The current case is the first reported case of radial artery pseudoaneurysm. There were also cases of abdominal compartment syndrome, compartment syndromes over the legs, upper arm and forearm due to rupture of peroneal artery, posterior tibial artery and ulnar artery. The first case was reported in 1992 with spontaneous bleeding from a gluteal artery resulting in a gluteal compartment syndrome and sciatic neuropathy. Prior to this case report, compartment syndrome as a complication of vEDS has also been reported in the literature from different countries. ![]() Median life expectancy of patient with vEDS is 48 years. ![]() The average age at the time of the first major complication was 23.5 years old. In a majority of patients, the diagnosis was only made when there is at least one major complication happened, which is defined as arterial rupture, dissection or organ rupture, like sigmoid colon perforation and perforated gravid uterus The risk of having complication at age of 20 is 25% and rises to more than 80% by age of 40. Signs of vEDS include: characteristic facial appearance (thin vermilion of lips, small chin, thin nose, large eyes), acrogeria (skin on the hands and feet appears prematurely aged) and thin translucent skin. Due to defects in type III collagen, body tissues and organs of patients with vEDS are fragile. It is due to pathogenic variants in CO元A1 gene and it is inherited in an autosomal dominant manner. Type IV or the vascular type Ehlers–Danlos Syndrome (‘vEDS’), described by Andras Barbaras in 1967, consists of 5 to 10% of EDS. Its overall prevalence lies between 1 in 10,000 to 25,000 in the general population. The patient has consented to the publication of this manuscript.Įhlers–Danlos syndrome (‘EDS’) is a group of hereditary connective tissue disorders, caused by various defects in the synthesis of collagen. To our knowledge, there were no complications and the reconstructed radial artery did not rupture ( Figure. Upon follow-up, the wound healed well and the patient has good right-hand function and he can write nicely with his right hand. It has been four years after the operation. Genomic investigation has been carried out and it confirmed that the patient has a heterozygous variant in the CO元A1 gene which is a DNA change c. Similarly, the adjacent small to medium-sized veins had focal deficient or fragmented elastic fibers noted in the tunica media. There was also a variable decrease in elastic fibers and smooth muscle cells in the tunica media. There was an absence of internal elastic lamina and loss of muscle wall at the aneurysmal sites. Microscopic examination by a pathologist found that the radial artery was with variable smooth muscle thickness, partial loss of muscle wall, aneurysmal dilatation, fibrinoid necrosis and pseudoaneurysm surrounded by a moderately dense mixed inflammatory infiltrate and fibroblastic reaction. ![]() There was no numbness over bilateral upper limbs ( Figure 1). Left forearm compartments were not tense. ![]() Repeated physical examination found tense forearm compartments, and there was also limited right wrist and fingers movement the radial pulse was weakened. He was therefore treated as cellulitis initially with antibiotics.ĭuring patient’s stay, progressive right forearm swelling was noted. White blood cell count was 17.2 × 10 9/L and C-reactive Protein (CRP) was 190.1 mg/L. His bilateral wrists movement was normal. His bilateral forearms were mildly erythematous with bruising, mildly tender and with an increase in temperature. On admission, he had normal blood pressure and was afebrile. The patient did not complain of any paraesthesia of both upper limbs. There was no recent history of epileptic convulsion, insect bite or trauma. A 30-year-old Chinese man labelled as Ehlers–Danlos Syndrome (‘EDS’) due to positive family history, with the past history of epilepsy, admitted to our unit in the year 2017 with bilateral forearm redness and swelling for two days, with the right side being more severe.
0 Comments
Leave a Reply. |