Abstract
Dear Editor,
We are presenting a nine year old male patient weighing 23 kg, diagnosed case of Proteus syndrome (PS) who was posted for closed reduction and internal fixation following fracture of left shaft of femur. He had a history of trauma of left leg one week before. Airway assessment showed Mallampati grade 4, tongue hemangioma and misaligned teeth. Systemic examination revealed multiple hemangiomas and asymmetric enlargement of both upper and lower limbs. All laboratory investigations were within normal limits.
After checking adequate nil per os (NPO) and parental informed consent, intravenous line (IV) was secured with a 26G cannula (VYGONÜLE V PTFE, Lars Medicare Pvt. Ltd.) in view of difficult IV access. We opted for inhalational induction with sevoflurane in 100% oxygen due to anticipated difficult airway. We put in a proper size Ambu LMA (Laryngeal Mask Airway, #2.5 for 20–30 kg) to secure the airway with spontaneous ventilation. After observing end tidal CO2 tracing without an oropharyngeal leak or gastric insufflation at fresh gas flows of 2 L/min, airway adequacy was confirmed. Atracurium (7.5 mg) & fentanyl (40 μg) were administered. Patient was then put on pressure controlled ventilation with tidal volume of 6–8 ml/kg, respiratory rate of 14–16/min & inspiratory/expiratory ratio 1:2. A fiber-optic bronchoscope was kept ready in case of any difficulty. For post-operative analgesia, ultrasound guided caudal block with 15 ml of 0.2% ropivacaine was administered. Neuromuscular blockade was reversed with neostigmine & glycopyrrolate and he was shifted to post-anesthesia care unit for observation. The patient was given deep venous thrombosis prophylaxis peri-operatively according to protocol.
Proteus syndrome (PS) is rare congenital malformation with multiple variants presentation. It’s named after the Greek Sea-God Proteus who could supposedly change forms to avoid being captured. These patients usually present with asymmetric enlargement of limbs, multiple epidermal nevi, lipoma, cranial hemi-hyperplasia, skull hyperostosis, spinal anomalies, ovarian, testicular, meningeal and/or parotid tumors, and cystic lung lesions.
Pennant et al. provided general anesthesia to a 14 y/o boy of PS with fiber-optic bronchoscope for difficult airway posted for orthopedic surgery. Pradhan et al. reported a case of a 7 y/o boy with PS having teeth malocclusion, high arched palate & inspiratory stridor where intubation was performed with McCoy laryngoscope. Sinha et al. described a case of difficult intubation with McCoy laryngoscope in a 22 y/o female patient undergoing emergency laparotomy for abdominal trauma. Ambu LMA has an airway tube which is pre-curved with no aperture bars helping in easy passage of fibre-optic bronchoscope or airway exchange catheter, if necessary for intubation. It may be used in conditions when it is critical to avoid multiple insertion attempts, in case of airway rescue or to avoid airway injury.
Proteus syndrome patients with difficult airway are successfully anesthetized using an Ambu LMA. This information is not mentioned before. Ambu LMA might be considered to be an alternative technique due to easy use. Further research is required to explore this particular syndrome from anesthetic point of view since only a few case reports are available at this time.’
Conflict of interest
There is no conflict of interest.