Find information on thousands of medical conditions and prescription drugs.

Ehlers-Danlos syndrome

Ehlers-Danlos syndrome is a group of rare genetic disorders that diminish the body's ability to make connective tissues. It is caused by the inability of the body to synthesize different collagen types or a defect in synthesis. Depending on the individual mutation, the severity of the disease can vary from extremely mild to life-threatening. There is no known cure, other than symptomatic treatments. more...

Ebola hemorrhagic fever
Ebstein's anomaly
Ectodermal Dysplasia
Ectopic pregnancy
Edwards syndrome
Ehlers-Danlos syndrome
Elective mutism
Ellis-Van Creveld syndrome
Encephalitis lethargica
Encephalomyelitis, Myalgic
Endocarditis, infective
Endomyocardial fibrosis
Eosinophilic fasciitis
Epidermolysis bullosa
Epidermolytic hyperkeratosis
Epiphyseal stippling...
EPP (erythropoietic...
Epstein barr virus...
Erythema multiforme
Esophageal atresia
Esophageal varices
Essential hypertension
Essential thrombocythemia
Essential thrombocytopenia
Essential thrombocytosis
Evan's syndrome
Ewing's Sarcoma
Exploding head syndrome
Hereditary Multiple...
Hereditary Multiple...
Hereditary Multiple...
Hereditary Multiple...


Common symptoms are unstable, flexible joints with a tendency to dislocate and subluxate, due to ligaments which are overly stretchable, and elastic, fragile, soft skin that easily forms welts and scars. "It was the recommendation of a workshop convened in Berlin by Beighton (1986) that the Ehlers-Danlos designation be used for joint hypermobility with skin changes" in contrast to hypermobility syndromes without skin changes, once known as EDS type 11 (OMIM 147900). Other symptoms can include eye problems and nearsightedness. Bone deformities such as pectus excavatum (sunken chest) or scoliosis may present early. Most serious are vascular and organ fragility, which are thankfully less frequent.


[List your site here Free!]

Gluteal compartment syndrome after total knee arthroplasty with epidural postoperative analgesia
From Journal of Bone and Joint Surgery, 7/1/01 by Pacheco, R J

We describe two patients who developed gluteal compartment syndrome after total knee arthroplasty (TKA) carried out under epidural analgesic infusion and light sedation. To our knowledge, this occurrence has not been described previously after TKA.

J Bone Joint Surg [BrI 2001;83-B:739-40.

Received 2 October 2000; Accepted after revision 16 January 2001

Gluteal compartment syndrome is rare. It is often unrecognised and may lead to disastrous sequelae including renal failure, sepsis and death. The gluteal region is not usually considered to contain compartments, but has clearly demonstrated non-distensible osseofascial boundaries. We report three cases in two patients after total knee arthroplasty (TKA) with epidural postoperative analgesia.

Case reports

Case 1. A 47-year-old obese man was admitted for a left TKA. His body mass index (BMI) was 41.66 (normal range 20 to 25) and his only relevant past medical history was of hypertension. The operation was carried out under epidural anaesthesia introduced at the L2/3 level. The patient was positioned supine. The total operating time was 2 hours and 15 minutes, with a tourniquet applied for 1 hour and 52 minutes. After the operation he was nursed in a semirecumbent position and standard care of pressure areas was instituted (two-hourly for 24 hours, eight-hourly for 24 hours and once daily afterwards). The epidural infusion was discontinued after 22 hours. The epidural levels were between T4 and T7, assessed by the return of cold sensation. His pain score was zero at all times. Seven hours after the epidural had been discontinued he started to complain of back pain and difficulty in finding a comfortable position. He became pyrexial (38.4degC).

The next day he complained of swollen, tender and painful buttocks. His pain was not relieved by non-opioid analgesia. An attempt was made to measure the gluteal compartment pressures, but the results were inconclusive since they were borderline in the upper limit of normality. There were no abnormal neurological findings. Clinically, he was thought to have bilateral gluteal compartment syndrome and underwent bilateral fasciotomies under general anaesthesia 44 hours after the initial operation. At operation both gluteus maximus muscles were described as `dusky'. The skin was closed primarily. He made a good recovery and was discharged nine days after admission.

At follow-up two months after TKA he complained of gluteal pain and dysaesthesia in the distribution of the sciatic nerve. Nerve-conduction studies undertaken at three months showed neurogenic changes in the right gluteus maximus and biceps femoris, diminished motor responses in the distribution of the posterior tibial nerve, but no evidence of significant distal axonal involvement. After one year, he still complained of right gluteal discomfort on sitting, which had improved considerably after physiotherapy and hydrotherapy.

Case 2. A 71-year-old man, weighing 81kg (BMI 26.44), underwent right TKA, under epidural anaesthesia at the LI/2 level, in the supine position. The operating time was 2 hours 25 minutes and a thigh tourniquet was applied for 1 hour and 50 minutes. The epidural infusion was continued for 43 hours. The epidural levels to cold sensation were between T6 and T9, with a pain score of zero at all times. After surgery he was immediately started on continuous passive movement of the knee. He was nursed in a semirecumbent position and standard pressure area care was instituted (two-hourly for 24 hours, eight-hourly for 24 hours and once daily afterwards). A right foot drop was noticed about 4.5 hours after the epidural anaesthesia had been discontinued. Examination showed loss of sensation in the distribution of the right sciatic nerve with no active movement of the ankle. There was swelling and tenderness of the right buttock and the skin in the right gluteal area was indurated and discoloured. The serum levels of urea and creatinine were raised. A diagnosis of right gluteal compartment syndrome was made and he immediately underwent right gluteal fasciotomy. Neurolysis of the sciatic nerve was performed; the nerve was swollen and there was oedema of gluteus maximus, but the muscle appeared to be viable. The wounds were left open; 48 hours later necrotic muscle was excised from beneath the fascia lata and from vastus lateralis and rectus femoris. The sciatic nerve was less oedematous. The skin was closed. There was no improvement in motor or sensory function distal to the knee. Nerve-conduction studies showed significant abnormalities in the sciatic nerve and an inability to obtain sensory or motor responses in the common peroneal and posterior tibial nerves.


