No abstract

These findings suggest that mechanical factors during embryonic life could explain the strong association between left-sided Poland syndrome and dextrocardia. According to this hypothesis, partial agenesis of 2 or more ribs is needed to displace the heart toward the right side. The peculiar features of dextrocardia when associated with Poland syndrome (neither associated with situs inversus nor complex intracardiac anomalies) support our hypothesis.

Background: Poland syndrome (OMIM: 173800) is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, breast, shoulder, arm, and hand. The extent and severity of the abnormalities vary among affected individuals. Main body: The aim of this work is to provide recommendations for the diagnosis and management of people affected by Poland syndrome based on evidence from literature and experience of health professionals from different medical backgrounds who have followed for several years affected subjects. The literature search was performed in the second half of 2019. Original papers, meta-analyses, reviews, books and guidelines were reviewed and final recommendations were reached by consensus. Conclusion: Being Poland syndrome a rare syndrome most recommendations here presented are good clinical practice based on the consensus of the participant experts.

Sciatic nerve injury caused by intramuscular injection in the gluteal region in the child seems as a sensory-motor palsy of the lower limb of variable degree. In preterm children or in children with severe perinatal distress, requiring intensive care, a drop foot is often missed or misdiagnosed as a malformative clubfoot or late diagnosed. Intramuscular drug injection (mainly antibiotics) during early infancy is another cause of injury. There are very few literature reports on postinjection trauma and on therapeutic indications in the child. The Authors report their experience in early microsurgical exploration of the sciatic nerve. From 1990 to 2004, we observed at different times from diagnosis 17 children with sciatic nerve palsy following intramuscular injection. Nine of them underwent nerve exploration surgery in the gluteal region (neurolysis in seven and nerve grafting in two). Conservative treatment was successful in only three cases showing early signs of recovery (at about 3 months of life). Complete recovery was observed only in five early treated cases, while late treated cases had only mild improvement after surgery. During surgery, anatomical variations predisposing to nerve injury were observed. The authors havingobserved better results and faster recovery in the early treated patients, stress the importance of a rapid therapeutic decision to avoid or limit foot deformities, sensory defects and lower limb length discrepancy due to paralysis during growth.