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Accident and Emergency
Published in Nagi Giumma Barakat, Get Through, 2006
In Klippel-Feil syndrome, a short neck limits head movements, the hairline is low at the back, there may be paralysis of the external rectus muscle in one or both eyes, and there is perceptive hearing loss that may be severe.
Paracondylar process combined with persistent first intersegmental vertebral artery: an anatomic case report and literature review
Published in British Journal of Neurosurgery, 2023
Haigui Yang, Xiaofei Bai, Xiaoli Huan, Tingzhong Wang
The PFIA comes from the failed obliteration of the first intersegmental artery. Embryologically, the vertebrobasilar artery develops slower than the carotid artery. When the vertebrobasilar artery has not formed, there are only 2 longitudinal neural arteries (LNAs) dorsal to the carotid artery. At this stage, the LNAs are plexuses rather than real arteries. The carotid artery supplies the LNAs through the presegmental and intersegmental arteries from the anterior circulation to the posterior circulation. After the formation of posterior communicating artery, all the presegmental arteries including primitive trigeminal artery, primitive otic artery, and primitive hypoglossal artery obliterate. Then, both upper LNAs anastomose into a basilar artery. Simultaneously, the lower LNA (segmental plexuses) anastomose to form a VA. Once the vertebrobasilar artery has formed, all the anterior-posterior intersegmental arteries obliterate to maintain a mature down-up blood flow in the vertebrobasilar artery.9 However, the order of the segmental plexuses anastomosis is from down to up. It means that the first intersegmental artery is the last one which obliterates. Genetic, hemodynamic, and environmental factors may cause the failure of its obliteration, which results in a PFIA.6 Occipitalized atlas is caused by the failure of embryonic sclerotome resegmentation which also occurs at similar embryonic stages when LNA segmental plexuses anastomosis progresses. That could explain why PFIA is usually associated with an osseous anomaly such as occipitalized atlas or Klippel-Feil syndrome.7
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.