Guided Growth in Leg Length Discrepancy in Beckwith-Wiedemann Syndrome: A Consecutive Case Series

Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder characterized by overgrowth, macroglossia, abdominal wall defects, neonatal hypoglycemia, predisposition to embryonal tumor, lateralized overgrowth, and leg length discrepancy (LLD), which can affect normal posture and gait. Aim of this study was to evaluate the effects of guided growth (temporary epiphysiodesis technique) as LLD management in BWS patients. Between 2007 and 2021, 22 BWS patients (15 F, 7 M) with a mean age of 7.9 years (2.9–14.4) and a mean LLD at first surgery of 3.65 cm (2–10), underwent temporary proximal tibial (PTE) and distal femur epiphysiodesis (DFE). In 18 patients the first surgical procedure was PTE, in one, DFE, and in 3 cases, PTE and DFE at the same time, respectively. Eleven patients reached equality of leg length after a mean follow-up of 7.7 years (3.7–13.0) and mean age of 13.3 years (12.7–27.5); 10 patients underwent 3 surgical procedures, one 7 procedures. Fifteen patients had no complications. No severe complications, infection, articular stiffness, or neuro-vascular lesions occurred in remaining patients; complications included secondary varus or valgus axial deviation in a total of 6 patients, and two screw breakages in two patients. Guided growth as a minimally invasive procedure seems efficient for LLD treatment with low complication rate in BWS patients.

Identification and Analysis of Genes Involved in Double Fertilization in Rice

Double fertilization is a key determinant of grain yield, and the failure of fertilization during hybridization is one important reason for reproductive isolation. Therefore, fertilization has a very important role in the production of high-yield and well-quality hybrid of rice. Here, we used RNA sequencing technology to study the change of the transcriptome during double fertilization with the help of the mutant fertilization barrier (feb) that failed to finish fertilization process and led to seed abortion. The results showed that 1669 genes were related to the guided growth of pollen tubes, 332 genes were involved in the recognition and fusion of the male–female gametes, and 430 genes were associated with zygote formation and early free endosperm nuclear division. Among them, the genes related to carbohydrate metabolism; signal transduction pathways were enriched in the guided growth of pollen tubes, the genes involved in the photosynthesis; fatty acid synthesis pathways were activated by the recognition and fusion of the male–female gametes; and the cell cycle-related genes might play an essential role in zygote formation and early endosperm nuclear division. Furthermore, among the 1669 pollen tube-related genes, it was found that 7 arabinogalactan proteins (AGPs), 1 cysteine-rich peptide (CRP), and 15 receptor-like kinases (RLKs) were specifically expressed in anther, while 2 AGPs, 7 CRPs, and 5 RLKs in pistil, showing obvious unequal distribution which implied they might play different roles in anther and pistil during fertilization. These studies laid a solid foundation for revealing double fertilization mechanism of rice and for the follow-up investigation.

MANAGEMENT OF FIRST-TIME PATELLAR DISLOCATION: A SURVEY OF PRISM MEMBERS (201)

Objectives: There are no current guidelines for optimal management of first-time patellar dislocation in children and adolescents. The objective of this study was to develop consensus-based guidelines for management of first-time dislocation in adolescents. Methods: A 29-question, case-based, multiple-choice survey was developed after 2-rounds of iterations by 20 members of the Patellofemoral RIG. The survey contained two case scenarios of first-time patellar dislocation in adolescents - one with and one without an osteochondral fracture. The survey was administered to primary sports medicine and orthopedic surgeons of the PRISM Society in April 2020 and the responses were analyzed. Consensus-based guidelines were generated when at least 66% of the respondents agreed. Results: 81/276 (29%) members responded to the survey and 6 consensus-based guidelines were generated from those responses (Table 1).There was no consensus between conservative (60%) and operative (40%) treatment of contralateral patellar dislocation. Free-text analysis revealed several variables that influence the decision-making process. Conclusions: There are changes in trends related to management of first-time dislocation in adolescents. MRI, though not recommended for all patients with first-time dislocation, was obtained by 51% of respondents. The survey provides details of conservative treatment, including the role and duration of bracing and physical therapy. For first-time dislocation with an osteochondral fracture, concomitant patellar stabilization is preferred over isolated fixation. Simultaneous guided-growth for genu valgum correction is recommended. There were several variables that influenced the decision-making process and there were several areas of discordance. Further research studies on these parameters could potentially improve outcomes. *PF RIG members: Matthew Ellington, MD, Craig J Finlayson, MD, Joseph Molony, Jr., PT, MS, SCS, CSCS, Nicole Friel, MD MS, Daniel W Green, MD, Jeffery L Mikutis, DO, Todd A Milbrandt, MD, James L Pace, MD, Albert M Pendleton, MD, Lauren H Redler, MD, Jason Rhodes, MD, Shannon D Safier, MD, John A Schlechter, MD, Beth E Shubin Stein, MD, Curtis Vandenberg, MD, Jeffrey M Vaughn, DO, Eric J Wall, MD, Drew E Warnick, MD, Moshe Yaniv, MD, Shital N Parikh, MD.

MANAGEMENT OF FIRST-TIME PATELLAR DISLOCATION: A SURVEY OF PRISM MEMBERS

Background: There are no current guidelines for optimal management of first-time patellar dislocation in children and adolescents. Purpose: To develop consensus-based guidelines for management of first-time dislocation in adolescents. Methods: A 29-question, case-based, multiple-choice survey was developed after 2-rounds of iterations by 20 members of the Patellofemoral RIG. The survey contained two case scenarios of first-time patellar dislocation in adolescents - one with and one without an osteochondral fracture. The survey was administered to primary sports medicine and orthopedic surgeons of the PRISM Society in April 2020 and the responses were analyzed. Consensus-based guidelines were generated when at least 66% of the respondents agreed Results: 81/276 (29%) members responded to the survey and 6 consensus-based guidelines were generated from those responses. [Table: see text] There was no consensus between conservative (60%) and operative (40%) treatment of contralateral patellar dislocation. Free-text analysis revealed several variables that influence the decision-making process. Conclusion: There are changes in trends related to management of first-time dislocation in adolescents. MRI, though not recommended for all patients with first-time dislocation, was obtained by 51% of respondents. The survey provides details of conservative treatment, including the role and duration of bracing and physical therapy. For first-time dislocation with an osteochondral fracture, concomitant patellar stabilization is preferred over isolated fixation. Simultaneous guided-growth for genu valgum correction is recommended. There were several variables that influenced the decision-making process and there were several areas of discordance. Further research studies on these parameters could potentially improve outcomes.