Diagnosis and ideal plan of management in this case?


Von recklinghausen's disease (Neurofibromas and skeletal abnormalities ) Take careful family history because u can follow up and treat the people at risk at initial stages. For this patient do through physical examination and good neurological evaluate and get evaluation with an ophthalmologist to rule out threat for his vision.Check his blood pressure if it his high look for renal artery stenosis and pheochromocytoma.Get a consult from orthopedician regarding deformities management.Get consulation from surgeons or neurosurgeons if there are any resectable tumours if it is going to benifit patient.Explain to his family members about the current status and tell them the importance of social and family support for him.
congenital nevi/junctional nevi associated with plexiform neurofibromatosis.congenital navi and neurfibroma are commonly associated
Von Reclingson, s disease with flexiform deformity and gigantism of the Rt upper limb.
NF 1 with plexiform neurofibromatosis/pseudoarthrosis.......MRI rt. upper limb to assess resectability but multiple lesions of this size and site mostly needs amputation......
NF 1 with plexiform defect.. can go for a Continuous infusion using a supra clavicular Catheter.. which later on could be used for amputation as well
Look for renal artery stenosis and pheochromocytoma because without controlling blood pressure v cannot proceed for debulking
Plexiform Neurofibromatosis,general status is fit for surgery,go for amputation.
Ideal treatment would be below shoulder amputation.
it seems to be Plexiform Neurofibromatosis
thanks for suggestions
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