# Failure of Mandibular Distraction Osteogenesis in Klippel- Feil Syndrome- 4: A Case Report of a Rare Syndromic Robin Sequence

## TL;DR

A rare case of Klippel-Feil syndrome-4 with Robin Sequence shows that a common surgical treatment for airway issues may not be effective.

## Contribution

Reports a novel case of KFS4 with RS and highlights the potential failure of mandibular distraction osteogenesis in this condition.

## Key findings

- The patient had significant airway obstruction despite mandibular advancement.
- The patient failed extubation twice and required a tracheostomy.
- KFS4's multiple anomalies may reduce the success of this surgical treatment.

## Abstract

Klippel-Feil syndrome-4 (KFS4), a rare autosomal recessive form of Klippel- Feil syndrome, is characterized by facial dysmorphism, nemaline myopathy, and short stature. Only 10 cases of KFS4 have been previously published in the literature. We report a novel case of a 1- month-old girl with known KFS4 and Robin Sequence (RS). At 2 months old, she underwent bilateral mandibular distraction osteogenesis to correct significant airway obstruction. Despite adequate mandibular advancement, the patient failed extubation twice and eventually required a tracheostomy. Due to the multiple anomalies present in KFS4, mandibular distraction osteogenesis may have a decreased likelihood of surgical success.