Developmental Dysplasia of the Hip (DDH): Etiology, Diagnosis, and Management.

Developmental dysplasia of the hip (DDH) is a complex disorder that refers to different hip problems, ranging from neonatal instability to acetabular or femoral dysplasia, hip subluxation, and hip dislocation. It may result in structural modifications, which may lead to early coxarthrosis. Despite identifying the risk factors, the exact aetiology and pathophysiology are still unclear. Neonatal screening, along with physical examination and ultrasound, is critical for the early diagnosis of DDH to prevent the occurrence of early coxarthrosis. This review summarizes the currently practised strategies for the detection and treatment of DDH, focusing particularly on current practices for managing residual acetabular dysplasia (AD). AD may persist even after a successful hip reduction. Pelvic osteotomy is required in cases of persistent AD. It could also be undertaken simultaneously with an open hip reduction. Evaluation of the residual dysplasia (RD) of the hip and its management is still a highly active area of discussion. Recent research has opened the door to discussion on this issue and suggested treatment options for AD. But there is still room for more research to assist in managing AD.

Acetabular remodeling after graft extrusion, rotation or impaction in Dega and Pemberton acetabuloplasties for developmental dysplasia of the hip.

Traditionally graft displacement following Pemberton and Dega acetabuloplasties involves internal fixation for treatment of developmental dysplasia of hip (DDH). This study was performed to assess the acetabular remodeling by conservative management of graft displacement among patients with DDH. This was a retrospective study of 20 patients 17 (85%) women and 3 (15%) men; mean age 22.90 ± 6.96 months with DDH who underwent Pemberton and Dega acetabuloplasties at King Khalid University Hospital, Riyadh between January 2013 and January 2018. All patients after losing acetabular correction during immediate postoperative period were treated by conservative management. The management involved application of Spica cast for 6 weeks that was trimmed to broomstick cast for an extended period of time and finally replaced by nocturnal abduction brace until normal acetabular index (AI) was achieved. The patients were followed up for a mean period of 44.60 ± 12.36 months. Out of the total, 18 (90%) patients with DDH were successfully treated by conservative management. The mean preoperative AI of 43.70° ± 5.91° improved to 21.35o ± 6.32o at the final follow-up (P < 0.001) which was no different when compared to the mean of unaffected hips (19.70o ± 2.96o; P < 0.44). The mean preoperative CEA of all the patients was negative that improved during the conservative treatment to 29.20° ± 10.0° which was no different when compared with the mean of unaffected (31.70° ± 4.64o; P = 0.32) hips at the final follow-up. doption of less aggressive approach for management of displaced, rotated or impacted autograft following acetabuloplasties among children with DDH was not only a useful conservative approach for remodeling of hips but also obviated the need for additional surgical intervention.

A Novel Modification of Woodward Procedure for Correction of Sprengel Deformity by Application of Anchoring Sutures.

PURPOSE: To report the functional and cosmetic results of cases with Sprengel deformity (SD) who underwent a novel technical modification in Woodward procedure for severe SD comprising of lowering of the scapula followed by stabilization of scapula with application of anchoring sutures. MATERIALS AND METHODS: Twenty-three children were operated at a mean age of 6.6 ± 2.5 years (range 3.4-11 years) and followed up for a mean period of 5.51 ± 1.98 years. RESULTS: The mean Cavendish score of the patients before procedure of 3.2 ± 0.45 was reduced to 1.7 ± 0.56 (p < 0.001) after the surgery. All patients improved in global shoulder abduction. The mean preoperative abduction of 97° ± 15.2° increased to a mean of 149° ± 14.1° post-operatively (p < 0.001). Significant improvement was also observed in mean Rigault grades after surgical intervention using anchoring suture modification of Woodward procedure. The mean Rigault grade of 2.7 ± 0.47 prior to surgery was reduced to 1.3 ± 0.47 (p < 0.001) at final evaluation. Age of the patients negatively correlated with the functional outcome (r = - 0.48; p < 0.02). The presence of an omovertebral bar or associated congenital anomalies did not affect the functional outcome. CONCLUSION: The modification of Woodward procedure for correction of SD by application of anchoring sutures was a reliable method for correction of SD particularly at younger age. The procedure was associated with significant improvement in shoulder abduction, cosmetic appearance and radiological grades over 5 year follow-up.

Hereditary 1,25-dihydroxyvitamin D-resistant rickets (HVDRR): clinical heterogeneity and long-term efficacious management of eight patients from four unrelated Arab families with a loss of function VDR mutation.

BACKGROUND: Vitamin D regulates the concentrations of calcium and phosphate in blood and promotes the growth and remodeling of bones. The circulating active form of vitamin D, 1,25-dihydroxyvitamin D, binds to the vitamin D receptor (VDR), which heterodimerizes with the retinoid X receptor to regulate the expression of target genes. Inactivating mutations in the VDR gene cause hereditary vitamin D-resistant rickets (HVDRR), a rare disorder characterized by an early onset of rickets, growth retardation, skeletal deformities, hypocalcemia, hypophosphatemia and secondary hyperparathyroidism, and in some cases alopecia. METHODS: We describe eight new HVDRR patients from four unrelated consanguineous families. The VDR gene was sequenced to identify mutations. The management of patients over a period of up to 11 years following the initial diagnosis is assessed. RESULTS: Although all patients exhibit main features of HVDRR and carry the same c.885C>A (p.Y295*) loss of function mutation in the VDR gene, there was heterogeneity of the manifestations of HVDRR-associated phenotypes and developmental milestones. These eight patients were successfully treated over a period of 11 years. All clinical symptoms were improved except alopecia. CONCLUSIONS: The study concludes that VDR sequencing and laboratory tests are essential to confirm HVDRR and to assess the effectiveness of the treatment.