Surgical Management of Shoulder and Knee Instability in Patients with Ehlers-Danlos Syndrome: Joint Hypermobility Syndrome.
Ehlers-Danlos Syndrome (EDS) is a hereditary disorder of the connective tissue, which has been classified into numerous subtypes over the years. EDS is generally characterized by hyperextensible skin, hypermobile joints, and tissue fragility. According to the 2017 International Classification of EDS, 13 subtypes of EDS have been recognized. The majority of genes involved in EDS are either collagen-encoding genes or genes encoding collagen-modifying enzymes. Orthopedic surgeons most commonly encounter patients with the hypermobile type EDS (hEDS), who present with signs and symptoms of hypermobility and/or instability in one or more joints. Patients with joint hypermobility syndrome (JHS) might also present with similar symptomatology. This article will focus on the surgical management of patients with knee or shoulder abnormalities related to hEDS/JHS.
Homere A
，Bolia IK
，Juhan T
，Weber AE
，Hatch GF
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Double-bundle anterior cruciate ligament reconstruction using autologous hamstring tendon hybrid grafts in a patient with hypermobile Ehlers-Danlos Syndrome: A case report.
Ehlers-Danlos syndrome (EDS) is a connective tissue disorder characterized by skin hyperextensibility, joint hypermobility, and tissue friability. Hypermobile type Ehlers-Danlos syndrome (hEDS) is considered one of the EDS subtypes characterized by generalized joint hypermobility. Although there have been a few case reports which described surgical considerations for anterior cruciate ligament (ACL) reconstructions in patients with other types of EDS, no reports have described those in patients with hEDS.
We report a case of ACL injury in an 18-year-old male patient with hEDS. The patient was successfully treated with an anatomic double-bundle ACL reconstruction using autologous hamstring tendon hybrid grafts which consist of hamstring tendons connected in a series with commercially available polyester tape. The autogenous tendon portion of the anteromedial and posterolateral bundles were composed of 4 and 2 strands of hamstring tendons, respectively. After 2 weeks of knee joint immobilization, continuous passive motion exercise of the knee joint and partial weight-bearing was allowed. A hinged knee brace was used for a period of 5 months postoperatively. Second-look arthroscopy at 30 months showed that the ACL graft had no laceration and an excellent coverage of the synovium. At 36 months after surgery, the side-to-side differences in the anterior laxity was remarkably improved. The operated knee showed negative Lachman test and had a full range of motion.
To the best of our knowledge, this represents the first report of anatomic double-bundle ACL reconstruction in patients with hEDS and demonstrates excellent clinical and functional outcomes.
Hishimura R
，Kondo E
，Matsuoka M
，Iwasaki K
，Kawaguchi Y
，Suzuki Y
，Onodera T
，Momma D
，Iwasaki N
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Entrapment neuropathies and polyneuropathies in joint hypermobility syndrome/Ehlers-Danlos syndrome.
This study aims to investigate the involvement of the peripheral nervous system in Ehlers-Danlos syndromes/hypermobility type patients with particular attention to entrapment syndromes.
We consecutively enrolled Ehlers-Danlos syndromes/hypermobility type patients. Patients underwent clinical, neurophysiological and ultrasound evaluations. Dynamic ultrasound evaluation was also performed in healthy subjects as control group.
Fifteen Ehlers-Danlos syndromes/hypermobility type patients and fifteen healthy subjects were enrolled. Most of patients presented tingling, numbness, cramps in their hands or feet. Clinical evaluation was normal in all patients. One patient was affected with carpal tunnel syndrome and one with ulnar nerve entrapment at elbow. One patient had an increased and hypoechoic ulnar nerve at elbow at ultrasound evaluation. Dynamic ultrasound evaluation of ulnar nerve at elbow showed, in patients, twelve subluxations and three luxations. In the control group dynamic evaluation showed one case of ulnar nerve luxation.
Statistical analysis showed a significant difference in the occurrence of ulnar nerve subluxation and luxation between patients and control subjects.
The study shows an inconsistency between symptoms and neurophysiological and ultrasound evidences of focal or diffuse nerve involvement. The high prevalence of ulnar nerve subluxation/luxation at elbow in Ehlers-Danlos syndromes/hypermobility type patients could be explained by the presence of Osborne ligament laxity.
Granata G
，Padua L
，Celletti C
，Castori M
，Saraceni VM
，Camerota F
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Ehlers-Danlos Syndrome: Arthroscopic Management for Extreme Soft-Tissue Hip Instability.
To present outcomes in a series of patients with Ehlers-Danlos syndrome (EDS)-hypermobility type who underwent hip arthroscopy for associated hip pain and extreme capsular laxity.
A retrospective chart review identified 16 hips with confirmed EDS--hypermobility type that underwent hip arthroscopy for continued pain and capsular laxity. All patients had complaints of "giving way" and pain, an easily distractible hip with manual traction under fluoroscopy, and a patulous capsule at the time of surgery. No patient had osseous evidence of acetabular hip dysplasia or prior confirmed hip dislocation. Outcomes were evaluated preoperatively and postoperatively with the modified Harris Hip Score (mHHS), the 12-Item Short Form Health Survey (SF-12), and a visual analog scale (VAS) for pain.
Evidence of symptomatic femoroacetabular impingement (FAI) was found in 15 hips (93.8%). The 16th hip had subjective giving way with a positive anterior impingement test and was easily distractible, had a labral tear, and had a patulous capsule at the time of surgery. The mean follow-up period was 44.61 months (range, 12 to 99 months). The mean preoperative lateral center-edge angle was 31.8° (range, 25° to 44°), and the mean Tönnis angle was 3.6° (range, -2° to 8°). Mean femoral version measured on computed tomography (CT) scans was 19.2° (range, -4.0° to 31.0°). Of the hips, 13 underwent primary arthroscopy and 3 underwent revision. All hips underwent hip arthroscopy with an interportal capsular cut only and arthroscopic capsular plication. There were 13 labral repairs, 2 labral debridements, 8 rim resections, 15 femoral resections, 2 psoas tenotomies, and 1 microfracture. Improved stability with an inability to distract the hip with manual traction under fluoroscopy was noted in all hips after plication. The mean alpha angle preoperatively was 58.7° on anteroposterior radiographs and 63.6° on lateral radiographs compared with 47.4° and 46.1°, respectively, postoperatively. There were significant improvements for all outcomes (mHHS, P = .002; SF-12 score, P = .027; and VAS score, P = .0004). The mean mHHS, SF-12 score, and VAS score were 45.6 points, 62.4 points, and 6.5 points, respectively, preoperatively compared with 88.5 points, 79.3 points, and 1.6 points, respectively, at a mean follow-up of 45 months. No EDS patients were lost to follow-up or excluded from analysis. The mean improvement in mHHS from preoperatively to postoperatively was 42.9 points, and there were no iatrogenic dislocations. One patient underwent further revision arthroscopy for recurrent pain, subjective giving way, and capsular laxity.
FAI and extreme capsular laxity can be seen in the setting of EDS. Although increased femoral version was common, acetabular dysplasia was not common in our study. Meticulous capsular plication, arthroscopic correction of FAI when present, and labral preservation led to dramatic improvements in outcomes and subjective stability without any iatrogenic dislocations in this potentially challenging patient population.
Level IV, therapeutic case series.
Larson CM
，Stone RM
，Grossi EF
，Giveans MR
，Cornelsen GD
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