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Russell-Silver syndrome with cleft palate: a case report

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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.

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Authors and Affiliations

  1. Department of Oral and Maxillofacial Surgery, Yamaguchi University Graduate School of Medicine, 1-1-1 Minami-kogushi, Ube, Yamaguchi, 755-8505, Japan

    Mami Shiraishi, Katsuaki Mishima & Hirotsugu Umeda

Authors
  1. Mami Shiraishi
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  2. Katsuaki Mishima
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  3. Hirotsugu Umeda
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Corresponding author

Correspondence to Katsuaki Mishima.

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The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Written, informed consent was obtained from the parent of the patient.

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Shiraishi, M., Mishima, K. & Umeda, H. Russell-Silver syndrome with cleft palate: a case report. Oral Maxillofac Surg 23, 113–117 (2019). https://doi.org/10.1007/s10006-018-0734-6

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  • DOI: https://doi.org/10.1007/s10006-018-0734-6

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