FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY

Eric Sandefur, D.O., Pediatric Orthopaedic Resident

William G. Mackenzie, M.D., Attending Pediatric Orthopaedic Surgeon

August 31, 1995

CLINICAL CASE PRESENTATION

ORTHOPAEDIC DEPARTMENT

THE ALFRED I. DUPONT INSTITUTE

WILMINGTON, DELAWARE

CASE HISTORY:
ETIOLOGY/INCIDENCE:

ONSET:

CHARACTERISTIC FEATURES:

1. FACIAL FEATURES:

2. SHOULDER GIRDLE FEATURES:

3. OTHER FEATURES:

LABS:
TREATMENT:

A. TECHNIQUES:

  1. Ketenjian (1978) described fasciodesis of scapula to the ribs utilizing fascial trips, Mersilene tapes (preferred), or Dacron strips
  2. Copeland and Howard (1978) used cortical struts and screws to fix the scapula to the ribs
  3. Bunch (1993) achieved fixation utilizing wires around the ribs and passed through the body of the scapula
  4. Jakab and Gledhill (1993) modified Bunch's procedure and used wire fixation only along medial border of scapula and ICBG
  5. Letournal (1990) passed a rib through a hole in the scapula and achieved fixation with plates, screws, and wires

B. COMPLICATIONS:

REFERENCES:
  1. Bunch W.H., Siegel I.M. Scapulothoracic Arthrodesis in Facioscapulohumeral Muscular Dystrophy. J. Bone Joint Surg. 75A:372-376,1993
  2. Copeland S.A., Howard R.C. Thoracoscapular Fusion for Facioscapulohumeral Dystrophy. J. Bone Joint Surg. 60B:547-551,1978
  3. Drennan J.C. Neuromuscular Disorders. In Morrissey RT9ed), Lovell & Winter's Pediatric Orthopaedics, Third Edition, 391-392,1990, Philadelphia, JB Lippincott
  4. 4.Jakab E., Gledhill R.B. Simplified Technique for Scapulocostal Fusion in Facioscapulohumeral Dystrophy. J. Pediatr. Orthop 13:749-751,1993
  5. Ketenjian A.Y. Scapulocostal Stabilization for Scapular Winging in Facioscapulohumeral Muscular Dystrophy. J. Bone Joint Surg. 60A:476-480,1978
  6. Letournel E., Fardeau M., Lytle J.O., Sirrautl M., Gosselin R.A. Scapulothoracic Arthrodesis for Patients Who Have Facioscapulohumeral Muscular Dystrophy. J. Bone Joint Surg. 72A:78-84,1990
  7. Mackenzie W.G., Ishikawa S. Complications of Neuromuscular Disorders. In Epp, Jr., CH & Bowen Jr., Complications in Pediatric Orthopaedic Surgery, 627-630,1995
  8. Tachdjian M.O. Facioscapulohumeral Muscular Dystrophy. In Tachdjian, MO(ed) Pediatric Orthopaedics, Second Edition, 2136,1990