Bojanić, Katarina

Objective Reference values of regional cerebral oxygen saturation (rScO 2 ) in preterm neonates across gestational age (GA) groups remain undefined. This study aimed to establish rScO 2 references for neonates born before 32 weeks of gestation. Design A prospective observational cohort study. Setting Large tertiary neonatal referral centre in Croatia. Patients Preterm neonates without major comorbidities born between 2018 and 2022 with normal cranial ultrasound findings. Interventions rScO 2 was assessed with near-infrared spectroscopy (NIRS) using neonatal sensors. Main outcome measures The primary analysis provided rScO 2 reference ranges for the entire preterm neonatal cohort, followed by stratified analyses based on GA: extremely preterm , <28.0 weeks; very preterm , 28.0–30.0 weeks; and very preterm , 30.1–32.0 weeks. Quantile regression was used to estimate the selected rScO 2 percentiles (5th, 10th, 50th, 90th and 95th) from 6 to 72 hours after birth. Results 114 neonates with median (IQR) GA of 29.9 (29.1–31.5) weeks were included: 22% were EP <28w , 29% were VP 28–30w and 49% were VP >30–32w . The median rScO 2 was 75.6% at 6 hours, 79.5% over the first 24 hours and exceeding 80% in the subsequent 48 hours. When analysed separately, notable differences emerged across prematurity categories, with the highest median rScO 2 observed in the VP >30–32w group. Supplementary analyses, comparing rScO 2 values across the three GA groups, revealed significant differences at 48 and 72 hours. The majority of the estimated rScO 2 fifth percentiles between 6 and 72 hours were in the range ≥60%, with a single lowest estimate of 56.3% at 6 hours in the EP <28w group. Thus, for all subgroups, the estimated fifth percentile of rScO 2 was above the brain hypoxic threshold. Conclusions The use of age-appropriate neonatal NIRS sensors provided the rScO 2 reference values for very and extremely preterm neonates without major comorbidities 6–72 hours after birth.

Background Congenital diaphragmatic hernia (CDH) has different clinical presentations depending on whether it is right sided (R-CDH) or left sided (L-CDH). Some have suggested that L-CDH and R-CDH may represent different syndromic phenotypes. This theory would be indirectly supported if different nondiaphragmatic anomalies were associated with laterality. We assessed whether CDH laterality is associated with specific types of nondiaphragmatic anomalies. Methods Cases of CDH were retrospectively identified from five centers, and associated congenital anomalies, prenatal diagnosis, demographics, birth characteristics, and side of the CDH were analyzed. CDH characteristics were summarized according to the absence (isolated) or presence (complex) of nondiaphragmatic malformations. Results Among 228 neonates with CDH, 140 (61%) had isolated CDH and 88 (39%) had complex CDH. Complex CDH was significantly associated with being small for gestational age (odds ratio [95% confidence interval, CI]: 8.3 [1.9–35.7]; p = 0.005) and having L-CDH (odds ratio [95% CI]: 3.6 [1.5–8.9]; p = 0.005). The overall proportion with anomalies differed by side (42% for L-CDH, 23% for R-CDH; p = 0.02), but the rates of anomalies in specific organ systems did not differ. Conclusion The rate of associated nondiaphragmatic anomalies by specific organ system did not differ between L-CDH and R-CDH, which suggests that they represent the same phenotypic entity.

SummaryBackgroundCongenital diaphragmatic hernia (CDH) is a rare anomaly with high mortality and long‐term comorbid conditions.AimsOur aim was to describe the presenting characteristics, treatment, and outcomes of consecutive patients with CDH treated at our institution.MethodsWe performed a retrospective cohort study and identified consecutive neonates treated for CDH from 2001 to 2015 at our institution. For all patients identified, we reviewed hospital and postdischarge data for neonatal, disease, and treatment characteristics. We determined hospital survival overall and also according to the presence of prenatal diagnosis, liver herniation into the chest (liver up), and the use of extracorporeal membrane oxygenation (ECMO) in addition to surgery. We evaluated postdischarge chronic conditions in patients with at least one year of follow‐up.ResultsThirty‐eight neonates were admitted for treatment during the study period. In three who were in extremis, life support was withdrawn. The other 35 underwent surgical repair, of whom eight received ECMO. The overall survival was 79% (30/38). Survival for those who had surgical correction of CDH but did not need ECMO was 89% (24/27); it was 75% (6/8) for those who received ECMO and had surgery. Hospital survival was lower for liver‐up vs liver‐down CDH (61% [11/18] vs 95% [19/20]; odds ratio, 0.08; 95% CI, 0.01–0.77; P = 0.01). Among survivors, the median duration of hospitalization was 31 (interquartile range, 20–73) days. Major chronic pulmonary and gastrointestinal disorders, failure to thrive, and neurodevelopmental delays were the most noted comorbid conditions after discharge, and all were more prevalent in those who required ECMO.ConclusionThe overall survival of neonates with CDH was 79%. Intrathoracic liver herniation was associated with more frequent use of ECMO and greater mortality. A substantial number of survivors, especially those who required ECMO, experienced chronic conditions after discharge.

