Assessment of fetal brain abnormalities
Hung N. Winn, Frank A. Chervenak, Roberto Romero in Clinical Maternal-Fetal Medicine Online, 2021
Spina bifida aperta, manifest form of spina bifida, is classified into four types: meningocele, myelomeningocele, myelocystocele, and myeloschisis. In myelomeningocele, the spinal cord and its protective covering (the meninges) protrude from an opening in the spine. In meningocele, the spinal cord develops normally but the meninges protrude from a spinal opening. The most common location of the malformations is the lumbar and sacral areas of the spinal cord. Chiari type II malformation and secondary hydrocephalus/ventriculomegaly are mostly, and scoliosis or kyphosis occasionally, associated with open spina bifida. Surface anatomy of the fetus and appearance of clubfoot, which occasionally manifests early in mid-gestation as a complication of spinal bifida, are easily demonstrable by using 3D ultrasound. The 3D ultrasound with maximum mode can demonstrate bony structure (Fig. 4) and is helpful to detect the spinal levels of lesion and to predict neurologic prognosis. Although most myelomeningoceles are demonstrated as a protruding swelling, fetal back appears flat in the type of myeloschisis; therefore, open spina bifida may be often overlooked. Because more than 80% of cases of open spina bifida are associated with ventriculomegaly due to Chiari type II malformation, demonstration of ventriculomegaly is usually the first observable sign leading to the detailed examination of spine and the subsequent diagnosis of spinal bifida.
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Justin C Konje in Complete Revision Guide for MRCOG Part 3, 2020
There are four different types of spina bifida. These are myelomeningocele, meningocele, spina bifida occulta and closed spina bifida. (a) Myelomeningocele is the most severe type, and here the baby’s spinal canal remains open along several vertebrae in the back (portions of the backbone or spine), allowing the spinal cord and the membrane that surrounds it to push out and form a sac in the baby’s back. (b) Meningocele is the second type in which the membranes around the spinal cord (meninges) push out through a defect in the spine, but the spinal cord usually develops normally so that surgery can often be used to remove the membranes without damaging the nerves. (c) Spina bifida occulta – this is the most common and mildest type of spina bifida. Here one or more vertebrae are not formed properly, but the gap in the spine is very small. It does not usually cause any problems, and most people are unaware that they have it. (d) Closed spina bifida – this consists of a diverse group of defects, in which the spinal cord is marked by malformations of fat, bone or meninges. In most of those with closed spina bifida, there are few or no symptoms; however, in a few, the malformation causes incomplete paralysis with urinary and bowel dysfunction.
DRCOG OSCE for Circuit B Answers
Una F. Coales in DRCOG: Practice MCQs and OSCEs: How to Pass First Time three Complete MCQ Practice Exams (180 MCQs) Three Complete OSCE Practice Papers (60 Questions) Detailed Answers and Tips, 2020
Spina bifida can be subdivided into spina bifida occulta, meningocele, meningomyelocele and myelocele. Spina bifida occulta is a bone deficit with an intact spinal cord and membranes. Infants may have a hairy tuft or dimple overlying the defect. This deficit carries a good prognosis. Meningocele is a condition in which the neural membranes bulge outwards through a bony deficit. This defect is often small and requires surgical closure. Prognosis is good if there is no associated neurological involvement. Meningomyelocele occurs when the neural plate is exposed or covered with a thin layer of leptomeninges. The exposed lesion should be covered immediately with a sterile non-adhesive dressing. Prognosis depends on the severity of the lesion and of the associated neurological and orthopaedic malformations. Myelocele is defined as exposure of the central cord and is incompatible with life.
Presence of Cervical Vertebral Anomalies with Concomitant Non-Communicating Hydrocephalus and Multicystic Kidney in a Female Fetus: Where VACTERL-H Meets MURCS
Published in Fetal and Pediatric Pathology, 2021
Christoph Dracopoulos, Michael Gembicki, Jann Lennard Scharf, Amrei Welp, Nadine Berg, Jan Weichert
The postmortem X-ray of the fetus revealed a suspected segmentation disorder of the cervical spine suitable with a Klippel Feil syndrome (KFS). The pathologic examination of the fetus confirmed a skin-covered defect at the level of the cervical vertebrae C6/C7 (Fig. 4) with concomitant meningocele expanding from vertebra C4 to C7 (Fig. 3). Fusion of multiple cervical vertebrae resulting in a shortened neck as typical for KFS was not seen. It further showed the severe hydrocephalus externus and internus corresponding to prenatal observations. MCDK on the left side also was verified, whereas the right kidney was normal. Due to the presence of three of the required core features (VRH), the final diagnosis of VACTERL-H association was made. This was underpinned with the finding of a normal inconspicuous uterus and urogenital configuration excluding a Mullerian duct anomaly. Other potential malformations in the context of a VACTERL association, such as anal atresia, tracheoesophageal fistula, cardiac defects, or limb deformities were excluded during autopsy. Lack of viable chorionic tissue precluded a postmortem genetic evaluation.
Diagnosis of central nervous system abnormalities: comparison of prenatal neurosonography and foetal magnetic resonance imaging findings
Published in Journal of Obstetrics and Gynaecology, 2022
Sureyya Saridas Demir, Erkan Cagliyan, Fatma Ceren Sarioglu, Handan Guleryuz, Sabahattin Altunyurt
Foetal MRI demonstrated corpus callosum anomalies with cerebellar hypoplasia in 3 cases and normal anatomic findings in 6 cases among 36 cases with suspicion of corpus callosum anomalies in foetal USG. Foetal MRI changed the diagnosis as diastematomyelia in one of the eight cases with neural tube defect with Chiari malformation type II. Foetal MRI reported one encephalocele with Chiari malformation type III in one case, and Chiari malformation type III in one case among four cases with a diagnosis of encephalocele in neurosonography. Myelomeningocele with hydrocephalus and Chiari malformation type III in one case, and Chiari malformation type II in one case were reported in MRI among four cases with suspicion of Myelomeningocele in foetal USG. Foetal MRI revealed additional information as meningocele with vermian hypoplasia in one of the three cases with a diagnosis of meningocele in neurosonography.
The association of spinal deformity with dural ectasia in neurofibromatosis type 1
Published in British Journal of Neurosurgery, 2019
Widening of the dural sac can present as a meningocele (protrusion of the sac from the spinal column filled with spinal fluid), resulting in destruction of surrounding vertebrae. Upon radiological analysis, a scalloping appearance of the vertebral bodies is often observed alongside enlargement of the intervertebral foramina and abnormal spinal deformities.3 These deformities are known to progress over time in a process known as ‘modulation’ and must therefore be monitored. Pre-emptive surgical stabilisation may be required to avoid worsening of the deformity and potential future complications.4 Previous studies have estimated that 9–38% of NF-1 patients experience a concurrent deformity whilst 85% of patient with meningoceles have concurrent kyphosis, scoliosis, or kyphoscoliosis present.5
Related Knowledge Centers
- Birth Defect
- Cervical Vertebrae
- Lumbar Vertebrae
- Meninges
- Thoracic Vertebrae
- Spinal Cord
- Vertebral Column
- Animal Embryonic Development
- Neurogenic Bladder Dysfunction
- Neurogenic Bowel Dysfunction