Bifid epiglottis with midline laryngeal hamartoma: a rare presentation of Pallister Hall syndrome

Authors

  • Saravanamuthu Thirunavukkarasu Department of Pediatrics and ENT Airway Surgery, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India
  • Kishore Sandu Airway Unit of the service of Oto Rhino Laryngology, Lausanne University Hospital (CHUV), Switzerland
  • Sasikumar Revathy Dr. Rangarajan Memorial Hospital, Mogappair East, Chennai, Tamil Nadu, India
  • Singaravelu Ramesh Department of Pediatric Anesthesiology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India
  • Selvarajan Namasivayam Department of Pediatric Surgery, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20205108

Keywords:

Bifid epiglottis, Pallister Hall syndrome, Laryngeal hamartoma

Abstract

Bifid epiglottis is a rare laryngeal anomaly that can be an isolated occurrence or as part of malformation syndromes, commonly the Pallister Hall syndrome (PHS). PHS presents with hypothalamic hamartomas, polysyndactyly, bifid epiglottis, imperforate anus and genitourinary abnormalities. We report a case of a 22 months old male child with features of PHS and Hirschsprung's disease. The presentations are with a hypothalamic mass, a bifid epiglottis and a midline mass in the larynx with symptoms of respiratory distress. The laryngeal lesion was endoscopically excised and its histology confirmed as hamartoma (laryngeal). The importance of an early diagnosis of PHS, genetic counselling and prompt management of a compromised airway and endocrine abnormalities is emphasized to have a better outcome.

Author Biographies

Saravanamuthu Thirunavukkarasu, Department of Pediatrics and ENT Airway Surgery, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India

Department of Pediatric ENT & Airway Surgery

Kishore Sandu, Airway Unit of the service of Oto Rhino Laryngology, Lausanne University Hospital (CHUV), Switzerland

Airway Unit of the service of Oto Rhino Laryngology

Singaravelu Ramesh, Department of Pediatric Anesthesiology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India

Dept. of Pediatric Anesthesiology

Selvarajan Namasivayam, Department of Pediatric Surgery, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India

Dept. of Pediatric Surgery

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Published

2020-11-24

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Section

Case Reports