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Anterior sacral meningocele is a rare congenital condition. Among 177 individuals familial occurrence was reported only seven times. In this report a family in which five members had anterior sacral meningoceles is presented and an autosomal dominant inheritance is suggested.
A case of surgical repair of progressive exophthalmos of the right eye in a 43-year-old woman with neurofibromatosis Type 1 (NF1) is presented. Preoperatively, the patient's ocular movements and visual fields were intact. Visual acuity was 20/30 on the right side and 20/20 on the left. Computerized tomography scanning demonstrated complete absence of the superolateral orbital wall on the right side with a large meningocele protruding into the right orbit. Intraoperatively, a new superolateral wall was constructed using the inner table of the left frontal bone as a bone transplant. A free galeoperiosteum flap was used for water-tight dural reconstruction. A few weeks postoperatively the patient's exophthalmos showed remarkable resolution. Her ocular movements, visual acuity, and visual fields remained unchanged. In conclusion, reconstruction of the superolateral wall and repair of a meningocele in a patient with NF1 is worthwhile and can be followed by excellent cosmetic results. More important, the patient's visual functions remain preserved.
Anterior sacral meningocele (ASM) is a relatively rare, congenital disorder. Usually it presents sporadically, but there are case reports of hereditary ASMs and evidence of a dominant mode of inheritance. In this article the authors describe a case illustrating the hereditary nature of ASM and present available literature on the disease. The authors present the case of a 19-month-old boy in whom an ASM was diagnosed during a workup for constipation. The child's 31-year-old mother had been treated for the same condition 20 years earlier, when she had presented with back pain. These cases are discussed in the context of previous reports of similar cases. There are several case reports in the literature in which an ASM occurred as a familial, isolated disorder (in the absence of other caudal abnormalities or syndromes). The condition is reported more commonly in women, but it is unclear whether this is a true difference in prevalence or a diagnosis or reporting bias. A review of the literature indicates an autosomal-dominant inheritance with variable penetrance and presentation. Anterior sacral meningoceles can be hereditary. Given the potential complications of the disease if left untreated and the simplicity of screening--obtaining an abdominal radiograph and the patient's clinical history--we recommend screening of immediate family members of affected individuals. Surgical treatment is recommended if an ASM is discovered.
The authors report a case of anterior sacral meningocele associated with a rectal fistula in a patient who had presented 20 years earlier with bacterial meningitis. To their knowledge, this is the first case in which a rectal fistula developed due to an anterior sacral meningocele. The clinical presentation, diagnosis, and treatment of this uncommon lesion is discussed.
Anterior sacral meningocele was first described in 1837. Most reported cases were associated with complications, including meningitis and death, because of misdiagnosis or inappropriate surgical approach. The authors present a case of anterior sacral meningocele accidentally discovered during pregnancy and provide unique magnetic resonance imaging documentation. The pathogenesis, management, and surgical technique are discussed.
A rare congenital anomaly consisting of a spheno-pharyngeal meningocele and cleft palate is presented. The repair of the meningocele was accomplished through an intra-oral transpalatal approach. A primary pharyngeal flap was used to accomplish the repair of the cleft palate. Fortunately, there were no important anomalies of the central nervous system. Twelve years later, the patient functions normally and speaks well. The only remaining symptom of midline craniofacial dysmorphia is a mild hypertelorism.
Rudimentary meningocele (RM) is a relatively rare developmental anomaly in which meningotherial elements are found in the skin. We present a case presenting with an annular alopecia over a rudimentary meningocele. Due to the occasional presence of connection or relationship to the central nervous system in RM, knowledge of the various clinical appearances including annular alopecia is clinically important.
Intrasacral meningoceles are rare conditions that can cause symptoms of nerve root compression. We report a case of a 18-year-old female, who presented with urinary incontinence. The neurological and urological evaluation revealed lack of detrusor muscle reflex. This finding supported the hypothesis of spinal disraphism. Magnetic resonance image revealed an intrasacral meningocele. The patient was submitted to surgical excision of the lesion without postoperative complications. In the present paper, the pathogenesis, clinical picture, neuroradiological findings and surgical treatment are discussed.
The anterior sacral meningocele is the congenital hernia of the thecal sac into the pelvis through a bone defect of the anterior wall of the sacrum. The association of anorectal anomaly, anterior sacral defect and presacral mass, known as Currarino's triad, can be diagnosed during the first decade of life in the eighty per cent of the cases However, in the incomplete syndrome, the symptoms appear in adults. In order to avoid late complications and due to the clear genetic origin of this uncommon pathology, the investigation of the family is of paramount importance for the early diagnosis and treatment. We review the literature and add two cases of anterior sacral meningocele with rectal fistula and incomplete Currarino's syndrome. In one of the cases the pathology is also present in another member of the same family and is complicated with meningitis. The importance of early diagnosis and treatment of these malformations and the need for fammily genetic investigation are discussed.
