Successful staged separation of total angular craniopagus.

BACKGROUND: Total craniopagus is an exceedingly rare condition in which surgical treatment is complex and potentially fatal. Over the last decades, a multistep surgical approach, which allows development of venous collateral circulation, has fostered a dramatic improvement of successful separation rates and neurological outcomes. Most of the experience derives from management of vertical craniopagus, the angular form being rarer and less amenable to successful surgical separation. METHODS: We present a case of total angular craniopagus twins observed at our Institution. Specific features included a large occipital fusion area with a bone defect, complete separation of brain and arterial vessels and a complex configuration of dural venous sinuses. The superior sagittal sinus of each twin preferentially drained to a single transverse sinus through a shared torcular. RESULTS: After an extensive diagnostic phase, including neuroimaging, tridimensional and virtual reality modelling, neurological, neurophysiological and rehabilitation assessment, a detailed multistep surgical plan, was proposed to a wide multidisciplinary team. The venous system was managed by taking advantage of the fact each twin's superior sagittal sinus was drained preferentially by the transverse sinus on the twin's left. The transverse sinuses were thus separated accordingly. CONCLUSIONS: Successful separation was achieved in three surgical steps over one year, with an excellent outcome for both twins.

Pediatric Mechanical Circulatory Support: Pathophysiology of Pediatric Hemostasis and Available Options.

Pediatric mechanical circulatory support (MCS) is considered a strategy for heart failure management as a bridge to recovery and transplantation or as a destination therapy. The final outcome is significantly impacted by the number of complications that may occur during MCS. Children on ventricular assist devices (VADs) and extracorporeal membrane oxygenation (ECMO) are at high risk for bleeding and thrombotic complications that are managed through anticoagulation. The first detailed guideline in pediatric VADs (Edmonton Anticoagulation and Platelet Inhibition Protocol) was based on conventional antithrombotic drugs, such as unfractionated heparin (UFH) and warfarin. UFH is the first-line anticoagulant in pediatric MCS, although its profile is not considered optimal in pediatric setting. The broad variation in heparin doses among children is associated with frequent occurrence of cerebrovascular accidents, bleeding, and thrombocytopenia. Direct thrombin inhibitors (DTIs) have been utilized as alternative strategies to heparin. Since 2018, bivalirudin has become the chosen anticoagulant in the long-term therapy of patients undergoing MCS implantation, according to the most recent protocols shared in North America. This article provides a review of the non-traditional anticoagulation strategies utilized in pediatric MCS, focusing on pharmacodynamics, indications, doses, and monitoring aspects of bivalirudin. Moreover, it exposes the efforts and the collaborations among different specialized centers, which are committed to an ongoing learning in order to minimize major complications in this special pediatric population. Further prospective trials regarding DTIs in a pediatric MCS setting are necessary and in specific well-designed randomized control trials between UFH and bivalirudin. To conclude, based on the reported literature, the clinical use of the bivalirudin in pediatric MCS seems to be a value added in controlling and maybe reducing thromboembolic complications. Further research is necessary to confirm all the results provided by this literature review.

Can visual inspection of the electrical activity of the diaphragm improve the detection of patient-ventilator asynchronies by pediatric critical care physicians?

BACKGROUND: Patient-ventilator asynchronies are challenging during pediatric mechanical ventilation. We hypothesized that monitoring the electrical activity of the diaphragm (EAdi) together with the "standard" airway opening pressure (Pao) and flow-time waveforms during pressure support ventilation would improve the ability of a cohort of critical care physicians to detect asynchronies in ventilated children. METHODS: We recorded the flow, Pao and EAdi waveforms in ten consecutive patients. The recordings were split in periods of 15 s, each reproducing a ventilator screenshot. From this pool, a team of four experts selected the most representative screenshots including at least one of the three most common asynchronies (missed efforts, auto-triggering and double triggering) and split them into two versions, respectively showing or not the EAdi waveforms. The screenshots were shown in random order in a questionnaire to sixty experienced pediatric intensivists that were asked to identify any episode of patient-ventilator asynchrony. RESULTS: Among the ten patients included in the study, only eight had EAdi tracings without artifacts and were analyzed. When the Eadi waveform was shown, the auto-triggering detection improved from 13% to 67% (P<0.0001) and the missed efforts detection improved from 43% to 95% (P<0.0001). The detection of double triggering, instead, did not improve (85% with the EAdi vs. 78% without the EAdi waveform; P=0.52). CONCLUSIONS: This single center study suggests that the EAdi waveform may improve the ability of pediatric intensivists to detect missed efforts and auto-triggering asynchronies. Further studies are required to determine the clinical implications of these findings.

Intranasal dexmedetomidine in pediatrics: update of current knowledge.

Intranasal dexmedetomidine, although still off-label, recently boasted an increasing consensus for different uses, namely, in diagnostic non-painful procedures, in painful procedures and in surgical premedication. However, at present, there is no consensus regarding indications, dosage and timing for administration. This article aims to provide a comprehensive literature analysis and summarize the more recent evidence of research on pediatric intranasal dexmedetomidine, in the effort to better delineate usefulness and limits for each specific indication. In summary, available pediatric evidence confirms efficacy and safety of dexmedetomidine for intranasal administration. Pharmacological profile for the various pediatric ages and procedures still needs quality studies and pharmacokinetic in-depth analysis.

Comparison of regional vs. systemic analgesia for post-thoracotomy care in infants.

BACKGROUND: In infants, post-thoracotomy analgesia traditionally consists of systemic opiates, while regional techniques have gained more favor in recent years. We compare the two techniques for thoracotomy in infants. METHODS: All consecutive patients below 6 months of age who underwent thoracotomy for congenital pulmonary malformations in the study period were retrospectively divided according to the chosen postoperative analgesia: Group S systemic opiates, Group R continuous regional (epidural or extrapleural paravertebral) block. We studied the following outcomes: need for NICU and mechanical ventilation, pain score, requirement for additional analgesics, heart rate 1 h postsurgery, time to pass first stool and to full feed, complications, and duration of hospitalization. RESULTS: Forty consecutive patients were included, 19 in Group S and 21 in Group R. Median age at surgery was 89 days (40-110) and 90 days (46-117), respectively. Five of 19 patients in Group S vs none in Group R required postoperative intensive care (P = 0.017). Patients in Group R had significantly lower postoperative heart rate (145 [138-150] vs. 160 [152-169] b·min(-1) , P = 0.007), earlier passage of first stools (24 h [12-24] vs. 36 h [24-48] P = 0.004), and earlier time to full feed (36 h [24-48] vs. 84 h [60-120] P = 0.0001) than those in Group S. The only observed complication was one catheter dislocation. CONCLUSION: In infants undergoing thoracotomy, loco-regional analgesia is effective and associated with a reduced intensity of postoperative care and earlier full feeding than systemic analgesia; it should therefore be considered a better option.