A case of craniocleidal dysostosis presenting with vascular complications.

A case of craniocleidal dysostosis was presented. In spite of the skeletal abnormalities present, the disease was not diagnosed until the patient, a married woman of 63, developed ischaemia of her right upper limb, followed by cerebral symptoms. An arch aortogram demonstrated an occlusion of the first part of the right subclavian artery involving the origin of the vertebral artery and a post-stenotic dilatation of the left subclavian artery due to costoclavicular compression. Such vascular abnormalities have not been previously described in this condition.

Neurofibrosarcoma of the duodenum in von Recklinghausen's disease.

The clinical features and surgical management of 2 patients with neurofibrosacroma of the duodenum in association with von Recklinghausen's disease are described. The tumours were excised and the resulting duodenal defects closed satisfactorily using the jejunal serosal patch technique. Three such neurofibrosarcomas reported previously in English are reviewed.

Multiple congenital aneurysms in childhood: report of a case.

This paper reports the case of a girl who, at the age of 7 years, presented with a saccular aneurysm of a brachial artery. Over the following 8 years a further 15 aneurysm appeared, involving limb and visceral arteries and the aorta. Some, but not all, of these aneurysms have been operated upon. Complications included renovascular hypertension due to bilateral intrarenal aneurysms. She had been followed up for 15 years. Three other similar cases have been found in the medical literature, and these are also referred to. In two of these cases the affected persons also exhibited some of the other connective tissue anomalies associated with the Ehlers--Danlos syndrome. These were not present in our patient. The relationship between the syndrome of multiple congenital aneurysms and the Ehlers--Danlos syndrome is discussed.

The subclavian artery in 16 patients with complete cervical ribs.

In a series of 16 patients presenting with symptoms due to the presence of a complete cervical rib, 8 complained of neurological symptoms only and 8 presented with major vascular symptoms. There were 21 complete cervical ribs in this series, 5 patients having bilateral cervical ribs, and these were excised. At operation particular attention was paid to the anatomy and pathology of the subclavian artery in relation to the cervical rib. Two anatomical variants were present. In type A cervical rib (16 cases) the subclavian artery crossed the first rib medial to the exostosis and all patients with major vascular symptoms were in this category. In type B (5 cases) the subclavian artery crossed the first rib lateral to the exostosis and symptoms, when present, were neurological rather than vascular. The two groups can be distingushed clinically and this may be of prognostic value. Post-stenotic dilatation of the subclavian artery acompanied 15 of the 21 complete cervical ribs and was attributed to compression of the artery between the cervical rib and the anterior scalene muscle. In 8 instances the post-stenotic dilatation was complicated by aneurysm and peripheral thrombo-embolism and this was regarded as a secondary phenomenon due to intermittent trauma at cost-clavicular level. A follow-up of up to 9 years would indicate that post-stenotic dilatation of mild or moderate degree is adequately treated by resection of the cervical rib and exostosis on first rib. When an aneurysm is present with localised disruption of the arterial wall with mural thrombus, it is necessary also to excise the aneurysm for, otherwise, there is a risk of further thrombo-embolic episodes.