Hyaline Fibromatosis Syndrome: Early Outcomes Following Major Craniofacial Mass Excision.

Hyaline fibromatosis syndrome (HFS) is a rare congenital disorder characterized by abnormal hyaline deposition within soft tissues. Hyaline fibromatosis syndrome manifests in 2 distinct forms: (1) infantile systemic hyalinosis and (2) juvenile hyaline fibromatosis. Infantile systemic hyalinosis, the more severe form, typically emerges in early childhood with extensive systemic involvement. In contrast, juvenile HFS is less severe, allowing patients to survive into adulthood. Common clinical manifestations include thickened skin, hyperpigmented patches, gingival hypertrophy, skin nodules, and progressive severe joint contractures, leading to significant morbidity and potential mortality. This case report describes a 7-year-old child who was diagnosed with HFS and presented with a very large, ulcerated, rapidly expanding craniofacial mass. The patient underwent successful treatment involving a multidisciplinary medical team and strategic surgical intervention, achieving favorable postoperative outcomes.

First anesthesia exposure effects on short-term neurocognitive function among 1- to 36-month-old children: a case-control pilot study.

Background: Multiple human studies have shown no significant long-term results of anesthesia exposure during early childhood compared to the general population; however, reports on short-term neurodevelopmental assessment before and after anesthesia exposure are limited. This study aimed to evaluate the short-term characteristics of neurocognitive function post-anesthesia in noncardiac surgery compared with baseline. Methods: This prospective case-control pilot study recruited healthy participants in the control group and hospitalized children in the anesthesia group. Children aged 1-36 months without previous anesthesia were included. Neurocognitive function was assessed at baseline and seven days after anesthesia administration using a cognitive scale of the Bayley Scales of Infant and Toddler Development, third edition. The control group received only a baseline assessment. The cognitive composite score had a mean of 100 and a standard deviation (SD) of 15, with a difference of score >1/3 SD (5 points) defined as clinically significant. Results: Twenty and 39 participants in the control and anesthesia groups, respectively, were included in the final analysis. The baseline cognitive scale score of the anesthesia group was statistically and clinically lower than that of the control group. The mean (SD) cognitive composite scores in the control and anesthesia group were 111.50 (11.71) and 97.13 (9.88), P<0.001. The mean difference [95% confidence interval (CI)] was -14.37 (-8.28 to -20.47). In the anesthesia group, the post-anesthesia cognitive composite score was statistically higher than that at baseline, but without clinical significance. The mean (SD) of baseline and post-anesthesia cognitive composite scores were 97.05 (9.85) and 101.28 (10.87), P=0.039, respectively. The mean difference (95% CI) was 4.23 (0.23-8.23). However, 7 (17.9%) participants had decreased cognitive composite scores after anesthesia exposure. Conclusions: Children in the anesthesia group had lower baseline cognitive composite scores than those in the control group. The post-anesthesia cognitive score did not decrease compared with the baseline assessment. Anesthetic exposure resulted in a decline in the cognitive composite score in 17.9% of the participants.

Incidences and outcomes of intra-operative vs. postoperative paediatric cardiac arrest: A retrospective cohort study of 42 776 anaesthetics in children who underwent noncardiac surgery in a Thai tertiary care hospital.

