Macrodactyly is a rare condition in which fingers, hands or limb growth is unregulated, resulting in overgrowth of tissues in the affected extremities. It is critical to properly assess these extremities for signalling pathway, psychological impact and potential surgical intervention, to achieve the best possible outcome for each patient. Treatment approaches can vary, and patient and family expectations weigh heavily on care complexity. Common surgical procedures may include epiphysiodeses, osteotomies, debulking procedures, carpal tunnel releases, toe transfers and amputations. The selection and timing of these surgeries is a vital component of the approach, as delayed healing and excessive scarring may occur. The purpose of this review is to assist in the navigation of decision-making and surgical timing for patients presenting with overgrowth manifesting itself as macrodactyly.

Upper extremity involvement in patients with arthrogryposis multiplex congentia is quite frequent. Treatment initially consists of stretching and splinting as significant gains can be seen in the first years of life. The goal of any surgical procedure is to improve upper extremity function and performance of daily living activities, yet it is important to treat each patient individually and understand that areas do not always need to be addressed surgically. Despite overall lower functioning scores in this patient population, quality of life scores are comparable to the general aged adjusted population. This article will discuss the clinical presentation, treatment procedures and outcomes when addressing the upper extremities of patients presenting with arthrogryposis.

Level II: prospective cohort study.

Background: There is significant controversy in the literature over rates of late-developing dysplasia following normal screening ultrasound in breech babies, with reported rates varying from 7% to 29%. The purpose of this study is to investigate the rates of radiographic dysplasia in breech babies after a normal ultrasound with a minimum of 1 year of radiographic follow-up. Methods: This study was an institutional review board–approved prospective study of all patients referred by their pediatrician for concern for developmental dysplasia of the hip between July 2008 and August 2014. We identified all subjects with breech presentation and excluded those with an abnormal initial examination/ultrasound or with <12 months of radiographic follow-up. Anterior-posterior pelvis films were obtained after >12 months and acetabular indices (AIs) were measured and compared with contemporary normative data. Dysplasia was diagnosed as >2 SDs above the mean. Results: A total of 654 patients were referred with a history of a breech presentation, and 150 (22.9%) were found to have clinical instability or sonographic evidence of dysplasia on initial presentation and were observed with serial imaging or treated. Of the remaining 504 subjects with a normal clinical examination and screening ultrasound, 133 (26.4%; 74.4% females, 25.6% males) were followed until at least 12 months of age. Of those presenting at age 12 to 14 months, the mean AI was 0.42±0.83 SD above the mean with a skew towards elevated AIs. At the final follow-up (mean: 20.7±6.7 mo), the mean AI was 0.05±0.92 SD above the mean, and only 3/133 (2.2%) patients had a dysplastic hip. No patients underwent treatment other than an observation during the study period. Conclusions: One in 5 breech babies have dysplasia at presentation, but late dysplasia following normal screening ultrasound may be less common than previously reported and may be due to our prolonged follow-up period. We recommend surveillance of breech babies with follow-up visits after 12 months of age since earlier visits may offer limited benefits. Level of Evidence: Level II—prospective prognostic study.

Background: Madelung deformity arises from a partial distal radial growth disturbance in combination with an abnormal hypertrophic ligament spanning the volar radius and carpus, termed, the Vickers ligament. The purpose of this study is to report long-term clinical and radiographic outcomes following Vickers ligament release and distal radial physiolysis in a population of skeletally immature patients with symptomatic Madelung deformity. Methods: Medical records were retrospectively reviewed of patients with Madelung deformity surgically treated between 1994 and 2005. All eligible patients who underwent a Vickers ligament release and distal radial physiolysis were contacted and invited to return to the clinic for follow-up. Results: Six patients (8 wrists) with Madelung deformity underwent Vickers ligament release and distal radial physiolysis. All were white females with a mean age at initial presentation of 11.4 years (10 to 12.8 y). Mean age at the time of initial surgery was 12.0 years (10.0 to 14.5 y). The median follow-up time was 10.6 years (5.8 to 21.9 y) and the average age at last follow-up was 23.1 years (17.5 to 32.2 y). Pain alone or in combination with concerns for deformity was the chief complaint in 6 of 8 of the wrists. At 1 year of clinical follow-up, 7 of 8 wrists were reported to be pain-free, and 6 of the 8 were noted to be completely pain-free at last follow-up. Motion in flexion, extension, pronation, supination, radial, or ulnar deviation was similar between the preoperative status and long-term follow-up. The average preoperative ulnar tilt was 35.1 degrees (SD: 8.5 degrees), average preoperative lunate subsidence was 1.9 degrees (SD: 1.8 degrees), and average preoperative palmar carpal displacement was 21.9 degrees (SD: 2.9 degrees). At the final follow-up, there was a large progression in lunate subsidence, but minimal change in ulnar tilt and palmar carpal displacement. At last clinical follow-up, 2 of the 6 patients had undergone a subsequent procedure including 1 radial dome osteotomy and 1 ulnar shortening osteotomy. Conclusion: In the skeletally immature patient population with Madelung deformity with growth potential remaining, distal radial physiolysis and Vickers ligament release is associated with relief of pain, preservation of motion, and, a reasonable rate of reoperation. Type of Study: This was a therapeutic study. Level of Evidence: Level II.

While ultrasound examination of leg veins has been largely accepted, assessment of the pelvic and lower abdominal veins has been largely ignored. The problem here is that the pelvic region contains important veins, which require assessment for deep vein thrombosis and pelvic and vulval varices, but these are superimposed by abdominal viscera. The need to assess the veins of the lower abdomen and pelvic cavity prompted us to develop a reproduceable technique of ‘windowing’ into the abdomen and pelvis to view certain veins. To view a certain vein, a nominated angle of approach from a particular anatomical point with the patient in a specific position will under most circumstances provide a sonic ‘window’ to that vein. There are eight of these such windows that can, in competent hands, be used to assess a variety of pelvic and lower abdominal veins.

Level III-therapeutic.

No abstract