AbstractBackgroundOutcomes of neonates with congenital diaphragmatic hernia (CDH) are variable; reports are frequently limited to the experience of single tertiary care centres—a possible source of bias. Population‐based studies decrease survivor bias and provide additional insight into this high‐mortality condition. The objective of this study was to examine the incidence and outcomes of CDH in Croatia.MethodsAll cases of CDH in Croatia from 2001 through 2013 were ascertained from public health records. Overall and sex‐ and region‐specific incidence rates were calculated, and characteristics associated with 1‐year survival were assessed.ResultsWe identified 145 cases of CDH during the study period, for an incidence of 2.67 per 10 000 total births. The incidence did not differ by calendar year (P = 0.38) or geographic region (P = 0.67). There was a slightly higher incidence among males (rate ratio, 1.37, 95% CI 0.99, 1.91). The 1‐year survival rate was 33.1% for the entire cohort and 47.9% for liveborns who received any treatment at an intensive care unit. From multivariable analysis, survival was decreased in neonates with left CDH, liver up (odds ratio 0.1, 95% CI, 0.03, 0.4) and increased when treated in a centre with higher case volume (odds ratio 12.8, 95% CI, 2.2, 72.1).ConclusionsThe incidence of CDH in Croatia is within the range of previous reports. Survival was substantially higher in neonates treated in a centre with higher case volume, which suggests that centralisation of medical care for CDH may be warranted in Croatia.

Summary Background Duchenne muscular dystrophy ( DMD ) and Becker muscular dystrophy ( BMD ) are associated with life‐threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial. Objective To review the perioperative outcomes of patients with DMD and BMD . Methods We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011. Results We identified 47 patients ( DMD , 37; BMD , 10) who underwent 117 anesthetic exposures ( DMD , 101; BMD , 16). Volatile anesthetic agents were used 66 times ( DMD , 59; BMD , 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both. Conclusions Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin‐deficient children.

Background Congenital hyposensitivity to pain or hereditary sensory and autonomic neuropathy represents a variety of disorders characterized by decreased perception of nociception, loss of other modalities of sensation, and variable expression of autonomic dysfunction. Sensory loss, especially that of pain, is associated with self-mutilations that may require frequent operations. Little is known about the safety of anesthesia for these patients. Methods The authors performed a computerized search of the Mayo Clinic medical records database between January 1996 and November 2005 for patients with congenital hyposensitivity to pain and related disorders who underwent general anesthesia. Medical records were reviewed for demographics, anesthetic techniques and agents, use of opioids, and perioperative complications. In addition, the authors conducted a comprehensive review of the literature to summarize the current knowledge regarding anesthesia for patients with congenital hyposensitivity to pain, and compared it with the patients with hyposensitivity to pain identified at the Mayo Clinic. Results The authors identified seven patients with hereditary sensory and autonomic neuropathy II, IV, or V and undefined variants of congenital pain hyposensitivity who generated 17 anesthesia records: 12 for orthopedic operations, 3 for sural nerve biopsies, and 2 for ophthalmologic procedures. In all patients, standard doses of volatile agents were used during anesthesia. Small amounts of opioids were used during the course of eight operations. Most patients experienced mild hypothermia (lowest temperature 34.7 degrees C), and none experienced hyperthermia. All patients were hemodynamically stable during otherwise uneventful anesthesia. During recovery from anesthesia, opioids were given to only one patient, a single dose of 1 mg morphine. Even after major orthopedic operations, the patient did not require additional analgesia. Conclusions The patients with profound congenital hyposensitivity to pain underwent anesthesia without any adverse events. The authors found that despite reduced pain perception, the requirements for volatile anesthetics were within the expected range for population with normal pain perception, but they did not require opioids postoperatively. Intraoperative mild hypothermia was easily managed by adjustment of environmental temperature.