Liberia Medical & Dental Association Journal

Extremely Unusual Case of Disabling Soft Tissue Ossification in Pediatric Patient

Authors:

1Kebede Gofer Gebretsadik, 2Robert Mulbah, 3Mogus Kebede Getahun

1John F. Kennedy Hospital Department of Radiology

2John F. Kennedy Hospital Department of Orthopedics

3Jackson K. Doe Hospital, Department of Pathology

Corresponding Author’s email: kgofer1986@gmail.com

Published At January 10, 2025 | ISSN 2521-5124

Abstract

Debilitating ossifications in soft tissue is an extremely rare phenomenon in literature. Fibrodysplasia Ossi­ficans Progressiva is a very unusual genetic disorder of Extra-Skeletal Bone formation mostly within muscle, tendons and fascia. Clinically it presents with exten­sive heterotopic ossification in soft tissue and malfor­mations in greater Toe and Thumb. During the earlier stages, clinical presentations may mimic Myositis Os­sificans, Scleroderma, Juvenile Fibromatosis, soft tis­sue sarcoma. But during the late phase, the differential Diagnosis is limited. Minor trauma and interventions will exacerbate the disease process and is not recom­mended.

Case presentation:

A case of 10 years old female patient who visited John. F. Kennedy Hospital Imaging Center, Monrovia, Li­beria on April 4th, 2021, with a complaint of progres­sive anterior chest and posterior chest deformity and swelling in the back for more than three years. She has no past medical illness. No history of trauma recorded. Her symptoms were waxing and waning initially but become progressive six months before her presenta­tion. On physical examination she had hard swelling on her back and anterior chest which has markedly affected the movement of her both arms. She also has short thumb and toe with deformity.

Plain CT-scan of the chest, abdomen and pelvis showed that she had extensive dystrophic ossifications in par­aspinal area which had formed a pseudo-articulation with ossifications arising from both humerus along the postero-lateral aspect. There were also branching soft tissue ossifications from both sides of the anterior chest within the substance of the pectoralis muscles.

Conclusion and Limitation: Diagnosis of extremely rare genetic disorders is a challenge in developing countries where there is limitation of genetic stud­ies, laboratory services and poverty. The introduction of CT scan at our imaging suite had greater impact in making our Radiological Impression.

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Search Keywords

Soft tissue ossification, Fibrodysplasia Ossificans Progressiva, FOP, genetic disorder, progressive bone formation

Supplementary Material

Extremely Unusual Case of Disabling Soft Tissue Ossification in Pediatric Patient ( Extremely Unusual Case of Disabling Soft Tissue Ossification in Pediatric Patient_2025-01-03_18-36-20.pdf )