We describe the rare cases of a 44-year-old woman and a 28-year-old woman each presenting with a large posterior sacrococcygeal mass and alternating symptoms of high and low intracranial pressure. The first patient underwent excision of her large sacral meningocele and simple ligation of the neck, resulting in resolution of all her associated symptoms. The second patient suffered traumatic rupture of the meningocele; she underwent excision of the redundant sac and repair of the dural defect using a musculofascial flap, also resulting in resolution of her symptoms.
Anterior sacral meningocele is defined as a spinal fluid-filled thecal sac in the pelvis communicating with the spinal subarachnoid space through a defect in the anterior sacral wall. Since scimitar shape of the sacrum is characteristic, diagnosis is easily confirmed by echography and myelography. The sac very often contains benign tumors and a thickened filum terminale which can achieve a tethered cord syndrome. This congenital malformation, whose autosomal inherited condition has been proposed, has usually few characteristic symptoms but can present itself as a neurogenic bladder from tethered cord origin. Neurosurgical treatment can prevent rupture of the meningocele with meningitis. In addition to symptomatic treatment of the neurogenic bladder, the urologist must advise neurosurgical operation to preserve potency and cure specific bladder dysfunction secondary to tethered cord syndrome.
A case of anterior sacral meningocele associated with tethered cord syndrome is reported. A 5-year-old boy was admitted for urinary and fecal incontinence which had persisted since his birth. Abdominal MRI and sacral CT showed a presacral cystic mass communicating with the spinal dural sac through a neck traversing a sacral bony defect and tethered spinal conus. Neurological examination showed that the patient had a neurogenic bladder, atrophy of the legs and anesthesia below the S3 level. Operative finding revealed a thickened filum terminale attached to the wall of the presacral meningocele. Agenesis of the nerve root below the S2 level was identified, and no neural elements entered into the sac. Untethering was performed. Postoperative course was uneventful and the patient is doing well with his neurogenic bladder gradually improving.
Klippel-Feil syndrome (KFS) is a heterogeneous entity, characterized by specific congenital anomalies of segmentation of the cervical spine. We report a case of KFS plus atretic meningocele in one identical twin and anencephaly in the other. These identical twins were both male with no chromosome defect. One of them had anencephaly diagnosed at the gestational age of 17 weeks and died soon after birth, whereas the co-twin had KFS with multiple congenital anomalies including short neck with low hairline, occipital atretic meningocele, butterfly vertebrae, hemivertebrae, cervico-thoracic scoliosis, left thumb preaxial polydactyly, and right undescended testis. He received rehabilitation during infancy and could walk at the age of 15 months. Both of these infants had preaxial polydactyly, which is an uncommon anomaly associated with KFS. In conclusion, iniencephaly and anencephaly are probably the extreme manifestations of KFS rather than different dysraphisms. Prompt clinical suspicion with early diagnostic imaging for spine lesions and occult opening of neural tube is mandatory for further intervention and rehabilitation.
A 34-year-old female patient presented with an intracranial subarachnoid hemorrhage and was found to have a dural arteriovenous fistula at the site of previous cervical meningocele repair. Subsequent occlusion was achieved with endovascular embolization. To our knowledge, the phenomenon of the development of a spinal dural fistula at the site of a meningocele repair has not been recorded before.
A routine chest X-ray in a 52-year-old man revealed a widening of the posterosuperior mediastinum and the patient was hospitalized on the suspicion of having a mediastinal mass lesion. Further investigations revealed an anterior cervicothoracic meningocele (intrathoracic meningocele), the dural herniation passing through two large midline congenital openings of the C7-T3 vertebral bodies which appeared partially fused and associated with other regional skeletal malformations.
A case of intrathoracic meningocele associated with neurofibromatosis is reported. Computed tomography (CT) demonstrated that the paravertebral mass had a characteristic low attenuation coefficient compatible with liquid content. CT-myelography further confirmed the diagnosis. We recommend that though intrathoracic meningocele is rare, it should be kept in mind when making a differential diagnosis in cases where the patient has neurofibromatosis with a posterior mediastinal mass.
One woman (aged 20) and two men (aged 57 and 29, respectively) presented with various urinary and abdominal symptoms, all of which were found at operation to have been caused by anterior sacral meningoceles. All three recovered uneventfully, though the woman was left with some residual numbness of the thigh. Anterior sacral meningocele is a rare condition in which a dural sac herniates into the pelvic region through a congenital defect in the sacrum. Since 1837 182 cases have been reported, and most of them presented with gastroenterological or urogenital signs and symptoms. We have reviewed their symptomatology, pathogenesis and treatment.
A case of lateral thoracic meningocele without neurofibromatosis is reported. CT-myelography revealed the mass has the communication with CSF space. So we could differentiate it from a posterior mediastinal tumor. It is important to think of the presence of lateral thoracic meningocele when any posterior mediastinal mass is detected.