Anesthesic Management for Escobar Syndrome: Case Report

Abstract

Escobar syndrome is a rare autosomal recessive disorder which is characterized by growth retardation, axillary, antecubital, popliteal digital, and intercrural joint flexion contracture, pterygium in the eyes, cleft palate, decreased lung capacity, genital abnormalities, and spinal deformity. In this case, we presented the anesthesic management of a 2-year-old child undergoing frontal sling operation for ptosis and amblyopia etiology exploration.

Use of Laryngeal Mask with Propofol Target Control Infusion for Anesthetic Management of a Patient with Escobar Syndrome

“…Escobar syndrome or multiple pterygium syndrome is a rare autosomal recessive disease characterized by webbing of the neck, elbows, and/or knee, and joints contractures that are usually progressive and can limit mobility [1,2]. In literature, there are several cases describing the anesthetic management of these patients [2][3][4][5]. We present case report of 10 years old boy, 22 kg, 150 cm height, who was scheduled for left tympanoplasty.…”

Escobar syndrome, difficult airway management in pediatrics: A case report

“…Multiple pterygium syndrome, whose non-lethal variant corresponds to Escobar syndrome, is a rare, autosomal recessive disease of unknown incidence. It is characterized by multiple skeletal, genitourinary and orofacial anomalies (1). Contracture bands (pterygium) around the joints is the of cause restricted mobility in these patients which, added to the association with cleft palate, ankyloglossia, syngnathia, micrognathia, restricted mouth opening and spinal anomalies, among others (2,3), constitute a challenge for the anesthesia care team, especially as concerns airway management.…”

Perioperative concerns of a patient with Escobar syndrome for ocular surgery

“…[ 1 ] Associated problems such as kyphoscoliosis, rib fusion, pectus excavatum, pulmonary hypoplasia, cardiac defects and risk of malignant hyperthermia make anaesthetic management challenging. [ 2 3 ] It is unique as airway management becomes progressively more difficult with increasing age as deformity of the airway by pterygia and contractures increase with age. We describe the anaesthetic management of a 6-year-old child with Escobar syndrome for nasolacrimal duct syringing and probing.…”

“…Patients with Escobar syndrome present for orthopaedic surgeries (scoliosis, tendon transfer, joint surgeries, contracture release, congenital hip dislocation), plastic surgery (cleft lip/palate, syndactyly), ophthalmology surgery (ptosis) and urogenital surgeries. [ 2 ]…”

“…Gamma subunit of AChR plays an important role in the action of muscle relaxants, hence we avoided muscle relaxants. [ 2 ]…”