Jeune's Asphyxiating Thoracic Dystrophy of the Newborn

N. Sarimurat; N. Elçioğlu; G. Topuzlu Tekant; M. Eliçevik; D. Yeker

doi:10.1055/s-2008-1071131

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Eur J Pediatr Surg 1998; 8(2): 100-101
DOI: 10.1055/s-2008-1071131

Case report

Jeune's Asphyxiating Thoracic Dystrophy of the Newborn

N. Sarimurat¹ , N. Elçioğlu² , G. Topuzlu Tekant¹ , M. Eliçevik¹ , D. Yeker¹

¹Department of Pediatric Surgery, Cerrahpaşa Medical Faculty, Istanbul University, Istanbul, Turkey
²Department of Pediatrics, Cerrahpaşa Medical Faculty, Istanbul University, Istanbul, Turkey

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Publication History

Publication Date:
25 March 2008 (online)

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Abstract

A 13-month-old patient with Jeune's thoracic asphyxiating dystrophy, was surgically treated using a methyl-methacrylate (acrylic) prosthesis. Although postoperative recovery was fast, the patient died of respiratory distress two months following surgery. Limitations of currently available surgical techniques and the need for long-term results will be discussed.

Zusammenfassung

Bei einer 13 Monate alten Patientin wurde eine methylmethacrylat(acrylic)-Prothese zur Thoraxdurchmessererweiterung verwandt. Obwohl die frühe postoperative Phase komplikationslos verlief, starb die Patientin nach zwei Monaten infolge einer Atmungsinsuffizienz. Mit diesem Fallbericht soll auf die eingeschränkten Möglichkeiten einer chirurgischen Behandlung des Jeune-Syndroms und die Langzeitprobleme hingewiesen werden.

Key words

Jeune's thoracic dystrophy - Respiratory insufficiency - Autosomal recessive disorders

Schlüsselwörter

Jeune-Syndrom - Thoraxdurchmessererweiterung - Prothese