A preterm female infant was admitted at birth with respiratory distress. On examination, she had an asymmetric right chest wall and ipsilateral small hand. Air entry was reduced over the right chest. A clinical diagnosis of Poland’s syndrome was made based on the hypoplasia of the right pectoral muscles, absent nipple, deformed ribs and symbrachydactyly of the ipsilateral hand. Chest X-ray suggested and ultrasound confirmed eventration of the right hemidiaphragm. ‘Subclavian artery supply disruption sequence’ (SASDS) theory by Bavnick and Weaver remains the most accepted pathogenic mechanism in Poland’s syndrome. This case reinforces SASDS theory associated with the genesis of Poland’s syndrome that relates to the pathogenicity of vascular disruption of subclavian artery, characteristics of which are unilateral pectoral defects, symbrachydactyly and eventration of the diaphragm. At 2 months, she underwent diaphragm plication. She is under review by our multidisciplinary surgical team for reconstruction of the chest deformity.
tertiary care hospital in Eastern India. The sample size was 56, calculated on the basis of a similar pediatric RCT. Patient enrolment occurred between May 2019 and July 2020. Children aged between 1 month and 12 years with suspected septic shock were randomized to receive either ultrasound or clinically guided fluid boluses (in a 1:1 ratio) and subsequently followed up for primary and secondary outcomes. Exclusion criteria were Dengue, Anaphylaxis, Ascites, and patients with pre-existing chronic kidney disease, interstitial lung disease, heart disease, and adrenal insufficiency. Ultrasound was used in the treatment group whenever there was clinical suspicion of inadequate perfusion. Results 68 children were enrolled in the study. 4 patients of Dengue and 4 patients who died within 24 hours were excluded. The number (%) of patients with fluid overload on day 3 of admission was significantly lower in the ultrasound group (25% vs. 62%, p=0.012) as was the CFB% on day 3 (6.8±6.6% vs. 13.4±10.7%, p=0.019). Total fluid bolus was significantly lower {median of 40(30-50) ml/kg vs. 50 (40-80) ml/kg, p=0.003}. Resuscitation time was significantly lower in the ultrasound group (13.4 ± 5.6h vs. 20.5 ± 8h, p=0.002) and so was the requirement of Furosemide (39.3% vs. 71.4%, p=0.016). None of the deaths in the ultrasound group were due to unresolved shock (p=0.101). There was no significant benefit derived from ultrasound in terms of ventilator duration, inotrope/vasopressor requirement, length of PICU/hospital stay, and mortality. Conclusions Due to a lack of comparative studies on this topic, our study adds insight into the utility of ultrasound in improving outcomes in septic shock. Ultrasound made the PICU physician exercise restraint in administering fluid boluses and allowed earlier initiation of inotropes. Ultrasound was found to be significantly better than clinically guided therapy, in preventing fluid overload as well as being quicker in achieving initial hemodynamic stabilization. Hence ultrasound is a potentially useful tool for fluid resuscitation in children with septic shock.
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