Abstract
Beals–Hecht syndrome is characterized by multiple joint contractures, arachnodactyly and kyphoscoliosis. We report the successful management of a patient diagnosed with Beals-Hecht syndrome who had micrognathia, a high arched palate and restricted mouth opening, who presented to us in the emergency for exploratory laparotomy. Oral intubation was successfully performed using aI-gel supraglottic airway as a conduit for intubation. DOI: http://dx.doi.org/10.4038/slja.v23i1.7326
Highlights
Beals-Hechtsyndrome or Congenital Contractural Arachnodactyly (CCA) is classified as distal arthrogryposis Type 9.1 Beals-Hecht syndrome is associated with difficult intravenous access, difficult airway, difficult positioning due to multiple contractures
Case report A 5 year old, 14kg male child diagnosed with Beals-Hecht syndrome (BHS) was posted for exploratory laparotomy with a diagnosis of peritonitis
The i-gel was pulled in a cephalic direction and ETT was pushed caudally along with the uncuffed red rubber tube, and once the proximal end of i-gel neared the edge of the mouth the ETT was grasped with fingers inside the mouth
Summary
Introduction Beals-Hechtsyndrome or Congenital Contractural Arachnodactyly (CCA) is classified as distal arthrogryposis Type 9.1 Beals-Hecht syndrome is associated with difficult intravenous access, difficult airway, difficult positioning due to multiple contractures. We successfully managed a five year old who presented for exploratory laparotomy with a clinical diagnosis of peritonitis. Case report A 5 year old, 14kg male child diagnosed with Beals-Hecht syndrome (BHS) was posted for exploratory laparotomy with a diagnosis of peritonitis.
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