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We report the case of a 4-year-old girl who presented spontaneous watery discharge through the left nostril due to a basal encephalocele. Two infrequent operative findings were the presence of an excavation of the cribriform plate, and not of a true osseous defect, and the absence of the left olfactory bulb.
The author presents a case of Encephalocele fronto-ethmoïdal in a 9 year old girl without hereditan or personal antecedent history. Surgery was performed the hernial sac was cut and the neck ligated; a piece of fascia of the temporal muscle was used as a graft. The patient was seen two years later with good result.
We have studied 15 occipital, 3 frontal and one parietal cases of encephaloceles from the point of view of the etiopathogenetic, clinic and especially diagnostic problem. The diagnosis of M.E. is based on: 1) the x-ray demonstration of the bone-gap by particular projections and by tomography; 2) in the frontal M.E. the presence of face deformity, with hipertelorism and widening of nose bridge and the finding of ectopic tissue in the nose or in the oral cavity in presence of palatoschisis. Cerebral angiography and pneumoencephalography, made by tomography, are useful to show the herniated brain, hydrocephalus and other associated anomalies. Finally we think computerized tomography (C.T.) is very important for the diagnosis, since it shows the herniation, the bone-gap and the kind of herniated tissue.
Meningoencephalocele is an uncommon condition in which brain tissue, meninges, or both protrude through a defect in the anterior cranial fossa and into the ethmoid sinus or nasal cavity. Much less often, brain tissue, meninges, or both protrude through a defect in the middle cranial fossa and into the sphenoid sinus. We report an unusual case of a middle fossa encephalocele that appeared as a lytic lesion of the skull base. The patient was treated successfully via a unique endoscopic transpterygoid approach--that is, an endoscopic approach through the maxillary sinus and pterygopalatine fossa and into the pterygoid process.
BACKGROUND: With the introduction and subsequent widespread acceptance of endoscopic surgery, otolaryngologists are increasingly being called on to care for patients with cerebrospinal fluid rhinorrhea and meningoencephaloceles. Patients with large encephaloceles and skull base defects present a special challenge. We present our experience with cranial bone grafts in treating this important entity. METHODS: Our clinical experience was reviewed from 1998 to 2001. Review parameters included defect size, cranial bone graft harvest site and size, and graft appearance on postoperative follow-up. RESULTS: Results revealed that 20 patients underwent defect repair with cranial bone graft. The average defect was approximately 0.92 x 0.7 cm; nine defects were located in the ethmoid roof, eight defects were in the sphenoid, and three defects were in the posterior table of the frontal sinus. Donor sites included 2 parietal, 3 frontal, and 15 temporal (mastoid). Grafts healed well and all defects remained closed on endoscopic and computerized tomographic follow-up. All donor sites healed well. CONCLUSION: Our experience indicates that cranial bone graft is an excellent material for endoscopic reconstruction of skull base defects. It confers special advantages in large defects, in defects with complex three-dimensional characteristics, and in patients with cerebrospinal fluid leaks associated with an elevated intracranial pressure.
The objective of the present study was to determine the presence of risk factors for the occurrence of neural tube defects. Data for 33,535 births which occurred at Hospital do Servidor Público Estadual de São Paulo from July 1973 to December 1986 were collected in a prospective manner as recommended by "Estudo Colaborativo Latino-Americano de Malformações Congênitas" (ECLAMC, Collaborative Latin American Study on Congenital Malformations). Twenty-six cases of neural tube defects were detected (0.77/1000 births). Of these, 11 were cases of spina bifida (0.39/1000 births), 9 of anencephaly (0.27/1000 births) and 6 of encephalocele (0.18/1000 births). We observed a higher frequency of polyhydramnios, premature labor, Apgar scores of less than 7 at the first and fifth minutes, low birth weight and intrauterine growth retardation.
We present two unrelated female patients with a complex pattern of congenital malformations including encephalocele, oesophageal atresia, abnormal lung lobation, congenital heart defects, anal anomalies, liver, spleen and radial defects. Clinical variability between the two cases can be seen as a result of variable expression. The pattern of anomalies in these two unrelated patients suggest that they may represent the same, as yet unknown, syndrome.
We report a case of a 6 month old baby boy who had congenital nasal encephalocele, repaired via the traditional staging procedure. The surgical techniques and procedures are described and discussed.
