The Orthopaedic Management of Human Disorganization Syndrome.

Human disorganization syndrome (HDS) is an extremely rare congenital syndrome characterized by a seemingly random distribution of multiple developmental anomalies involving all three germinal layers. Case Report: We report a rare case of a female child whose congenital anomalies are consistent with HDS. The orthopaedic features of this patient include a popliteus pterygium with an associated flexion contracture secondary to an elongated biceps femoris tendon that attached to the gastrocnemius-soleus muscle complex, two finger-like appendages, a tethered cord, a lipomeningomyelocele at the level of L5, and a leglength discrepancy. The patient was treated with a splinting program, release of the biceps femoris tendon at its erroneous insertion from the gastrocs-soleus, and surgical excision of the finger-like appendages. She underwent three subsequent soft-tissue releases to address recurrence of the knee flexion contracture and an anteromedial and lateral distal femoral eight plate procedure for guided growth and slow correction of the remaining flexion deformity. Conclusion: The treatment of HDS can be quite complex and can present with a variety of anomalies with distinctive orthopaedic features correctable with surgical management, including soft-tissue releases, excision of appendages, and growth modulation.

Pediatric Burns from Glass-Fronted Fireplaces in Canada: A Growing Issue Over the Past 20 Years.

There is an alarming lack of public awareness surrounding the safety of glass-fronted fireplaces. This has resulted in an active campaign from the American Burn Association Prevention Committee. One issue encountered while advocating for prevention among manufacturers is the lack of corroborating and accurate data. The purpose of this study was to examine the annual trends and epidemiology of glass-fronted fireplace-related burn injuries to children less than 15 years old, who presented to Canadian emergency departments between 1990 and 2010. Records of pediatric burn injuries related to glass-fronted fireplaces were extracted from the Canadian Hospitals Injury Reporting and Prevention Program database for the study period (1990-2010). Cases were analyzed in terms of anatomic area affected, demographics, seasonality, safety device use, and injury severity. A total of 616 cases of burns from glass-fronted fireplaces were identified. The incidence increased at an average of 2.7 cases per year. This is a greater than 20-fold increase over 20 years. Seventy-five percentage of the cases occurred in children less than 2 years, and 95% occurred in children less than 5 years. The study demonstrated a growing risk from glass-fronted fireplace burns, likely due to the increasing popularity of household gas fireplace units. These units are a particular risk to children less than 2 years, attributable to their developing mobility and reduced reaction time. This is a preventable injury that should be addressed through changes to legislation and manufacturing.

Modified slide tracheoplasty in a newborn with bronchial and carinal stenosis.

Congenital tracheal stenosis (CTS) is a life-threatening condition that is associated with significant morbidity and mortality particularly when symptomatic presentation occurs in the newborn period. The most challenging form of CTS is long segment congenital tracheal stenosis (LSCTS) with compromise of the carina and main stem bronchi. We report the case of a newborn with severe distal tracheal stenosis with carinal and main stem bronchial involvement who was managed successfully with a modified slide and autologous rib graft tracheoplasty. The patient was discharged from hospital without ventilator support or oxygen requirement at 2 months of age. The details of this case and the description of the surgical procedure are presented and the related literature is reviewed.