BACKGROUND: The reported incidence of paediatric perioperative cardiac arrest (PPOCA) in most developing countries ranges from 2.7 to 22.9 per 10 000 anaesthetics, resulting in mortality rates of 2.0 to 10.7 per 10 000 anaesthetics. The definitions of 'peri-operative' cardiac arrest often include the intra-operative period and extends from 60 min to 48 h after anaesthesia completion. However, the characteristics of cardiac arrests, care settings, and resuscitation quality may differ between intra-operative and early postoperative cardiac arrests. OBJECTIVE: To compare the mortality rates between intraoperative and early postoperative cardiac arrests (<24 h) following anaesthesia for paediatric noncardiac surgery. DESIGN: A retrospective cohort study. SETTING: In a tertiary care centre in Thailand during 2014 to 2019, the peri-operative period was defined as from the beginning of anaesthesia care until 24 h after anaesthesia completion. PATIENTS: Paediatric patients aged 0 to 17 years who underwent anaesthesia for noncardiac surgery. MAIN OUTCOME MEASURES: Mortality rates. RESULTS: A total of 42 776 anaesthetics were identified, with 63 PPOCAs and 23 deaths (36.5%). The incidence (95% confidence interval) of PPOCAs and mortality were 14.7 (11.5 to 18.8) and 5.4 (3.6 to 8.1) per 10 000 anaesthetics, respectively. Among 63 PPOCAs, 41 (65%) and 22 (35%) occurred during the intra-operative and postoperative periods, respectively. The median [min to max] time of postoperative cardiac arrest was 3.84 [0.05 to 19.47] h after anaesthesia completion. Mortalities (mortality rate) of postoperative cardiac arrest were significantly higher than that of intra-operative cardiac arrest at 14 (63.6%) vs. 9 (22.0%, P = 0.001). Multivariate analysis of risk factors for mortality included emergency status and duration of cardiopulmonary resuscitation with adjusted odds ratio 5.388 (95% confidence interval (1.031 to 28.160) and 1.067 (1.016 to 1.120). CONCLUSIONS: Postoperative cardiac arrest resulted in a higher mortality rate than intra-operative cardiac arrest. A high level of care should be provided for at least 24 h after the completion of anaesthesia. TRIAL REGISTRATION: None. CLINICAL TRIAL NUMBER AND REGISTRY URL: NA.

Risk of Hypoxemia by Induction Technique Among Infants and Neonates Undergoing Pyloromyotomy.

BACKGROUND: In patients presenting for pyloromyotomy, most practitioners prioritize rapid securement of the airway due to concern for aspiration. However, there is a lack of consensus and limited evidence on the choice between rapid sequence induction (RSI) and modified RSI (mRSI). METHODS: The medical records of all patients presenting for pyloromyotomy from May 2012 to December 2018 were reviewed. The risk of hypoxemia (peripheral oxygen saturation [Spo2], <90%) during induction was compared between RSI and mRSI cohorts for all patients identified as well as in the neonate subgroup by univariate and multivariable logistic regression analysis. Complications (aspiration, intensive care unit admission, bradycardia, postoperative stridor, and hypotension) and initial intubation success for both cohorts were also compared. RESULTS: A total of 296 patients were identified: 181 in the RSI and 115 in the mRSI cohorts. RSI was associated with significantly higher rates of hypoxemia than mRSI (RSI, 30% [23%-37%]; mRSI, 17% [10%-24%]; P = .016). In multivariable logistic regression analysis of all patients, the adjusted odds ratio (OR) of hypoxemia for RSI versus mRSI was 2.8 (95% confidence interval [CI], 1.5-5.3; P = .003) and the OR of hypoxemia for multiple versus a single intubation attempt was 11.4 (95% CI, 5.8-22.5; P < .001). In multivariable logistic regression analysis of neonatal subgroup, the OR of hypoxemia for RSI versus mRSI was 6.5 (95% CI, 2.0-22.2; P < .001) and the OR of hypoxemia for multiple intubation versus single intubation attempts was 18.1 (95% CI, 4.7-40; P < .001). There were no induction-related complications in either the RSI and mRSI cohorts, and the initial intubation success rate was identical for both cohorts (78%). CONCLUSIONS: In infants presenting for pyloromyotomy, anesthetic induction with mRSI compared with RSI was associated with significantly less hypoxemia without an observed increase in aspiration events. In addition, the need for multiple intubation attempts was a strong predictor of hypoxemia. The increased risk of hypoxemia associated with RSI and multiple intubation attempts was even more pronounced in neonatal patients.

The Performance of the Neck Circumference for a Difficult Laryngoscopy in Obese Patients.