There are three different anatomical compartments in the gluteal region. That of the gluteus maximus lies with its own fascia, the tensor fascia lata compartment is enveloped by the gluteal fascia and the lateral fibrous coverings of the hip, while that of the gluteus medius and minimus is enclosed in the gluteal fascia and the iliac bone. An increase in pressure in any of these may lead to the symptoms of compartment syndrome with swelling, pain at rest and on muscle stretching, dysaesthesia and paralysis. If the clinical presentation is consistent with a compartment syndrome exploration is recommended, regardless of the pressure measurements.1

The gluteal compartment syndrome is uncommon with only 38 unilateral and five bilateral cases reported in the literature.2 It has been described after long periods of unconsciousness as a result of drug 1,3,4 and alcohol abuse,1,5 trauma following rupture of the superior gluteal or medial circumflex femoral arteries, and pelvic fractures, the lateral decubitus posture after total hip arthroplasty6,7 and lithotomy,8 sickle-cell induced infarction,9 in the Ehlers-Danlos syndrome,10 following acute exertional rhabdomyolysis2 and epidural analgesic infusion with motor blockade," but not after TKA.

Obese patients nursed in the sitting position may be at risk,ll and epidural infusion for postoperative relief of pain may increase this risk. Increasing pain despite analgesic medication is the main symptom, and should alert clinicians to the diagnosis. In our cases this was masked by postoperative deep sensory and motor epidural blockade. Epidural infusion for postoperative pain relief should therefore be used with caution and, if motor blockade occurs, the infusion rate or strength should be diminished or discontinued until motor function has recovered.11 Vigilant nursing care is essential in these patients in order to diminish pressure, and to detect swelling, induration and discoloration of the skin in the gluteal area, We speculate that continuous passive movement while the effects of epidural anaesthesia were still present, may have been a contributory factor in our second case, because of increased pressure on the gluteal area and of the knee movement apparatus which made optimal nursing care difficult.

While we have addressed this issue with relevance to TKA, regional anaesthesia may affect the recognition of a compartment syndrome in any limb after surgery. There is an increasing trend among anaesthetists to abolish postoperative pain, which may be ideal in theory, but can lead to the risk of missing important clinical symptoms and signs which herald a complication such as we have described.

No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.


1. Schmalzried TP, Neal WC, Eckardt JJ. Gluteal compartment and crush syndromes. Clin Orthop 1992;277:161-5.

2. Kuklo TR, Tis JE, Moores LK, Schaefer RA. Fatal rhabdomyolysis with bilateral gluteal, thigh and leg compartment syndrome after the Army Physical Fitness Test: a case report. Am J Sports Med 2000;28:112-6.

3. Torrens C, Marin M, Mestre C, Alier A, Nogues X. Compartment syndrome and drug abuse. Acta Orthop Belg 1993;59:143-6.

4. Hill SL, Bianchi J. The gluteal compartment syndrome. Am Surg 1997;63:823-6.

5. Bleicher RJ, Sherman HF, Latenser BA. Bilateral gluteal compartment syndrome. J Trauma 1997;42:118-22.

6. Pai VS. Compartment syndrome of the buttock following a total hip arthroplasty. J Arthroplasty 1996;11:609-10.

7. Lachiewicz PZ, Latimer HA. Rhabdomyolysis following total hip arthroplasty. J Bone Joint Surg [Br] 1991;73-B:576.

8. Leventhal I, Schiff H, Wulfsohn M. Rhabdomyolysis and acute renal failure as a complication of urethral surgery. Urology 1985;26:59-61.

9. Kaufman G, Choi B. Ischemic necrosis of muscles of the buttock. J Bone Joint Surg [Am] 1972;54-A:1079-82.

10. Schmalzried TP, Eckardt JJ. Spontaneous gluteal artery rupture resulting in compartment syndrome and sciatic neuropathy: report of a case in Ehlers-Danlos syndrome. Clin Orthop 1992;275:253-7.

11. Kontrobarsky Y, Love J. Gluteal compartment syndrome following epidural analgesic infusion with motor blockade. Anaesth Intensive Care 1997;25:696-8.

R. J. Pacheco, S. Buckley, N. J. Oxborrow, A. C. Weeber, K. Allerton From Friarage Hospital, Northallerton, England

R. J. Pacheco, MRCS, Research Fellow in Orthopaedics 14 Carling Close, Bradford, West Yorkshire BD7 4RU, UK.

S. Buckley, FRCS Orth, Specialist Registrar in Orthopaedics 6 Hudson View, Tadcaster, North Yorkshire LS24 8JE, UK.

N. J. Oxborrow, Specialist Registrar in Orthopaedics Level 5, Clinical Sciences Building, St James's University Hospital, Leeds LS7 TS, UK.

A. C. Weeber, FCS (SA), Consultant Orthopaedic Surgeon K. Allerton, FCS (SA), Consultant Orthopaedic Surgeon Orthopaedic Department, Friarage Hospital, Northallerton, North Yorkshire DL6 IJG, UK.

Correspondence should be sent to Mr R. J. Pacheco.

Copyright British Editorial Society of Bone & Joint Surgery Jul 2001
Provided by ProQuest Information and Learning Company. All rights Reserved

Return to Ehlers-Danlos syndrome
Home Contact Resources Exchange Links ebay