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Published on: February 29, 2020
Prevention of auricular deformity in children with diastrophic dysplasia
Sharon L Cushing1, Rebecca L Swanson, Kathleen C Y Sie
1Division of Pediatric Otolaryngology, Head and Neck Surgery, Seattle Children's Hospital, Seattle, WA, United States; Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Toronto, ON, Canada.
Insights
Diastrophic dysplasia can cause auricular swelling and deformity in infants. This study suggests conforming molds, not incision and drainage, are better for treating ear swelling and preventing deformity.
Area of Science:
- Orthopedics
- Pediatric Genetics
- Plastic Surgery
Background:
- Diastrophic dysplasia is a rare genetic disorder characterized by skeletal abnormalities.
- Infants with diastrophic dysplasia frequently develop auricular swelling, often leading to permanent ear deformity.
- Current literature lacks established guidelines for the initial management of auricular swelling in this condition.
Observation:
- Two siblings with diastrophic dysplasia were treated for auricular swelling.
- One sibling's ear was treated with incision and drainage.
- The other sibling's ear was treated with conforming auricular molds (pressure alone).
Findings:
- The ear treated with incision and drainage showed a worse outcome compared to the ear treated with conforming molds.
- Conforming auricular molds provided a simple and effective method for compression of auricular swelling.
- This approach aims to prevent the characteristic permanent ear deformity associated with diastrophic dysplasia.
Implications:
- This study introduces a novel, non-invasive approach for managing auricular swelling in diastrophic dysplasia.
- The use of conforming molds may be a crucial intervention to preserve ear structure in affected infants.
- Further research should explore the long-term efficacy and widespread applicability of this conservative treatment.
Abstract:
In diastrophic dysplasia, auricular swelling commonly occurs in early infancy, inevitably leading to deformity. Till date, no description exists in the literature for the initial treatment of auricular swelling in this population. We present two siblings with diastrophic dysplasia on whom auricular swelling was treated with incision and drainage or conforming auricular molds. The ear treated with incision and drainage had worse outcome than those treated with pressure alone. This paper presents a novel but simple approach to the compression of auricular swelling in the setting of diastrophic dysplasia, using conforming molds with the goal of preventing permanent deformity.
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