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Procedural sedation for children with Cockayne syndrome: Caveats and concerns

 Department of Neuroanesthesia and Neurocritical Care, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, Karnataka, India

Date of Submission26-Apr-2021
Date of Decision30-Nov-2021
Date of Acceptance26-Dec-2021
Date of Web Publication30-Jan-2023

Correspondence Address:
Rohini M Surve,
Department of Neuroanesthesia and Neurocritical Care, Third Floor, Faculty Block, National Institute of Mental Health and Neurosciences, Bangalore 560029, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_96_21


How to cite this URL:
R Palaniswamy S, Surve RM, Giribabu P. Procedural sedation for children with Cockayne syndrome: Caveats and concerns. J Pediatr Neurosci [Epub ahead of print] [cited 2023 Sep 28]. Available from: https://www.pediatricneurosciences.com/preprintarticle.asp?id=368823

   Short Report Top

Sedation for diagnostic imaging entails careful planning of anesthetic technique with anesthetic choices titrated to sustain hemodynamic stability and spontaneous respiration, more so in patients with an anticipated difficult airway. Pediatric airway presents a unique set of challenges due to the inherent anatomical dissimilarities to an adult airway. Children with neurological morbidity with associated underlying congenital syndromes are rare encounters in regular neuroanesthesia practice. Nonetheless, working insight into the associated underlying multisystemic involvement allows one to plan and execute a safe anesthetic management plan. One such clinical diagnosis requiring a thorough evaluation and preparation is the rare, autosomal recessive neurodegenerative disorder called the Cockayne syndrome (CS), also known as the Neill–Dingwall syndrome. It is characterized by stunted physical and neurological growth, premature aging, and oculo-dental abnormalities.[1] A typical bird-like facies described in CS features large prominent ears with a pointed nose, micrognathia, retrognathia, hollow cheeks, and sunken eyes. CS is known to be associated with growth failure and multisystemic involvement.[2] We present the anesthetic management for the conduct of magnetic resonance imaging (MRI) of a 3-year-old child suspected to have CS. Suspicion was based on the history suggestive of global developmental delay with regression of speech and language-related milestones at 1 year of age. On examination, the only prominent facial features were microcephaly and enophthalmos. No other obvious features of facial dysmorphism were evident. The child was 80 cm tall and weighed 10 kg. Dystonic posturing of the neck and limbs hindered adequate supine positioning. Pre-anesthetic evaluation was unremarkable. We anticipated and hence were prepared for the management of a difficult airway. Procedural sedation (PS) for MRI was achieved using intravenous (IV) bolus of midazolam at 0.05 mg/kg and infusion of dexmedetomidine at 1 μg/kg/h. Oxygen was supplemented through nasal prongs. During the periprocedural period, cardio-respiratory parameters were monitored using pulse oximetry, non-invasive blood pressure, capnography, and electrocardiograph. No additional neuromonitors were used due to concerns about MRI compatibility. The child remained hemodynamically stable, and the entire procedure was completed uneventfully. MRI confirmed the diagnosis with the presence of bilateral basal ganglia calcification with bilateral cerebral and cerebellar hypoplasia[3] and diffuse hypomyelination, as depicted in [Figure 1].
Figure 1: T-2-weighted plain MRI in the sagittal plane showing generalized cerebral and cerebellar atrophy, with brain stem thinning in Cockayne syndrome

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PS has been administered for diagnostic radiographic procedures using relatively short-acting medications to facilitate maintenance of patient’s spontaneous breathing and stable cardiorespiratory parameters, with no delay in recovery. Anesthetics such as IV midazolam, propofol, ketamine, and dexmedetomidine are commonly used agents. Among these, dexmedetomidine is one which does not cause respiratory depression and is effective as an analgesic-sedative agent with less PS failure rate, especially at the higher doses (2 μg/kg/h). Thus, for this case, we chose dexmedetomidine for PS at a dose of 1 μg/kg/h, to facilitate completion of the MRI study without requiring additional anesthetic supplements and to prevent airway collapse. A review of the literature revealed reports of similar experiences with these patients presenting for various surgeries. Airway management has been reported to be challenging wherein, advanced airway gadgets have been successfully used for airway rescue in these patients. Most of these children have required general anesthesia for elective dental and ophthalmic surgeries. In anticipation of difficult airway, inhalational anesthesia has been preferred over IV induction in most of these reported cases, with one patient requiring rapid sequence induction.[4] Difficult bag-mask ventilation could be rendered easy with the use of oral airway and laryngeal mask airway (LMA). Challenges may be encountered at different stages of securing the airway. A difficult view of glottis on direct laryngoscopy has also been reported due to the anterior larynx and high arched palate. Smaller than the planned size of the endotracheal tube was required in children with stiff epiglottis and narrowed cricoid. Blind endotracheal intubations were required in two of these reported cases. LMA has been a savior in a can’t intubate and can’t ventilate scenario. Recovery from anesthesia was complicated by delay in emergence with associated hypertension and ST depression. Amid these, there are reported cases of uneventful airway management similar to ours.[2] Disease progression can lead to mental retardation, cerebellar ataxia, and ocular blindness. With a reported average life expectancy of around 10–12 years, adults with CS requiring anesthesia services have been reported to undergo successful regional anesthetic management during cesarian section surgery.[5]

Awareness of this syndrome and its knowledge about its clinical implications on the anesthetic management are desired. Despite the rarity of this disease, these patients frequently require PS or general anesthesia for surgeries during its course. Hence, careful evaluation, planning, and preparation are emphasized in the safe anesthetic care of these patients.

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   References Top

Nance MA, Berry SA. Cockayne syndrome: Review of 140 cases. Am J Med Genet 1992;42:68-84.  Back to cited text no. 1
Khawaja AA, Tobias JD. Perioperative care of a pediatric patient with Cockayne syndrome. J Med Cases 2017;8:330-33. doi:10.14740/jmc2913w  Back to cited text no. 2
Soffer D, Grotsky HW, Rapin I, Suzuki K. Cockayne syndrome: Unusual neuropathological findings and review of the literature. Ann Neurol 1979;6:340-8.  Back to cited text no. 3
Wooldridge WJ, Dearlove OR, Khan AA. Anaesthesia for Cockayne syndrome. Three case reports. Anaesthesia 1996;51:478-81.  Back to cited text no. 4
Rapin I, Weidenheim K, Lindenbaum Y, Rosenbaum P, Merchant SN, Krishna S, et al. Cockayne syndrome in adults: Review with clinical and pathologic study of a new case. J Child Neurol 2006;21:991-1006.  Back to cited text no. 5


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