosal flap at a tertiary skull base surgery centre in north India. Fifteen children with clinic-radiological nasal meningoencephaloceles with or without CSF rhinorrhoea were included. Radiological scans included CT scans and MRI scans for all cases to delineate soft tissue and bony architecture. All children underwent endoscopic excision of encephaloceles and repair of the skull base defect. The authors were successful in fourteen cases. Successful cases included 4 children less than 6 months of age who underwent single-layer rotated septo-mucosal flaps, thereby avoiding donor site morbidity. As endoscopic excision of nasal encephalocele is an established management approach, the authors take the concept of minimally invasive surgery a step further by successfully managing these lesions with single-layer repair. Vascularized septo-mucosal flap seems to be the only determining factor for a successful repair for congenital meningoencephalocele. However, a prospective study comprising a larger subset of patients would substantiate the assumption.
Frontoethmoidal encephalocele. Report of a case.
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.
Publicações recentes
CSF Oculorrhea Presenting as "Tearing" following the Repair of Fronto-Ethmoidal Encephalocele.
[Progress of the diagnosis and treatment of congenital nasal dermoid sinus cysts].
Delayed Cerebrospinal Fluid Rhinorrhea Associated With Ethmoidal Encephalocele After Resection of Remote Meningioma.
Endoscopic management of congenital nasal meningoencephaloceles: a short series of 15 paediatric cases.
Brain Herniation Through the Cribriform Plate: Review and Comparison to Encephaloceles in the Same Region.
📚 EuropePMC14 artigos no totalmostrando 8
CSF Oculorrhea Presenting as "Tearing" following the Repair of Fronto-Ethmoidal Encephalocele.
Pediatric neurosurgery[Progress of the diagnosis and treatment of congenital nasal dermoid sinus cysts].
Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgeryDelayed Cerebrospinal Fluid Rhinorrhea Associated With Ethmoidal Encephalocele After Resection of Remote Meningioma.
CureusEndoscopic management of congenital nasal meningoencephaloceles: a short series of 15 paediatric cases.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryBrain Herniation Through the Cribriform Plate: Review and Comparison to Encephaloceles in the Same Region.
CureusFrontoethmoidal encephalocele. Report of a case.
NeurocirugiaSecondary encephalocele in infant following subdural empyema repaired endoscopically-A case report.
International journal of pediatric otorhinolaryngologyCombined transnasal and transoral endoscopic approach to a transsphenoidal encephalocele in an infant.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryAssociações
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- CSF Oculorrhea Presenting as "Tearing" following the Repair of Fronto-Ethmoidal Encephalocele.
- [Progress of the diagnosis and treatment of congenital nasal dermoid sinus cysts].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery· 2021· PMID 33541005mais citado
- Delayed Cerebrospinal Fluid Rhinorrhea Associated With Ethmoidal Encephalocele After Resection of Remote Meningioma.
- Endoscopic management of congenital nasal meningoencephaloceles: a short series of 15 paediatric cases.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery· 2020· PMID 32382868mais citado
- Frontoethmoidal encephalocele. Report of a case.
- Brain Herniation Through the Cribriform Plate: Review and Comparison to Encephaloceles in the Same Region.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:141118(Orphanet)
- MONDO:0015394(MONDO)
- GARD:19951(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55785445(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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