Background: Sarcopenia is highly prevalent in end-stage liver disease and is associated with adverse perioperative outcomes. However, its association with rocuronium pharmacodynamics during liver transplantation (LT) remains insufficiently defined. Aim: This study aimed to evaluate the association between sarcopenia, neuromuscular blockade kinetics, and clinical outcomes in LT recipients. Methods: In this prospective observational cohort study, 139 adult LT recipients were classified as sarcopenic (n = 70) or non-sarcopenic (n = 69) based on EWGSOP2 criteria, including SARC-F, handgrip strength, and psoas muscle index (PMI). Rocuronium (1 mg/kg, ideal body weight) was administered at induction, and quantitative neuromuscular monitoring was performed using train-of-four (TOF). The primary outcome was time to complete neuromuscular blockade (T0). Secondary outcomes included intraoperative neuromuscular recovery parameters, perioperative clinical variables, and postoperative outcomes. Multivariable GLM analyses were performed to evaluate factors associated with sarcopenia and T0, while logistic regression models were used to assess factors associated with mortality. Results: Sarcopenic patients exhibited significantly reduced PMI (p < 0.001) and lower handgrip strength (p = 0.001). In the baseline binomial-logit GLM, age was independently associated with sarcopenia (OR = 1.034, p = 0.025). The onset of neuromuscular blockade was significantly prolonged in the sarcopenic group (T0: 100 vs. 80 s; p < 0.001). In the adjusted Gamma regression model, sarcopenia remained significantly associated with longer T0 after adjustment for age, sex, MELD score, BMI, and hemoglobin level (adjusted ratio = 1.232, 95% CI: 1.105–1.372, p < 0.001). Postoperatively, they demonstrated prolonged extubation time (10 vs. 7 h; p < 0.001), extended ICU stay (9 vs. 6 days; p < 0.001), and higher mortality (27.1% vs. 8.7%; p = 0.009). In multivariable logistic regression, sarcopenia was independently associated with mortality (OR = 3.26; p = 0.023), while each additional ICU day was associated with an approximate 9% increase in mortality risk in the secondary model. Conclusions: Sarcopenia was associated with altered rocuronium pharmacodynamics in LT recipients, primarily characterized by delayed onset of complete neuromuscular blockade, and this association persisted after adjustment for age and other baseline clinical variables. Sarcopenic recipients also showed prolonged extubation time, longer ICU stay, and higher mortality. These findings support the integration of sarcopenia into perioperative risk stratification and individualized neuromuscular management strategies in this high-risk population.

Background: DeSanto-Shinawi syndrome (DESSH; OMIM #616708) is an exceedingly rare autosomal dominant neurodevelopmental disorder caused by heterozygous loss-of-function of the WAC gene (10p12.1), characterised by developmental delay, intellectual disability, behavioural abnormalities, and autistic features. To our knowledge, no perioperative anaesthetic experience in this condition has been described in the published literature, and no condition-specific anaesthetic data are available to guide management. Case presentation: An 11-year-old boy (35 kg, ASA physical status II) with molecularly confirmed DESSH (2.8-Mb heterozygous deletion at 10p12.1p11.23 encompassing WAC ) and comorbid autism spectrum disorder underwent multiple dental treatments under general anaesthesia. Because condition-specific pharmacological data were unavailable and malignant hyperthermia (MH) susceptibility could not be formally excluded in a previously uncharacterised genetic disorder, propofol–remifentanil total intravenous anaesthesia (TIVA) was selected as a precautionary trigger-free technique, with dantrolene sodium vials sufficient for the maximum cumulative dose of 20 mg·kg⁻¹ kept immediately available (not pre-reconstituted). Nasotracheal intubation with a 6.0 mm cuffed spiral-reinforced tube was performed under videolaryngoscopy with Magill forceps. Anaesthesia was maintained with propofol and remifentanil for approximately three hours, with depth titrated to a Bispectral Index (BIS) of 40–60 and neuromuscular blockade continuously monitored by train-of-four (TOF) acceleromyography. At the end of surgery, with quantitative TOF monitoring confirming a moderate depth of block, sugammadex 2 mg·kg⁻¹ was administered, and full recovery of neuromuscular function (TOF ratio ≥ 0.9) was confirmed prior to extubation. The patient recovered without postoperative nausea and vomiting, emergence agitation, respiratory events, or bleeding, and was discharged home on the same day. Conclusions: In this patient with DESSH, a multimodal strategy of propofol–remifentanil TIVA with precautionary MH preparedness, objective depth and quantitative neuromuscular monitoring, and nasotracheal intubation under videolaryngoscopy was feasible and uneventful. The present report provides an initial empirical reference point for clinicians encountering this ultra-rare neurodevelopmental disorder in the perioperative setting; it does not, however, establish safety across the syndrome and should be interpreted as a single-case description.