Successful anesthetic and airway management in Coffin-Siris syndrome with congenital heart disease: Case report

Abstract

Introduction: Coffin-Siris Syndrome (CSS) is a rare congenital malformation syndrome characterized with mild to severe developmental and cognitive delay, coarse facial features, fifth digit aplasia or hypoplasia associated with ectodermal, constitutional and organ-related (cardiac/neurolo gical/gastrointestinal/genitourinary. . .) anomalies. Here, we have reported a successful anesthetic and airway management in a case of 5-year old boy with CSS who underwent congenital heart surgery. Case report: A 5-year old male child weighing 14 kg, who was diagnosed as CSS underwent operation for the repair of partial atrioventricular septal defect and secundum atrial septal defect. This case report pertains to the successful anesthetic and airway management in the background of difficult airway and presence of various cardiac abnormalities. Although patient was anticipated to be difficult for intubation due to laryngomalacia, micrognathia, macroglossia, tracheal intubation was performed without any difficulty using fiber-optic laryngoscopy. At the end of the operation, the patient was transferred to the cardiovascular intensive care unit and was extubated when his spontaneous breathing was satisfactory 4 h later after the operation without any complication. Results and discussion: CSS often requires surgery and anesthetic intervention. The abnormal facial and airway as well as mental related features may lead intubation difficult, potentially due to short neck, large tongue and lips, poor dentition and poor communication. Thinking that the practicing anesthetist needs to have appropriate knowledge for this entity and the equipment for managing difficult airway should readily be available. One of these patients which successfully managed without any complication was described in this brief report.

2022 American Society of Anesthesiologists Practice Guidelines for Management of the Difficult Airway *

“…224–227,300–303 Case reports also observed successful intubation with flexible intubation scopes (Category B4-B evidence ). 304–356…”

Successful difficult airway management of a child with Coffin‐siris syndrome

“…Awake intubation via FOB or VL can be tried, but our patient was not available for this procedure due to be child. Altun et al informed that 5‐year‐old child was intubated via FOB and oral fiberoptic intubation has been done successfully in a patient with CSS who has difficult airway and laryngomalacia. But, VL is a good alternative airway device in difficult airway cases.…”

Anesthetic management in a child with Coffin Siris syndrome

“…Numerous case reports have described the genotypic and phenotypic profile of CSS; however, few have described anesthetic management of patients with CSS, the details of which are mentioned in Table 1 . [ 3 - 5 ] Spinal abnormalities such as spina bifida, scoliosis, etc., have been associated with CSS. Although no such abnormalities were found in our patient, we did not contemplate neuraxial block in our patient as IIN/IHN has proven to be equal or more efficacious and safer than caudal/central neuraxial block.…”