Anesthetic management of three pediatric cases with Pena–Shokeir syndrome

Abstract

Pena-Shokeir syndrome is a rare, early lethal disease. It is characterized by fetal growth restriction; craniofacial deformities, for example micrognathia and microcephaly; multiple ankyloses; and pulmonary hypoplasia. For patients with this syndrome, maintenance of airway and control of perioperative respiratory complications are important for anesthetic management. We report 3 pediatric cases of Pena-Shokeir syndrome undergoing tracheostomy and arthrolysis under general anesthesia using sevoflurane, nitrous oxide, fentanyl, and vecuronium bromide. Anesthetic procedures including mask ventilation, tracheal intubation, and extubation were successfully performed without complications during and after surgery. In patients with Pena-Shokeir syndrome, inhalational anesthetics can be safely used for induction and maintenance of anesthesia, although it is important to assume that difficult airway management might be encountered.

Pena-Shokeir syndrome: current management strategies and palliative care

“…If full resuscitative efforts are agreed upon, the resuscitation team must anticipate that the newborn will have respiratory distress secondary to pulmonary hypoplasia and be prepared for a difficult intubation 74 due to edema of the head, 75 , 76 a short neck, 76 and micrognathia. 75 , 76 Various airway devices must be available, including a face mask, oral and nasal airways, laryngeal mask airways, and endotracheal tubes with the addition of a fiber optic or video laryngoscope, if possible. 2…”

“… 74 The presence of cerebral malformations may also increase the risk of seizures, which require anticonvulsive therapy. 69 , 73 , 75 These patients may also sustain fractures postnatally as the bones are hypoplastic with decreased calcification. 78 …”

“…However, the use of sevoflurane for induction and maintenance, and rocuronium or vecuronium for muscle relaxation has been successful without adverse events reported in many patients. 74 , 75 …”