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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.

Objectives: We aimed to document the anesthetic management and metabolic, hemodynamic, and clinical outcomes of liver-graft recipients who subsequently undergo nontransplant surgical procedures. Materials and Methods:We retrospectively analyzed the data of 96 liver-graft recipients who underwent 144 nontransplant surgeries between October 1998 and April 2016 at Başkent University Hospital. Results: The median patient age at the time of nontransplant surgery was 32 years, and 35% were female (n = 33). The median time between transplant and nontransplant surgery was 1231 days. The most frequent types of nontransplant surgery were abdominal (22%), orthopedic (16%), and urologic (13%). Seventy patients had an American Society of Anesthesiologists status of 2 (49%); the status was 3 in 71 patients (49%) and 4 in 3 patients (2%). Of the 144 procedures, 23 were emergent (16%) and 48% were abdominal. General anesthesia was used in 69%, regional anesthesia in 19%, and sedoanalgesia in 11%. Twenty-five patients required intraoperative bloodproduct transfusion (17%). Intraoperative hemodynamic instability developed in 17% of patients, and hypoxemia developed in 2%. Eleven patients remained intubated at the end of surgery (8%). Of the 144 procedures, 19 (13%) required transfer to the intensive care unit, 108 (75%) transferred to the ward, and the remaining 17 (12%) were discharged on the same day. Eight patients developed respiratory failure (6%), 7 had renal dysfunction (5%), 4 had coagulation abnormalities (3%), and 10 had infectious complications (7%) in the early postoperative period. The median hospital stay was 4 days, and 5 patients (4%) developed rejection during hospitalization. Five patients died of respiratory or infectious complications (4%). Conclusions: Most liver-graft recipients who undergo nontransplant surgery are given general anesthesia, transferred to the ward after the procedure, and discharged without major complications. We suggest that orthotopic liver transplant recipients may undergo nontransplant surgery without any postoperative graft dysfunction.

We believe that appropriate patient selection, the use of well-designed anaesthesia and surgical protocols play important roles in increasing the success rate of patient outcomes and early and late complications in laparoscopic obesity surgery.