DMD and kyphoscoliosis in 11 year old

Question 1

what is the cobb angle

Answer 1

used to measure the severity of scoliosis

angle of perpendicular lines from upper surface of most cephalad tilted vertebrae and the lower surface of the most caudad tilted vertebrae

Question 2

cobb angle: angles greater than ___ degrees are abnormal and surgery is recommended for angles greater than __ to __ degrees

Answer 2

10
60 to 65

Question 3

cobb angles around ___ degrees often result in pHTN during exercise while angles around __ degrees lead to pHTN at rest

Answer 3

70

110

Question 4

are you concerned about his SOB

Answer 4

yes because this potentially represents significant cardiopulmonary dysfunction 2/2 to neuromuscular scoliosis and or DMD

chest wall deformity associated with significant kyphoscoliosis can lead to impaired pulmonary development, RLD, V/Q mismatch

Question 5

what does long term hypoxia lead to

Answer 5

hypoxic pulmonary vasoconstriction and hypercapnia which leads to pHTN and RV failure

Question 6

abnormal production of dystrophin that occurs with DMD may lead to what

Answer 6

cardiomyopathy, ventricular dysrhythmias, mitral regurgitation from CT replacing myocardium
decreased cardiopulmonary reserves, ineffective cough, retained secretions and pneumonia 2/2 chronic respiratory muscle weakness
chronic aspiration 2/2 to impaired laryngeal reflexes
nocturnal oxygen desat and OSA which can contribute to pHTN and cor pulmonale

Question 7

what would be the primary focus of your physical exam

Answer 7

obtain a thorough history concering his exercise tolerance, progression of severity of SOB, any cardiac dysrhythmias

onset and progression of his DMD and kyphoscoliosis

extent of spinal deformity

focused airway neuro and CP assessment

Question 8

how would you evaluate CP status further

Answer 8

examine for breath sounds, murmurs (could have MV prolapse from DMD), JVD, enlarged liver, lower extremity edema

get an ECG, PFTs (determines severity of pts RLD and facilitates decisions regarding post op ventilatory support, ABG (to ID hypoxemia or acidosis contributing to pHTN) CXR to ID cardiomegaly or PNA

if pt is wheelchair bound then order stress echo to ID any pHTN (RV and or atrial hypertrophy), cardiomyopathy, MVP

Question 9

EKG that can be found with DMD

Answer 9

tachycardia, tall R waves in V1
prominent Q waves in the limb leads
short PR
inverted T waves

Question 10

airway eval and DMD and scoliosis

Answer 10

neck mobility, cervical scoliosis may compromise airway

size of tongue (30% pts with DMD have macroglossia

difficulty swallowing (DMD pts have diminished reflexes and increased risk aspiration)

Question 11

would you do a neuro assessment and why

Answer 11

yes to identify pre existing neurologic deficits

Question 12

would you premedicate this patient

Answer 12

I would administer reglan, pepcid, and bicitrate due to delayed gastric emptying diminished laryngeal reflexes associated with DMD

would also consider giving antisialogogue to reduce oral secretions especially since the patient will be prone (would not give if patient was already tachycardic)

I would try to avoid any premeds that might contribute to respiratory depression since his increasing SOB potentially represents significant cardiopulmonary compromise 2/2 to DMD and kyphoscoliosis/