[MIM 159 050, 300 376]

(Becker-Kiener syndrome)

Incidence: 1/18 000 to 1/31 000 boys. X-linked recessive transmission of the DMD gene (Xp21.2) coding for dystrophin. It is a dystrinopathy with later onset of the clinical manifestations  and slower progression than in Duchenne muscular dystrophy (see this term), but the cardiac involvement is more serious: conduction disorders, arrhythmia, cardiomyopathy (sometimes: indication of heart transplant). Muscle cramps, CK levels are elevated. Carrier women may present the disease on a minor mode (elevation of CK level, heart disease in adulthood as well as mild muscle involvement).

Anesthetic implications: 

similar to those of Duchenne disease (see this term). No succinylcholine; risk of acute rhabdomyolysis in presence of a halogenated volatile anesthetic (hyperKalemic cardiac arrest, dark urine following anesthesia ...). These episodes are often wrongly qualified as 'malignant hyperthermia'; cardiac complications (unknown cardiomyopathy).

References : 

-        Breucking E, Reimnitz P, Schara U, Mortier W.
Anesthetic complications. The incidence of severe anesthetic complications in patients and families with progressive muscular dystrophy of the Duchenne and Becker types (in German).
Anaesthesist 2000; 49:187-95.

• Gurnaney H, Brown A, Litman RS. 
  Malignant hyperthermia and muscular dystrophies. 
  Anesth Analg 2009; 19: 1043-8. 

• Hermans MCE, Pinto YM, Merkies ISJ, de Die-Smulders CEM, Crijns JHGM, Faber CG.
  Hereditary muscular dystrophies and the heart. 
  Neuromuscul Disord 2010; 20: 479-92
• Hoogewaard EM, van der Wouw PA, Wilde AAM, Bakker E, Ippel PF et al. 
  Cardiac involvement in carriers of Duchenne and Becker muscular dstrophy. 
  Neuromuscul Disord 1999; 9: 347-61.
• Segura LG, Lorent JD, Weingarten TN, Scavonetto F, Bojanic K, Selcen D, Sprung J. 
  Anesthesia and Duchene or Becker muscular dystrophy: review of 117 anesthetic exposures. 
  Pediatr Anesth 2013; 23 : 855-64
• Pregardien C, Nassogne M-C, Uksel D, Veyckemans F.
  Case report: red urine after day care strabismus surgery.
  A&A Case Reports 2017; 8: 75-7.

Updated: November 2019