Severe Developmental Dysplasia Of The Hip Research Articles

Accuracy of portable navigation during THA in patients with severe developmental dysplasia of hip.

Correct cup placement in total hip arthroplasty (THA) for patients with developmental dysplasia of the hip (DDH) is considerably difficult. This study aimed to analyze the orientation accuracy of cup insertion during THA using a portable navigation system in patients with DDH. In this retrospective cohort study, we analyzed data from 64 patients who underwent THA using infrared stereo camera-matching portable navigation. Patients underwent THA via the anterolateral approach in the lateral decubitus position. Navigation records for intraoperative cup angles, postoperative cup angles measured on computed tomography (CT) images, and cup angle measurement differences were measured and compared between patients with non-DDH/mild DDH and severe DDH. Furthermore, the predictive factors for outliers of accurate acetabular cup placement were analyzed. The average measurement absolute abduction differences (postoperative CT-navigation record) were 3.9 ± 3.5° (severe DDH) and 3.3 ± 2.6° (non-DDH/ mild DDH), and the anteversion differences were 4.7 ± 3.4° (severe DDH) and 2.3 ± 2.1° (non-DDH/ mild DDH). The anteversion difference was different between the two groups. Multivariate analysis showed that the navigation difference (absolute difference in anteversion between postoperative CT and navigation records of > 5°) was significantly associated with severe DDH (odds ratio [OR]: 3.3; p = 0.049, 95% confidence interval [CI]: 1.0-11.1) and posterior pelvic tilt (OR: 1.1; p = 0.042, 95% CI: 1.0-1.27). In patients with severe DDH, it is important to pay close attention during THA using portable navigation. However, the average difference was < 5º even in patients with severe DDH, and the accuracy may be acceptable in a clinical setting when the cost is considered.

Computer simulation study of cementless cup placement for dysplastic hip osteoarthritis using subluxation percentage of Crowe classification.

It is often difficult to achieve adequate bone coverage of the cup in total hip arthroplasty in cases of severe developmental dysplasia of the hip (DDH). This study aimed to evaluate the relationship between subluxation percentage of Crowe classification and cup center-edge (cup-CE) angle to investigate whether subluxation percentage according to the Crowe classification is a useful indicator for cementless cup placement. Cementless cup placement was simulated in 77 women with DDH in 91 hips (Crowe I, 35 hips; Crowe II, 35 hips; and Crowe III, 21 hips) using computed tomography-based computer simulation software. The cups were placed at the anatomic hip center (AHC) and 10-mm high hip center (HHC). The relationship between the subluxation percentage and cup-CE angle was evaluated using Spearman's rank correlation coefficient. In addition, the cutoff values for the subluxation percentage that satisfied a cup-CE angle ≥0° were determined using the receiving operating characteristic curve. The cup-CE angle was negatively correlated with the subluxation percentage in both AHC and 10-mm HHC (correlation coefficient ρ = -0.542 [p < 0.01] and -0.704 [p < 0.01], respectively). The cutoff values for subluxation percentage that satisfied a cup-CE angle ≥0° were 56.1% and 73.6% for AHC and 10-mm HHC, respectively. Cementless cup placement in AHC is difficult in cases with the subluxation percentage ≥56.1%, and HHC reconstruction or femoral structural autograft technique should be considered as an alternative. Moreover, placement at 10 mm above AHC is difficult in cases with subluxation percentage ≥73.6%.

The Outcome of Total Hip Arthroplasty Without Subtrochanteric Shortening Osteotomy in Severe Developmental Dysplasia of the Hip

Background: Total hip arthroplasty (THA) is a surgical technique to correct developmental dysplasia of the hip (DDH). Various THA-based methods have been developed for managing end-stage osteoarthritis in patients with DDH Crowe type III or IV. Objectives: In this study, we evaluate the Harris hip score (HHS) before and after cementless THA without subtrochanteric shortening osteotomy (STO) in patients with DDH Crowe type III or IV who were candidates for THA, as well as complications and the need for revision surgery. Methods: This retrospective cohort study was conducted on patients with DDH Crowe type III and IV, who were candidates for cementless THA using the Watson Jones technique without shortening STO. The HHS was calculated and recorded in their medical files before surgery. Patients with missing or incomplete medical files, without written informed consent, with neurovascular diseases, immunosuppressive drug use, congenital bone and articular anomalies, low back pain radiating to the lower extremities, simultaneous fractures, a history of lower extremity fractures, or joint infections were excluded. The HHS was recalculated during outpatient follow-up. Neurovascular defects and the need for reoperation were also evaluated. All data were recorded and analyzed. Results: Thirty-two patients were enrolled with a mean ± SD age of 50.34 ± 15.45 years, most of whom were women (75%). The patients were followed for 2 to 7 years. Postoperative nerve defects were observed in only 1 (3.1%) patient. Hip reoperation was performed in 1 (3.1%) patient. The mean ± SD of HHS before surgery was 50.10 ± 12.48, which increased significantly to 77.99 ± 15.60 after surgery (P &lt; 0.001). Furthermore, the HHS was evaluated before and after the intervention for each gender, showing a significant increase in both. Conclusions: Cementless THA without shortening osteotomy can improve HHS in patients with DDH Crowe III and IV, providing satisfactory outcomes. