1/6. klippel-feil syndrome plus atretic meningocele in one identical twin and anencephaly in the other.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.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
2/6. Symptomatic cervical disc herniation in a pediatric Klippel-Feil patient: the risk of neural injury associated with extensive congenitally fused vertebrae and a hypermobile segment.STUDY DESIGN: A case report. OBJECTIVE: To report the occurrence of a herniated cervical disc following a high-impact activity in a pediatric Klippel-Feil patient who presented with spinal cord compression, myelopathy, and myelomalacia requiring posterior instrumented fusion followed by an anterior discectomy and fusion at the hypermobile nonfused segment. SUMMARY OF BACKGROUND DATA: The primary hallmark of klippel-feil syndrome (KFS) is the presence of at least one congenitally fused cervical segment. Studies have reported the potential risk of cervical injury from hypermobility associated with the nonfused cervical segment in KFS. The manifestation of a cervical disc herniation in the pediatric KFS patient is rare. To the authors' knowledge, the development of a symptomatic cervical herniated disc attributed to mechanical fatigue following a high-impact activity has not been addressed in the literature with respect to the pediatric KFS patient having extensive cervical fusion and a hypermobile segment. methods: A 16.8-year-old KFS boy with occipitalization of C1 and fusion of C2-C3 and C4-T1 presented with myelopathy, severe cord compression, and myelomalacia stemming from a left-sided herniated cervical disc at C3-C4 with onset following an 8-foot high rooftop jump. On radiographic evaluation, the patient's C3-C4 segment was hypermobile. RESULTS: The patient was operatively managed via a same-day combined posterior-anterior procedure. The posterior aspect of the procedure entailed a posterior lateral mass plate-screw fixation at C3-C4 with autologous iliac crest bone fusion. Anteriorly, a discectomy was performed at C3-C4 with application of an interbody tricortical autograft. After surgery, the patient wore a halo vest for 3 months, followed by a soft collar for an additional 3 months. On final follow-up at 39 months, the patient was asymptomatic with no instrumentation-related complications, fusion of the posterior graft-bed and anterior interbody graft was noted, and cervical alignment was maintained. CONCLUSIONS: A hypermobile segment in the pediatric KFS patient is a risk factor that may lead to cord compression. A symptomatic herniated cervical disc may develop from an excessive mechanical load stress in a pediatric KFS patient with multiple fused segments. In such a patient, a same-day combined posterior-anterior procedure provides cord decompression and stabilizes the spine with a favorable outcome.- - - - - - - - - - ranking = 0.032678420312988keywords = herniation (Clic here for more details about this article) |
3/6. Cervical meningocele and associated spinal anomalies.Simple meningoceles are infrequent forms of dysraphism and are often benign. They have been associated with other spinal anomalies. The uncommon cervical meningocele may have a higher propensity to be associated with other spinal anomalies. Four patients with cervical meningocele are presented with radiographic evaluation and clinical course. Multiple abnormalities were documented radiographically and operatively, including hydrocephalus, Chiari malformation, hydromyelia, lipomeningomyelocele, tethered cord, thickened filum terminale, diastematomyelia, klippel-feil syndrome, and thoracic hemivertebrae. Prior to the development of any late neurological abnormality from associated spinal anomalies, magnetic resonance imaging is recommended early in a child born with a simple meningocele.- - - - - - - - - - ranking = 1.3333333333333keywords = meningocele (Clic here for more details about this article) |
4/6. klippel-feil syndrome, iniencephalus, anencephalus, hindbrain hernia and mirror movements: overdistention of the neural tube.Bony anomalies encountered in the 'no neck' form of klippel-feil syndrome (KFS) are a wide, short, fused, bifid, retroflexed spinal canal; craniolacunia, cranium bifidum, and acrania. The only symptom may be mirror movement (MM). The CNS anomalies are hindbrain hernia, hydrocephalus, hydromyelia, syringomyelia, meningocele, myelocele, encephalocele, and anencephalus. In severe KFS, i.e. iniencephalus (IN) and in anencephalus (AN), the inion is in contact with the back. In both there is hindbrain hernia and the left thorax may contain the stomach tethered to an anterior spina bifida. KFS results from distortion of somites by an overdistended neural tube. A neural tube that fails to close cannot overdistend.- - - - - - - - - - ranking = 0.16666666666667keywords = meningocele (Clic here for more details about this article) |
5/6. Ectopic lungs in a human fetus with klippel-feil syndrome.The hyperplastic lungs protruding out of the thoracic cavity are described in a full term still-born male cotwin having klippel-feil syndrome, single umbilical artery, dextrocardia and a right sided aorta. Both lungs herniated into the abdomen through bilateral defects in the diaphragm. The righ lung, in addition, herniated superiorly toward the base of the skull. The pulmonary herniation appears to be due to spinal retroflexion which reduces the capacity of the thoracic cage. The various anomalies observed can be attributed to the disturbances caused by single umbilical artery.- - - - - - - - - - ranking = 0.0065356840625975keywords = herniation (Clic here for more details about this article) |
6/6. Cervico-thoracic neurenteric cyst: clinicoradiological correlation with embryogenesis.Two cases of cervico-dorsal neurenteric cysts are presented, one located dorsal and the other ventral to the spinal cord, with associated Klippel-Feil anomaly, meningocele, spinal dysraphism and possibly spinal cord malformation. One patient was operated on as a neonate for a cervico-thoracic meningocele and presented during adolescence with spastic quadriparesis. Imaging revealed spina bifida, a dorsally located neurenteric cyst and a tendency towards split cord malformation. The other child presented with cervico-thoracic kyphoscoliosis and paraparesis. Imaging demonstrated persistence of a cervico-thoracic remnant of the primitive neurenteric canal through the upper thoracic spine.- - - - - - - - - - ranking = 0.33333333333333keywords = meningocele (Clic here for more details about this article) |