Nasal dermoids, gliomas, and encephaloceles are uncommon congenital lesions that result from aberrant embryologic development. We have treated 46 children with these nasal lesions. In view of the potential intracranial connection, patients are at risk for intracranial infection, and early surgical correction is thus imperative. Neuroimaging studies may help to predict intracranial involvement.
Transvaginal sonography identified an anterior parietal encephalocele that was not well-delineated by abdominal scanning. High resolution with access to the fetal head makes this modality ideal for studying the intracranial anatomy.
This is a neuropathological report of the youngest case ever recorded of tectocerebellar dysraphia with posterior encephalocele. All previously described stigmata were ascertained as well as a few additional findings. Some morphological features of this syndrome similar to and some different from the Dandy-Walker syndrome and the Cleland-Chiari (Arnold-Chiari) anomaly are briefly discussed. Finally the possibility of clinical diagnosis and favourable prognosis of this syndrome are suggested.
We present a case of an intranasal encephalocele in a 6-year old girl, that appears as an intranasal mass with CSF rhinorrhea. The surgical treatment is described, specially the extracranial step through a paralateronasal via. The embryogenesis, classification, diagnosis and management of these rare congenital lesions are discussed.
A rare case of sphenoethmoidal encephalocele with detailed pathological studies is reported. Only a few cases have been documented and were reviewed. The etiology and embryology of the congenital malformation remains speculative with an agreement on their development at an early embryonic stage. The mass presenting in the nasopharyngeal cavity remains often undetected unless causing respiratory embarrassment and is frequently misdiagnosed. Thorough radiological investigations detect the bone defect and outline the extent of the herniated mass and associated cerebral abnormalities. Small lesions have been successfully repaired. Larger ones prove less amenable to surgery and carry a high mortality.
The authors report the case, non forming part of any known syndrome, of a newborn with encephalocele, high cranial schisis, ocular anomalies, cleft palate and congenital clubfoot. The photographic documentation of the malformations is not possible due to a technical error in taking the photographs (lack of light in the room). Alterations to cerebral nervous tissue were histologically and cytologically characterised by immaturity and block of normal development during the early stages. For this reason the alteration of primary neuro-ectodermal tissue appears to be the initial factor with secondary involvement of anatomically and functionally contiguous fields and tissues, such as cranial bones and the eyes. The alteration of the nervous system resulted in the incapacity of the fetus to make fetal movements, making it vulnerable to extrinsic compression, above all uterine pressure. This led to the adoption of fixed postures with articular blockage most evident in the lower limbs (congenital clubfoot). The pathogenic noxa was not identified.
Congenital midline nasal masses are rare entities and therefore easily misdiagnosed or missed altogether. This article reviews the three most common congenital midline nasal masses: dermoids, gliomas, and encephaloceles. Discussion entails embryology, diagnosis, radiologic workup, surgical therapy, and complications.
Spontaneous encephalocele of the temporal bone is an uncommon condition (37 cases to date) associated with cerebrospinal fluid otorrhea. It results from a congenital dehiscence of the tegmen tympani and tegmen mastoideum. The AA. report 3 additional cases and review methods for diagnosing and surgical treatment.
Temporal bone encephalocele (TBE) has become less common as the incidence of chronic mastoid infection and surgery for this condition has decreased. Due to its declining incidence, the diagnosis of TBE may be delayed and result in the development of serious complications such as cerebrospinal fluid leak, meningitis, epidural or subdural abscess. Six cases of large (>1 cm) TBE of diverse etiology are described. Two patients had suffered previous temporal bone fractures, two had had prior mastoidectomy, and two patients had long-standing chronic mastoiditis. Two patients had undergone prior unsuccessful transmastoid repair. All patients underwent successful tegmen-dural repair with autogenous fascia, bone, and/or cartilage, primarily via a combined mastoid-middle fossa approach. Accurate dural closure and support of intacranial contents are imperative to prevent recurrence. We find that permanent repair can best be performed with a combined mastoid middle cranial fassa approach.
We report ten sporadic cases of Brazilian patients with facial midline defects, callosal agenesis, basal encephalocele, and ocular anomalies. This very rare cluster of anomalies has been well reported before. However, only until recently it is recognized as a syndrome belonging to frontonasal dysplasia spectrum. The ten cases confirm a distinct clinical entity and help to define the phenotype more precisely than previously. Up to now etiology remains unknown, although we conjecture that it is due to a mutation in TGIF gene.