Ulnar Ray Deficiency: A New Classification and a Unique Case Report.

Ulnar ray deficiency is a rare congenital upper limb defect. We report on a unique case with hand-on-flank deformity on the one side and limb truncation on the contralateral side. The standard of care for the hand-on-flank deformity is to do humerus osteotomy to reposition the hand anteriorly. However, the right limb truncation in our patient made the senior author decide not to do the osteotomy. Final assessment showed that the posterior hand position enabled the patient to reach the ano-genital areas, the pocket, and the mouth. It was concluded that in case of hand-on-flank deformity in one limb and limb truncation of the contralateral limb, osteotomies to bring the hand anteriorly are not advised.

Mid-Term Results of Partial Trapeziectomy and Simple FCR Tendon Graft Interposition in a Selected Group of Patients with Thumb Carpometacarpal Joint Arthritis.

Introduction There are several surgical options for the treatment of osteoarthritis of the first carpometacarpal joint (CMCJ1). We introduce our technique of partial trapeziectomy and flexor carpi radialis (FCR) tendon graft interposition in a selected group of patients with CMCJ1. Objective The main purpose of this article is to investigate the mid-term results of our technique. Patients and Methods This is a retrospective study of 24 patients with CMCJ1 arthritis (23 females and 1 male with a mean age of 68 years) who were treated with partial trapeziectomy and simple FCR tendon graft interposition. Patient selection for this procedure was based on two prerequisites: the absence of scaphotrapezial arthritis and the absence of severe ligament laxity or severe subluxation of the CMCJ1. All patients had a mean follow-up of 6 years. Pre- and postoperative standard assessments were done. Results There were no postoperative complications and a zero-revision rate. All parameters significantly improved after surgery ( p < 0.05). All patients were "very satisfied" with the outcome. Conclusion The procedure of partial trapeziectomy and FCR tendon graft interposition can give an excellent mid-term outcome with a zero-revision rate if utilized in a selected group of patients with CMCJ1 arthritis.

Delta Like-1 Gene Mutation: A Novel Cause of Congenital Vertebral Malformation.

Skeletal development throughout the embryonic and postnatal phases is a dynamic process, based on bone remodeling and the balance between the activities of osteoclasts and osteoblasts modulating skeletal homeostasis. The Notch signaling pathway is a regulator of several developmental processes, and plays a crucial role in the development of the human skeleton by regulating the proliferation and differentiation of skeletal cells. The Delta Like-1 (DLL1) gene plays an important role in Notch signaling. We propose that an identified alteration in DLL1 protein may affect the downstream signaling. In this article, we present for the first time two siblings with a mutation in the DLL1 gene, presenting with congenital vertebral malformation. Using variable in silico prediction tools, it was predicted that the variant was responsible for the development of disease. Quantitative reverse-transcription polymerase chain reaction (PCR) for the Notch signaling pathway, using samples obtained from patients, showed a significant alteration in the expression of various related genes. Specifically, the expression of neurogenic locus notch homolog protein 1, SNW domain-containing protein 1, disintegrin, and metalloproteinase domain-containing proteins 10 and 17, was upregulated. In contrast, the expression of HEY1, HEY2, adenosine deaminase (ADA), and mastermind-like-1 (MAML-1) was downregulated. Furthermore, in a phosphokinase array, four kinases were significantly changed in patients, namely, p27, JANK1/2/3, mitogen- and stress-activated protein kinases 1 and 2, and focal adhesion kinase. Our results suggest an implication of a DLL1 defect related to the Notch signaling pathway, at least in part, in the morphologic abnormality observed in these patients. A limitation of our study was the low number of patients and samples. Further studies in this area are warranted to decipher the link between a DLL1 defect and skeletal abnormality.

National Approach to Standardize and Improve Mechanical Ventilation.

NASAM (National Approach to Standardize and Improve Mechanical Ventilation) is a national collaborative quality improvement project in Saudi Arabia. It aims to improve the care of mechanically ventilated patients by implementing evidence-based practices with the goal of reducing the rate of ventilator-associated events and therefore reducing mortality, mechanical ventilation duration and intensive care unit (ICU) length of stay. The project plans to extend the implementation to a total of 100 ICUs in collaboration with multiple health systems across the country. As of March 22, 2019, a total of 78 ICUs have registered from 6 different health sectors, 48 hospitals, and 27 cities. The leadership support in all health sectors for NASAM speaks of the commitment to improve the care of mechanically ventilated patients across the kingdom.

A modified technique of two-staged extensor tendon reconstruction in zones 6-8 in a patient with absent palmaris/plantaris tendons: A case report.

INTRODUCTION: The indications for two-staged extensor tendon reconstruction are rare and only 14 previously reported cases were found in the literature. In these cases, silicone rods are inserted in the first stage. Few months later, the palmaris longus / plantaris tendon grafts are usually used to replace the silicone rods. CASE REPORT: we encountered a patient with major defects of the extensor tendons of all fingers extending from the proximal one third of zone 6 to zone 8. The patient had no palmaris or plantaris tendons. We utilized a modified technique of reconstruction using the split flexor carpi radialis as the tendon graft and the flexor carpi ulnaris as the motor tendon. At final follow-up, there was full active extension of the fingers. However, there was limitation of wrist flexion because of the harvesting of both wrist flexors. DISCUSSION: We describe a modified technique of two-staged extensor tendon reconstruction which may be used in patients with absent palmaris/ plantaris tendons. CONCLUSION: In patients with absent palmaris/ plantaris tendons and major defects of the extensor tendons of all fingers, the use of split flexor carpi radialis is an adequate alternative for reconstruction and gives a good functional outcome.