# A Challenge in Perioperative Anesthetic Management: A Case Report of an Infant With Concurrent Ullrich Congenital Muscular Dystrophy and Pierre Robin Sequence

## TL;DR

This case report details the successful anesthetic management of an infant with two rare conditions, Ullrich congenital muscular dystrophy and Pierre Robin sequence.

## Contribution

The report provides insights into managing anesthesia for a patient with concurrent UCMD and PRS, highlighting unique challenges and solutions.

## Key findings

- The patient with COL12A1-variant UCMD and PRS was successfully managed under anesthesia.
- Careful airway management and selection of anesthetic agents were crucial for avoiding complications.
- The case demonstrates a multidisciplinary approach to perioperative care in complex congenital conditions.

## Abstract

Ullrich congenital muscular dystrophy (UCMD) is a severe form of progressive congenital muscular dystrophy, and it presents with severe hypotonia, recurrent respiratory infections, respiratory failure, and problematic airway management secondary to congenital anatomical abnormalities and high aspiration risk. Pierre Robin sequence (PRS) is a rare congenital syndrome presenting at birth with a triad of micrognathia, glossoptosis, and chronic upper airway obstruction leading to known difficult airway management and opioid sensitivity. Perioperative management of both UCMD and PRS requires careful consideration of airway management, choice of anesthetic agents, and potential involvement of respiratory complications; each condition presents its own unique challenges. This report discusses the successful, complete anesthetic management of a patient with collagen 12 (COL12A1)-variant UCMD and concurrent PRS.