BACKGROUND AND OBJECTIVE: There is conflicting evidence as to whether neck circumference (NC) is related to a difficult laryngoscopy in obese patients. The objectives of this study were to determine the performance of the NC in defining a difficult laryngoscopy, and to identify factors predicting a difficult laryngoscopy among those obese patients. MATERIAL AND METHOD: This prospective study was conducted in adult patients with a body mass index (BM) of greater or equal to 30 kg/m² who underwent conventional endotracheal intubation. Preoperative NC, the modified Mallampati test (MMT), the thyromental distance (TM) and the sternomental distance (SM) were measured. A difficult laryngoscopy was defined as a Cormack-Lehane laryngoscopic view of grade III or IV. RESULTS: Five hundred patients (366 females; 134 males), with a mean BMI of 34.3 ± 4.6 kg/m² (minimum = 30.0 kg/m², and maximum = 68.4 kg/m²) and who had elective surgery planned, were enrolled. The incidence of a difficult laryngoscopy was 7.2%. The overall performance of the NC using the area under the receiver operating characteristic curves of a difficult laryngoscopy was 0.574, with a 95% confidence interval (CI) of 0.53 to 0.618. The optimal cutoff point of the NC for a difficult laryngoscopy was 37.1 centimeters. It provided sensitivity and specificity of 75.5% (95% CI 57.8, 87.9) and 42.5% (95% CI 37.9, 47.2). The positive and negative predictive values were 9.2% and 95.6%. After multivariate analysis, factors affecting a difficult laryngoscopy were high grades of MMT (odds ratio (OR) 2.23, 95% CI 1.10, 4.50) and NC/TM (OR 1.78, 95% CI 1.15, 2.74). CONCLUSION: The NC per se was not a good predictor of a difficult laryngoscopy. However the ratio of the NC to the TM and the MMT were factors predicting a difficult laryngoscopy in obese patients.

Perioperative glycaemic control for preterm infant with transient neonatal hyperglycaemia and gastroschisis.

BACKGROUND: Neonatal hyperglycaemia is a rare metabolic disorder. There are no reports of an association between neonatal hyperglycaemia and gastroschisis. CASE PRESENTATION: This report presents preoperative and intraoperative management of blood sugar in a low birth weight Thai preterm neonate with gastroschisis and a diagnosis of neonatal hyperglycaemia. The patient underwent an emergency, multi-staged, surgical repair under general anaesthesia. CONCLUSION: Anaesthesiologists should be aware of possible perioperative dysglycaemic conditions in these patients. Proper timing of surgery and appropriate preanaesthetic preparation are necessary to reduce the morbidity and mortality related to hyperglycaemia and gastroschisis. CONSENT: The patient's guardian has given consent for the case report to be published.

Appropriate Size of Laryngeal Mask Airway in Thai Children: Is It Age or Weight that Better Correlated.

Background: Clinicians are divided regarding laryngeal mask airway (LMA) size selection for pediatric patients, primarily whether age or body weight is an appropriate predictor. Objective: Determine if body weight and/or age are reliable predictors in LMA size selection in Thai children. Material and Method: Retrospective review of patient medical records was conducted between October 1, 2012 and December 31, 2013. Seven hundred forty one patients, aged 0 to 8 years, were evaluated for the present study. Age and weight correlation for LMA size selection was determined. Appropriate LMA size was identified by successful LMA insertion after induction of anesthesia. Results: Our findings indicate that size 1 LMA for patients weighing less than 7 kilograms, size 1.5 LMA for patients weighing 7 to 11 kilograms, size 2 LMA for patients weighing 11 to 17 kilograms, and size 2.5 LMA for patients weighing 15 to 28 kilograms is appropriate. There was poor correlation between children's age and LMA size. Conclusion: LMA size selection should be based on patient's weight. Our findings revealed applicable and specific weight range for LMA selection in Thai pediatric patients

Evaluating compliance with institutional preoperative testing guidelines for minimal-risk patients undergoing elective surgery.

BACKGROUND: Few investigations preoperatively are important for low-risk patients. This study was designed to determine the level of compliance with preoperative investigation guidelines for ASA I patients undergoing elective surgery. Secondary objectives included the following: to identify common inappropriate investigations, to evaluate the impact of abnormal testing on patient management, to determine factors affecting noncompliant tests, and to estimate unnecessary expenditure. METHODS: This retrospective study was conducted on adult patients over a one-year period. The institute's guidelines recommend tests according to the patients' age groups: a complete blood count (CBC) for those patients aged 18-45; CBC, chest radiograph (CXR) and electrocardiography (ECG) for those aged 46-60; and CBC, CXR, ECG, electrolytes, blood glucose, blood urea nitrogen (BUN), and creatinine (Cr) for patients aged 61-65. RESULTS: The medical records of 1,496 patients were reviewed. Compliant testing was found in only 12.1% (95% CI, 10.5-13.9). BUN and Cr testings were the most frequently overprescribed tests. Overinvestigations tended to be performed on major surgery and younger patients. Overall, overinvestigation incurred an estimated cost of US 200,000 dollars during the study period. CONCLUSIONS: The need to utilize the institution's preoperative guidelines should be emphasized in order to decrease unnecessary testing and the consequential financial burden.