The first experience of conducting the anesthetic support during a simultaneous surgery in a Kindler syndrome patient

BACKGROUND: Kindler syndrome is a rare autosomal recessive disease, one of the forms of congenital epidermolysis bullosa. Clinically, the disease manifests by the development of bubbles on the skin and mucosal membranes with further cicatrization, as well as by the development of narrowing in the esophagus, the urethra, the vagina and the urinary ducts. In the presented clinical case of the first ever in Russia conducted simultaneous surgery in the settings of general combined anesthesia in a patient with Kindler syndrome, evaluation was carried out for the criteria of difficult airways, special attention was paid to the method of conducting the endotracheal intubation, to monitoring the vital functions of the organism and to following the multimodal analgesia principle. Due to the high risk of post-operative nausea and vomiting, a necessity was justified for increasing the antiemetic effect. CLINICAL CASE DESCRIPTION: The main indications to conducting the surgery in a female patient aged 49 years with congenital epidermolysis bullosa (Kindler syndrome) were the complaints of significant difficulties and pain upon swallowing, decreased appetite, presence of dysphagia with a background of inhomogeneous circular narrowing of the esophagus, pain when moving the eyes and the absence of nasal breathing with a background of nasal vestibule atresia. The main tasks of surgical treatment were the elimination of incapacitating complications and improving the quality of life for the patient. The multiplicity of stages in the treatment process was deemed impractical due to the necessity of conducting three anesthetic support procedures with high risk of additional damaging the oropharynx and upper airways during tracheal intubation, due to which, a decision was drawn up on arranging a simultaneous surgical treatment of complications of the main disease. The duration of surgery was 195 minutes, while the anesthesia lasted for 210 minutes. The performed procedures included the elimination of eyelid eversion, the dissection of symblepharon, the excision of the nasal vestibule atresia and the endoscopic dilation of esophageal stricture. The postoperative period was uncompromised with reported restoring the functioning of nasal breathing, of the visual organs and with the elimination of dysphagia. Upon the examination conducted 11 months after surgery, there were no signs of recurrence of the eliminated complications of the main disease. CONCLUSION: Increasing the safety and preventing the iatrogenic complications during the course of anesthesia in patients with epidermolysis bullosa is the most important task. The development of modern medical technologies with using the microsurgical and the endoscopic methods along with the personalized approach in selecting the anesthetic support allow for wider usage of simultaneous surgeries in the treatment of complications in Kindler syndrome patients.