Nuss procedure for pectus excavatum in a patient with cleidocranial dysplasia
Satoshi Takagi1, Daisuke Hamatake2, Eri Morinaga3
1Department of Plastic and Reconstructive Surgery, Faculty of Medicine, Fukuoka University, 7-45-1, Nanakuma, Jonan-ku, Fukuoka, 814-0180, Japan. stakagi@fukuoka-u.ac.jp.
Insights
Cleidocranial dysplasia, a skeletal disorder, co-occurred with pectus excavatum in a boy. The Nuss procedure successfully corrected the chest deformity, showing good long-term results.
Area of Science:
- Orthopedics
- Pediatric Surgery
- Genetics
Background:
- Cleidocranial dysplasia (CCD) is a rare autosomal skeletal disorder characterized by impaired ossification.
- Pectus excavatum (PE) is a congenital chest wall deformity.
- Co-occurrence of CCD and PE is uncommon, presenting unique clinical challenges.
Observation:
- A 9-year-old boy with diagnosed cleidocranial dysplasia presented with an independent pectus excavatum deformity.
- The patient underwent the Nuss procedure for pectus excavatum correction.
- Concerns existed regarding potential delayed bone healing or remodeling due to CCD.
Findings:
- The Nuss procedure was successfully performed without perioperative complications.
- The patient experienced an uneventful post-operative recovery.
- Long-term monitoring (5 years, 6 months) with a Nuss plate demonstrated sustained correction of the pectus excavatum deformity without recurrence.
Implications:
- The Nuss procedure is a viable and effective treatment for pectus excavatum, even in patients with cleidocranial dysplasia.
- This case suggests that CCD does not necessarily preclude successful surgical correction of PE.
- Further research may explore the long-term bone remodeling in patients with CCD undergoing thoracic surgery.
Abstract:
Cleidocranial dysplasia is an autosomal skeletal disorder resulting from delayed or abnormal ossification of bony growth. Pectus excavatum independently presented in a 9-year-old boy with cleidocranial dysplasia and was corrected using the Nuss procedure. There were no perioperative complications, and the post-operative course was uneventful. Although there were concerns regarding extraordinary late consolidation or remodeling of the bony thorax, placement of a Nuss plate for 5 years and 6 months improved the patient's concave deformity without re-depression.
