A Rare Case Report on Management of Fibrodysplasia Ossificans Progressiva with Ventricular Septal Defect

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Ms. Achal Shende, Ms. Pallavi Dhole, Aniket Pathade , P.S. Pande

Abstract

Background: Fibrodysplasia ossificans progressiva is a progressive fibrodysplasia. It's a rare genetic condition. This disorder is characterised by soft tissue ossification and congenital stigmata of the limbs. the case report of a male child who was tracked for 10 years, starting at the age of three years and nine months when the diagnosis was made. A newborn was born with a ventricular septal defect and bilateral hypoplasic hallux valgus. The transsternal method was used to fix this flaw. When the 32-month period was over, Following that, neck motion was restricted, and ectopic ossification developed. Oral prednisone or other anti-inflammatory medicines were used to treat the sickness of four crises aggravation. The medicine sodium etidronate (5 to 10 mg/kg/day) was prescribed for roughly six years, but treatment was stopped for a few days due to osteopenia. The course of disease has been relentless, with restrictions to severe movement.


 


Patient specific information: A male child was admitted in acharya Vinoba Bhave rural hospital on dated 10/07/2021 with the chief complaint of pain, fever, swelling, reduced mobility in joints, knees, wrists, shoulders, spine, and neck, ossification of short toe and thumb, progressive fusing of neck vertebrae, muscle and fibrous tissue , tumor like swelling in shoulder and other joints, abnormal lateral curvature of the spine etc.


 


Main symptoms or important clinical findings: A male child born to a  non-consanguineous parents as their firstborn admitted to  acharya Vinoba bhave rural hospital on dated 10/07/2021 with the  chief complaint of pain, fever, swelling in tendons, the patient had undergone various investigation like careful history ,Complete blood count, X-ray, CT scan, MRI, and other physical examinations are all available.  


 


Conclusion: Fibro dysplasia ossificans is a progressive fibro dysplasia. It has been characterized as a connective tissue disorder due to overexpression of a bone morphogenetic protein. There is no any curative therapy is available& their  management is dependent on the child’s deformity, with a focus on preventing aberrant ossification. As a result, clinician awareness of the disease is critical.

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