Mami Shiraishi1, Katsuaki Mishima2, Hirotsugu Umeda1. 1. Department of Oral and Maxillofacial Surgery, Yamaguchi University Graduate School of Medicine, 1-1-1 Minami-kogushi, Ube, Yamaguchi, 755-8505, Japan. 2. Department of Oral and Maxillofacial Surgery, Yamaguchi University Graduate School of Medicine, 1-1-1 Minami-kogushi, Ube, Yamaguchi, 755-8505, Japan. kmishima@yamaguchi-u.ac.jp.
Abstract
BACKGROUND: Russell-Silver syndrome (RSS) is characterized by intrauterine growth retardation, short stature without postnatal catch-up growth, and an inverted triangular face with relative macrocephaly. There have been few case reports of RSS with cleft palate, in which perioperative problems such as difficult intubation due to trismus and impossibility to wear a mouth gag due to growth failure of the mandible were described. The case of a female RSS patient with cleft palate who underwent palatoplasty is reported. CASE PRESENTATION: Although her weight was particularly low (5920 g), palatoplasty was performed under general anesthesia at 3 years and 6 months of age. Despite limited mouth opening, intubation was relatively easy. Although her mandibular alveolar width was narrow, a Dingman mouth gag could be tightly fastened around her mouth. Postoperatively, the patient was transferred to the intensive care unit without extubation due to pharyngeal edema. On the following day, since the pharyngeal edema had improved, the endotracheal tube was extubated, and her respiratory status was subsequently stable. CONCLUSIONS: In RSS patients with cleft palate, there have been a few reports of pharyngeal edema. Thus, the risk of pharyngeal edema must be considered in such patients.
BACKGROUND:Russell-Silver syndrome (RSS) is characterized by intrauterine growth retardation, short stature without postnatal catch-up growth, and an inverted triangular face with relative macrocephaly. There have been few case reports of RSS with cleft palate, in which perioperative problems such as difficult intubation due to trismus and impossibility to wear a mouth gag due to growth failure of the mandible were described. The case of a female RSSpatient with cleft palate who underwent palatoplasty is reported. CASE PRESENTATION: Although her weight was particularly low (5920 g), palatoplasty was performed under general anesthesia at 3 years and 6 months of age. Despite limited mouth opening, intubation was relatively easy. Although her mandibular alveolar width was narrow, a Dingman mouth gag could be tightly fastened around her mouth. Postoperatively, the patient was transferred to the intensive care unit without extubation due to pharyngeal edema. On the following day, since the pharyngeal edema had improved, the endotracheal tube was extubated, and her respiratory status was subsequently stable. CONCLUSIONS: In RSSpatients with cleft palate, there have been a few reports of pharyngeal edema. Thus, the risk of pharyngeal edema must be considered in such patients.
Authors: E L Wakeling; S Abu Amero; M Alders; J Bliek; E Forsythe; S Kumar; D H Lim; F MacDonald; D J Mackay; E R Maher; G E Moore; R L Poole; S M Price; T Tangeraas; C L S Turner; M M Van Haelst; C Willoughby; I K Temple; J M Cobben Journal: J Med Genet Date: 2010-08-03 Impact factor: 6.318