Nuss procedure for pectus excavatum in a patient with cleidocranial dysplasia
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CASE REPORT
Nuss procedure for pectus excavatum in a patient with cleidocranial dysplasia Satoshi Takagi1 · Daisuke Hamatake2 · Eri Morinaga1 · Ai Morita1 · Akinori Iwasaki2 · Hiroyuki Ohjimi1 Received: 10 March 2020 / Accepted: 4 September 2020 © The Japanese Association for Thoracic Surgery 2020
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. Keywords Cleidocranial dysplasia · Pectus excavatum · Nuss procedure
Introduction Cleidocranial dysplasia (CCD) is an autosomal skeletal disorder resulting from delayed or abnormal ossification of bony growth [1]. Herein, we report a case of CCD in a 9-year-old boy who also had pectus excavatum. Based on our protocol, Nuss plates are ordinarily implanted for 3 years [2]. In this case, the plates remained in situ for more than 5 years because of our concern regarding the unnatural ossification in the patient.
Case A 9-year-old boy with pectus excavatum presented to our center with a desire for treatment of his chest deformity. He had been diagnosed with CCD, which was confirmed clinically based on an unclosed anterior fontanel and opposition of the shoulders anteriorly (Fig. 1a). Three-dimensional computed tomography of the bony thorax revealed * Satoshi Takagi stakagi@fukuoka‑u.ac.jp 1
Department of Plastic and Reconstructive Surgery, Faculty of Medicine, Fukuoka University, 7‑45‑1, Nanakuma, Jonan‑ku, Fukuoka 814‑0180, Japan
Department of General Thoracic, Breast and Pediatric Surgery, Faculty of Medicine, Fukuoka University, 7‑45‑1, Nanakuma, Jonan‑ku, Fukuoka 814‑0180, Japan
2
a hypoplastic sternum and clavicles and lateral positioning of the costochondral junction with relatively large cartilaginous costal components; these findings were extraordinary for his age group and were likely to be characteristic of CCD (Fig. 1b). The Haller index was 3.5, which indicated a moderate excavatum deformity [3]. We attempted using the Vacuum bell; however, the patient could not tolerate it because of the pain. Although we were concerned that there may have been little or no post-operative bone consolidation and/or remodeling owing to the underlying CCD, the Nuss procedure was performed on this patient in the standard protocol [2]. A single bar was securely inserted through the fourth intercostal spaces using a thoracoscope and our sternal elevator for better precision [4]. The patient tolerated the surgery well, and his postoperative course was uneventful. After 3 years, his height increased by 15 cm, and the chest radiograph showed restricted thora
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