Management of Craniosynostosis in Lethal Perinatal Hypophosphatasia.

Although perinatal lethal hypophosphatasia (HPP) was once a disease with a universally poor prognosis, it has now become a rare but treatable condition with the advent of enzyme replacement therapy with asfotase alfa. As a result, a greater population of patients with perinatal HPP are presenting with abnormal head shape and craniosynostosis. The authors present here 3 cases of perinatal lethal HPP, 1 treated with traditional open cranial vault remodeling and 2 treated utilizing distraction osteogenesis techniques. All patients demonstrated outcomes comparable to those previously reported with traditional observation or open cranial vault repair. Thorough consideration and discussion between the surgical team and patient's family is needed to determine a treatment plan that best addresses the goals of patient and family in light of recent advances in medical treatment in this rare patient population in which surgical interventions were previously nearly impossible. This article further supports the safety and efficacy of surgical intervention and explores the utility of distraction osteogenesis to address craniosynostosis in this patient population.

Contemporary concepts for the bilateral cleft lip and nasal repair.

The bilateral cleft lip and nasal deformity presents a complex challenge for repair. Surgical techniques continue to evolve and are focused on primary anatomic realignment of the tissues. This can be accomplished in a single-stage or two-stage repair early in infancy to provide a foundation for future growth of the lip and nasal tissue. Most cleft surgeons currently perform a single-stage repair for simplifying patient care. Certain institutions utilize presurgical orthopedics for alignment of the maxillary segments and nasal shaping. Methods for the bilateral cleft lip repair are combined with various open and closed rhinoplasty techniques to achieve improved correction of the primary nasal deformity. There is recent focus on shaping the nose for columellar and tip support, as well as alar contour and alar base position. The authors will present a new technique for closure of the nasal floor to prevent the alveolar cleft fistula. Although the alveolar fistula is closed, alveolar bone grafting is still required at the usual time in dental development to fuse the maxilla. It is paramount to try and minimize the stigmata of secondary deformities that historically have been characteristic of the repaired bilateral cleft lip. A properly planned and executed repair reduces the number of revisions and can spare a child from living with secondary deformities.

Design, development, and validation of a take-home simulator for fundamental laparoscopic skills: using Nintendo Wii for surgical training.

Assuring quality surgical trainees within the confines of reduced work hours mandates reassessment of educational paradigms. Surgical simulators have been shown to be effective in teaching surgical residents, but their use is limited by cost and time constraints. The Nintendo Wii gaming console is inexpensive and allows natural hand movements similar to those performed in laparoscopy to guide game play. We hypothesize that surgical skills can be improved through take-home simulators adapted from affordable off-the-shelf gaming consoles. A total of 21 surgical residents participated in a prospective, controlled study. An experimental group of 14 surgical residents was assigned to play Marble Mania on the Nintendo Wii using a unique physical controller that interfaces with the WiiMote controller followed by a simulated electrocautery task. Seven residents assigned to the control group performed the electrocautery task without playing the game first. When compared with the control group, the experimental group performed the task with fewer errors and superior movement proficiency (P < 0.05). The experimental group demonstrated increased ambidexterity with improvement in proficiency of the nondominant hand over time. In conclusion, the Nintendo Wii gaming device along with Marble Mania serves as an effective take-home surgical simulator.