Successfully Tracheostomy and Monobloc Advancement with External Frontofacial Distraction Followed by Simultaneous Adenoidectomy, Conchotomy, and Septoplasty in Severe Late Crouzon Syndrome

Authors

  • Diar Mia Ardani
  • Magda Rosalina Hutagalung
  • Irwan Kristyono

DOI:

https://doi.org/10.37506/mlu.v21i2.2722

Keywords:

Adenoidectomy, conchotomy, septoplasty, crouzon syndrome.

Abstract

A 10-year-old male patient presented with Severe Late Crouzon Syndrome, with turri-brachycephaly, and
severe exorbitism of both eyes, visual loss, beaked nose and maxilla hypoplasia. The head circumference was
54 cm and 90 precentile. Patient had a high arched palate, tonsil hypertrophy and type III malocclusion. From
the multidisciplinary meeting, in the first stage it was decided to first secure the airway with Tracheostomy
then immediately followed by Monobloc Advancement with External Frontofacial Distraction. Then in
the second stage, after removal of the rigid external distractor, by 9 months after first surgery, the patient
underwent a simultaneous procedures of Adenoidectomy, Conchotomy, and Septoplasty. Thus, after patient
got stable in follow-up, Nasendoscopy and Tracheostomy devices was removed. After 11 months follow-up.
Visual function still not getting better. However turri-brachycephaly, exorbitism of both eyes, beaked nose
and maxilla hypoplasia, head circumference, arched palate, tonsil hypertrophy and the malocclusion were
getting much reduced, and the patient getting happier and more active.

Author Biographies

Diar Mia Ardani

Medical Staff, Pharyngeal Larynx Division, Department of Otorhinolaryngology–Head and Neck Surgery, Faculty
of Medicine, Universitas Airlangga–Dr. Soetomo General Academic Hospital, Surabaya, Indonesia

Magda Rosalina Hutagalung

Medical
Staff, Department of Plastic Reconstructive and Aesthetic Surgery, Faculty of Medicine, Universitas Airlangga–Dr.
Soetomo General Academic Hospital, Surabaya, Indonesia

Irwan Kristyono

Medical Staff, Department of Otorhinolaryngology–
Head and Neck Surgery, Faculty of Medicine, Universitas Airlangga–Dr. Soetomo General Academic Hospital,
Surabaya, Indonesia

Published

2021-03-12

How to Cite

Diar Mia Ardani, Magda Rosalina Hutagalung, & Irwan Kristyono. (2021). Successfully Tracheostomy and Monobloc Advancement with External Frontofacial Distraction Followed by Simultaneous Adenoidectomy, Conchotomy, and Septoplasty in Severe Late Crouzon Syndrome. Medico Legal Update, 21(2), 450-454. https://doi.org/10.37506/mlu.v21i2.2722