Wormian bones in osteogenesis imperfecta and other disorders

Abstract

When are Wormian bones significant is not an easy question to answer, but its relevance is important in relation to bone dysplasias such as osteogenesis imperfecta. Recognition will differ with age of patient, radiographic objectivity, and personal subjectivity. In order to attempt an answer, the skull radiographs of 81 cases of osteogenesis imperfecta of varying ages were examined for the presence of Wormian bones. These were compared against the incidence of Wormian bones in 500 skull radiographs of normal children. Significant Wormian bones as against normal developmental variants were considered to be those more than 10 in number, measuring greater than 6 mm by 4 mm, and arranged in a general mosaic pattern. They were found in all the cases of osteogenesis imperfecta but not in the normal skulls. The occurrence of significant Wormian bones in other bone dysplasias from our material and that of the literature was recorded. Other incidental findings in the skulls of the cases of osteogenesis imperfecta were also appraised.

Pathologies génétiques des collagènes et conséquences sur le développement cranio-facial

“…La taille élargie des sutures, et les formations de type os wormiens semblent témoi-gner de la capacité d'adaptation réduite des os de la voûte crânienne à suivre la croissance de la masse neurale [7,13]. La base du crâne et le condyle mandibulaire sont d'origine enchondrale comme les os longs.…”

The role of an ophthalmologist in suspected non-accidental injury

“…5 These showed characteristic features of generalized osteopenia, abnormal thinning of cortical bone, and the presence of multiple small bone islands within cranial suture lines (Wormian bones). 6,7 The parents were immediately informed of the revised diagnosis of OI and given full access to the baby.…”

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both the retina and choroid found on angiography added weight to a diagnosis of vasculitis, particularly giant cell arteritis, 6 and led to a temporal artery biopsy. Clinical suspicion was confirmed with a positive biopsy, despite both normal ESR and CRP.

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Alternative option for osteogenesis imperfecta and trigeminal neuralgia

“…1,4 Basilar invagination, a clinical manifestation, may cause cranial nerve compression due to odontoid process protrusion through foramen magnum into the intracranial cavity. 5,6 The trigeminal nerve may be involved, resulting in neuralgic pain, often refractory to drug treatment. Other possible pathogeneses include arachnoid adhesions and increased vascularization in the foramen magnum area.…”

“…Normal anatomical relations are distorted by bone deformity and the lower surface of the foramen ovale may be posteromedially displaced, thus making the conventional route for percutaneous approaches virtually impossible to use. 5,7,8 The authors found that Mayfield clamps, necessary in the previous alternatives, also imply high complication risk given the potential fragmentation of wormian bones and deformity worsening. In cases of OI, cannulation of the foramen ovale and classical percutaneous treatment can be a useful, safe and efficient alternative using CT-guided puncture for patients with trigeminal neuralgia and other bone deforming diseases that severely affect the skull base.…”