Codes G71.00, Muscular dystrophy, unspecified, G71.01, Duchenne or Becker muscular dystrophy, G71.02, Facioscapulohumeral muscular dystrophy, and G71.09, Other specified muscular dystrophies, were created to specifically identify different types of muscular dystrophy.
Muscular dystrophy is a group of diseases caused by genetic mutations that impede the production of proteins involved in muscle growth and development. It causes progressive weakness and degeneration of the skeletal muscles that control movement. There are several major types and dozens of sub-types of muscular dystrophy. Most are extremely rare. The disorders differ by distribution and extent of muscle weakness, age of onset, rate of progression and pattern of inheritance.
Duchenne muscular dystrophy is the most common form of muscular dystrophy. It is due to the absence of the protein, dystrophin, and causes weakness and wasting of the skeletal and cardiac muscles. Boys are most commonly affected. Symptoms of muscle weakness affecting the hips, thighs, pelvis and shoulders are noticeable between the ages of 1-6 years old as the child has difficulty walking, sitting and running. The progression is rapid. Most individuals affected by Duchenne muscular dystrophy will need a respirator by age 12 as heart and respiratory muscles become weak due to worsening muscle atrophy. There may be learning and memory impairments.
Faulty or inadequate amounts of dystrophin also causes Becker muscular dystrophy, as the same gene is involved, but with different mutations. It is very similar to Duchene muscular dystrophy, with similar signs and symptoms, but symptoms start later in childhood, are less severe and progress more slowly. Patients with Duchenne muscular dystrophy typically live into their twenties, while patients with Becker muscular dystrophy can survive into their forties or beyond.
Facioscapulohumeral muscular dystrophy begins in the teenage years and causes progressive weakness of the muscles of the face, shoulder blades and upper arms, as indicated by the name of the condition. Difficulty smiling, or fully closing the eyes is usually the first symptom of the disease. Weakness in the muscles of the shoulder causes winging, or protruding of the scapulae from the back. The trunk and lower extremities are eventually affected.Muscular dystrophy cannot be cured. Medication and therapy for functional disabilities help to manage symptoms. Non-steroidal anti-inflammatory drugs slow and reduce the muscle degeneration; immunosuppressants delay damage to dying muscle cells and antibiotics treat any infections.
A patient with facioscapulohumeral dystrophy (FSHD) presents with chronic right shoulder pain and scapular winging. Due to the persistent and refractory symptoms, the surgeon performed a scapulothoracic fusion with iliac crest bone graft. During surgery, the surgeon fused the scapula to the underlying ribs. Bone graft harvested from the iliac crest was used as an autograft, and the scapula was wired to the adjacent ribs creating a stable platform. The bone graft was supplemented with some cancellous allograft croutons to increase its volume and was placed along each rib in the interface between the rib and scapula. How is the diagnosis and procedure coded?
Assign code G71.02, Facioscapulohumeral muscular dystrophy, for the diagnosis.
Assign the following codes for the procedure:
0PH504Z Insertion of internal fixation device into right scapula, open approach, for fixation of the scapula to the ribs
0PU507Z Supplement right scapula with autologous tissue substitute, open approach, for the iliac bone autograft applied to the site, and
0PU50KZ Supplement right scapula with nonautologous tissue substitute, open approach, for the allograft croutons applied to the site
0QB20ZZ Excision of right pelvic bone, open approach, for the harvesting of the autologous bone for grafting
The placement of iliac crest bone graft and allograft is captured with the root operation “Supplement” – “Putting in or on biological or synthetic material that physically reinforces and/or augments the function of a portion of a body part.”