Poland syndrome (PS) has been described as unilateral pectoral muscle deficiency variably associated with ipsilateral thoracic and upper limb anomalies. Bilateral hypoplasia/aplasia of the pectoralis muscle and upper limb defects in association with variable thoracic muscles, chest wall deformities and lower limb defects have been infrequently reported in the literature. We report on a 3(1/2)-year-old girl with clinical features consisting in bilateral asymmetric pectoral muscle defects (complete agenesis on the left side and agenesis of the sternocostal head on the right side), nipple hypoplasia, left rib defect, and right hand symbrachydactyly. In this study, we reviewed the bilateral features present in our patient and those described in the literature. Hypotheses explaining bilateral features in PS are reviewed.

Myofibromatosis is an uncommon disorder of infancy, characterized by proliferation of myofibroblasts in solitary or multiple nodules. The clinical characteristics depend on the involved sites: Myofibromatosis may develop as a musculoskeletal form, with non-painful swellings and eventual mass effect symptoms, or as a generalized form with visceral involvement and organ failure. Prognosis and therapy vary between the abovementioned patterns. When there is no visceral involvement, the tumors may regress spontaneously; however, the visceral form may represent a lifethreatening condition with poor outcome and it requires aggressive management. Imaging assessment of disease spread is mandatory to determine diagnosis, prognosis and therapy. Due to the young age of the patients, a radiation-free evaluation is recommended. We herein describe a case of musculoskeletal myofibromatosis diagnosed in a 3-month-old male infant, investigated by serial wholebody magnetic resonance imaging (MRI) examination. The histological analysis and MRI characteristics enabled a correct diagnosis and organ involvement assessment with no radiation exposure. Moreover, whole-body MRI sequences provided a detailed evaluation of the disease within a short time frame, reducing the time of sedation, which is required to perform MRI in very young patients. Therefore, whole-body MRI was found to be accurate and safe in the diagnosis and follow-up of multicentric infantile myofibromatosis

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Scaphoid nonunion followed by necrosis of bone segments is a common pathologic condition for the hand surgeon, and the difficulty of its management is well known. The total titanium scaphoid replacement, although not well-described in the literature, in our experience represents a reasonable choice in the treatment of this condition. Strict patient selection is necessary to achieve good clinical results. The titanium avoids the silicone synovitis, a well-described complication of silastic implants. Furthermore, this technique permits other surgical steps in case of failure.

Knowledge of MRI findings of lingual venous malformations is useful for differential diagnosis with other high intensity lingual lesions on T(2) weighted images. This discrimination is achievable using iv paramagnetic contrast medium.

A 34-year-old male patient developed scaphoid nonunion with necrosis in both fragments. A scaphoid replacement was performed using a titanium custom-made 3D-printed implant. At 1-year follow-up, good clinical and radiographic outcome was obtained. Titanium custom-made 3D-printed implants may offer a good surgical solution for patients requiring total scaphoid replacement.

Following wrist trauma due to a karate accident, an 18-year-old male patient developed non-union of the scaphoid with proximal pole necrosis and significant scapholunate gap from an evident scapholunate interosseous ligament (SLIL) injury. A custom-made 3D-printed titanium implant was used for scaphoid replacement and SLIL reconstruction. Good clinical and radiographic outcomes were observed at his 2-year follow-up. Custom-made 3D-printed titanium implants are thought to provide a surgical solution for patients requiring site-specific scaphoid replacement while allowing the reconstruction of the scapholunate ligament.

Background: Although hand and upper limb malformations are quite frequent, up to now very few reports have been published on epidemiology. The aim of this study is to evaluate the number of infants who presented with hand and upper limb malformations from 2010 to 2015 in Italy. Methods: A retrospective analysis of a pediatric population presenting with hand and upper extremity malformations was carried out, gathering reports achieved from eight Italian Centers of pediatric hand surgery. Other factors such as gender, date and region of birth, family distribution of malformations and associated syndromes, were analysed. Results: Out of 3,100,421 live births, 765 children presented with hand and upper limb malformations. The incidence was 2,5/10,000 live births with a predominance of males and the right side. Radial polydactyly was the anomaly with the highest percentage, closely followed by simple syndactyly, simbrachidactyly and complex syndactyly. Less common conditions were the triphalangic thumb, thumb in palm, proximal radioulnar synostosis and Sprengel deformity. Inheritance of and familial predisposition to those malformations was recorded in 25 cases, while 84 children presented with syndromes related to hand anomalies. Conclusions: In conclusion the incidence of hand and upper extremity malformations in Italy is lower than that registered in other countries. The retrospective nature of the study combined with the fact that some defects frequently evade pediatric hand surgeon consultations are some possible limitations of the study. However, our data confirmed that, in spite of the decrease in the birth rate in Italy, the trend of congenital hand disorders maintained a stable trend. We aim to integrate this study with a prospective analysis and to involve the institutional health authorities in other countries so as to register the correct incidence of hand and upper extremity defects.