The Vascular Type is considered the most serious form of EDS due to the possibility of spontaneous arterial vessel or organ rupture. If a patient presents with severe head, chest, abdominal, back or limb pain – it should be considered a TRAUMA SITUATION. This patient’s complaint should be immediately investigated using MRA, MRI, or CT-Scan testing — not x-rays.
Here is a condensed list of life-saving surgical and post-operative suggestions for patients with Ehlers-Danlos Syndrome – Vascular, Type IV. Although considered rare, clinical diagnosis of EDS Vascular is often difficult . In a trauma situation do not assume that your EDS patient has been typed correctly. EDS Vascular is a life- threatening connective tissue disorder that affects all tissue, arteries and internal organs making them extremely fragile.
Roughly 1/2 of all cases of Vascular EDS are new mutations with no family history. The other 1/2 are familial, inherited from an affected parent. Vascular EDS is autosominal dominant.
1. CT scans or MRI’s – immediately
2. No arteriographics, enemas, or endoscopies
3. Non-invasive techniques only – no stress/tension on skin, organs, or vessels – extreme care during physical exam or passing nasogastric tubes
4. Anesthesiologist please note: when intubating – fragile mucus membranes throughout – a lower peak volume pressure may be necessary
5. Vascular surgeon’s assistance anticipated in every surgery – meticulous, gentle handling of internal organs, and vessels
6. Plastic surgeon’s presence may be necessary
7. Aneurysm – a small soft tipped catheter with micro coil (memory) has been successful in some cases
8. Abdominal aneurysm – Double woven velour/Teflon grafts
9. Colonic rupture – consider permanent colostomy/ileostomy to reduce the risk of recurrent perforation
10. Padded clamps with red rubber catheter covers (Fogarty Hydrogrips)
11. Use Lange’s lines for incisions – whenever possible (Teflon sutures)
12. Incision pressure – use 1/3 -to- 1/2 less pressure, with meticulous, gentle dissections – avoid tension/stress on suture lines.
13. Ligation of vessels – use surgical hemoclips and umbilical tapes – where anastomosis is required, buttressed sutures by Teflon or felt pledgets
14. If necessary the sacrifice of a non-essential organ or limb to save a life must be considered
1. Monitor for: peritonitis, pneumoperitoneum, and/or other infections
2. Monitor for: ruptures, cysts, and abscesses
3. Monitor for: wound dehiscence, ileus, gastrointestinal bleeding
4. Monitor for: arteriovenous and/or intestinal fistula
5. Monitor for: aneurysms, embolus, hematoma
6. Monitor for: eventration of diaphragm, pleural effusion, pneumothorax
7. Monitor liver for: bleeding, changes in pressure and/or function
8. Wound packs and abdominal binders (reduce risk of incisional hernia)
9. Monitor for: increased or erratic blood pressure
10. IV placement: may be problematic due to fragile veins (If necessary, permanent access port catheter has been used)
11. Less IV pressure: slower rate when administering fluids
12. Immediate evaluation – of any change in vitals or additional complaints
13. The most non-invasive post-operative care available is recommended
14. Be vigilant – as status can change abruptly with this patient
“Microangiopathy of the skin capillaries with microbleedings, presence of microaneurysms and increased transcapillary diffusion. Microvascular involvement appears to be an additional manifestation of the syndrome.” (Superti-Furga et al. 1992)