Ketodex for anesthetic management in a case of Proteus syndrome

Sir, Proteus syndrome (PS) is a rare (1/million) hamartomatous genetic disorder characterized by disproportionate overgrowth of various body tissues.[1,2] A 21-year-old female patient of 176 cm in height and 58.5 kg in weight with PS presented with abnormal uterine bleeding. She had a history of an episode of pulmonary embolism and, on evaluation with venous Doppler, superficial venous thrombosis at the level of great saphenous vein was diagnosed and was on tablet rivaroxaban 10 mg OD. She was scheduled for examination under anesthesia endometrial curettage with Mirena insertion. She was anemic (hemoglobin 8.9 g/dL), had skin lesions on the neck, hyperostosis of the right upper and lower limb, and macrodactyly of the right hand and toes [Figure 1]. Airway examination revealed Mallampatti class-III, large tongue, a thyromental distance of 4.5 cm, elongated asymmetrical neck, asymmetrical face, mild torticollis, restricted neck movements, and poor submandibular compliance [Figure 1]. She had multiple vertebral enchondromas, thoracic kyphoscoliosis, and severe restrictive lung disease. Echocardiography revealed mild pulmonary artery hypertension and right ventricular diastolic dysfunction.Figure 1: Clinical features of Proteus syndrome: (a) skin lesion on neck; (b) macrodactyly of the right hand and toes; (c) thoracic kyphoscoliosis and (d) asymmetrical face with elongated asymmetrical neckThe rivaroxaban was stopped 24 h before the procedure, and high-risk informed consent was obtained. A difficult airway cart with a videolaryngoscope and supraglottic devices was kept ready. Intravenous (IV) sedation was started with dexmedetomidine 1 μg/kg (60 μg over 10 min infusion) and ketamine 0.5 mg/kg as a slow bolus. Dexmedetomidine infusion was continued at the rate of 0.5 μg/kg/h, and 20 mg IV bolus of ketamine was administered during the procedure. The procedure was started after the Ramsay Sedation Scale score of 3 was reached. Oxygen support was started at 4 L/min with nasal prongs. Injection tranexamic acid 500 mg, ondansetron 0.1 mg/kg and paracetamol 1 g were administered. The procedure lasted 30 min, the patient maintained stable hemodynamics and adequate respiration. The patient was restarted on tab rivaroxaban 24 h after the procedure. Clinical features of PS include disproportionate asymmetric hemihypertrophy, partial bilateral gigantism of hands and feet, thickened palms and soles, pigmented naevi, mesodermal tumors, macrocephaly with cranial hyperostosis, cystic lung disease, venous varicosities, anomalous teeth, scoliosis, elongated neck, vertebral enlargement, hemivertebrae, and scoliosis.[1,2] Asymmetrical face, maxillary and mandibular dysmorphism, abnormal teeth, and lymphangiomatous airway masses can make airway management challenging.[3] A previous report described hypertrophied epiglottis blocking the glottic view and overcome by the use of a McCoy laryngoscope.[3] In another case, using a fiberoptic bronchoscope helped secure the airway successfully.[4] In the index case, we anticipated a difficult airway and kept appropriate preparations ready. Vascular complications like thromboembolic events are known in PS, similar to the present case.[2] Dexmedetomidine has analgesic, amnesic, sedative, and anxiolytic properties. A distinct advantage of dexmedetomidine is maintaining respiratory force and preserving airway patency.[5] However, dexmedetomidine can produce bradycardia and hypotension and is not considered sufficient alone for painful procedures. Ketodex is an ideal combination negating each other’s adverse effects while potentiating benefits.[5] The index case had a difficult airway and severe respiratory disease; the use of ketodex sedation avoided the need for airway maneuvering and respiratory compromise. Other common options for sedation could be midazolam, fentanyl, propofol, and ketofol. However, these may have chances of cardiorespiratory depression, airway compromise, lack of analgesia, or delayed recovery. In conclusion, ketodex sedation with vigilant monitoring helped us successfully manage the case. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.