We were concerned that our patient’s underlying disease coupled with the invasive surgery might cause postoperative respiratory failure. Therefore, our anesthetic management focused first on adequate postoperative pain relief. We performed epidural analgesia for preventing possible respiratory problems resulting from mental retardation and laryngomalacia which are often seen in KOS [2, 5], although epidural anesthesia was not required for analgesia after sternocostal elevation according to previous reports [6, 7].
Next, our anesthetic management focused on safe extubation. Specifically, we performed a bronchoscope before and soon after extubation to evaluate airway patency. We measured her respiratory pattern before extubation to evaluate respiratory parameters and respiratory depression.
We also attempted to prevent postoperative agitation after awakening from general anesthesia. Kain ZN et al. reported that emergence delirium was likely to be caused by several variables, including a child’s underlying temperament, preoperative anxiety, use of some anesthetics and other drugs, and other conditions such as pain . Because the patient was developmentally disabled, we were anxious about postoperative agitation. Dexmedetomidine is an α2-adrenoceptor agonist with sedative, anxiolytic, sympatholytic, and analgesic-sparing effects, and minimal depression of respiratory function . Ibacache ME et al. showed that dexmedetomidine administered after inducing anesthesia reduced post-sevoflurane agitation in children, with no adverse effects . In this case, dexmedetomidine prevented emergence agitation when administered prior to recovery from general anesthesia.
Our patient had several craniofaciocervical features. In particular, micrognathia and short webbed neck are associated with airway management. Micrognathia is concerned with anterior mandibular space. Any condition that makes the space small relative to the size of the tongue will make tracheal intubation more difficult . Short webbed neck is concerned with flexion and extension of the neck. Flexion and extension of the neck is necessary to align the axes of the trachea and oral cavity to provide a line of sight for intubation . Although our patient had micrognathia and short webbed neck, these craniofaciocervical features did not make tracheal intubation difficult to perform. It is unclear how craniofaciocervical features change with growth.
In July 2020, we searched two major databases (e.g., PubMed, Google Scholar) to identify relevant articles, “Anesthetic management of Kagami-Ogata syndrome.” The initial search terms were Kagami-Ogata syndrome and general anesthesia. No date restrictions and research design filters were imposed on the searches. We excluded articles published in abstract form, in a language other than English. One article was found on the initial literature search. The article was “Anesthetic management of a child with Kagami-Ogata syndrome complicated with marked tracheal deviation: a case report” on PubMed and Google Scholar. The article reported on anesthetic management of 2-year-old child with KOS complicated with marked tracheal deviation and small bell-shaped thorax . In patients with KOS, various degrees of thoracic abnormality change with increasing age and small bell-shaped thorax tends to ameliorate with age . In the course of treatment for KOS, mechanical ventilation was performed in most patients and tracheostomy was carried out in about one-thirds of patients . Unlike the earlier reported case of 2-year-old child with KOS, it is likely that anesthetic management of older children with KOS is not required to consider the effect of small bell-shaped thorax. However, it is necessary to consider the clinical severity and degree of pectus excavatum and the effect of the treatments the patient have received, i.e., mechanical ventilation and tracheostomy.
KOS is a rare disease, with fewer than 100 patients in Japan . Only one study has been reported on anesthetic management of patients with KOS. In addition, the study reported on anesthetic management of 2-year-old child with KOS. However, considering the survival rate of children with KOS (73.5%) , the number of surgical procedures for older children with KOS such as our case is likely to increase.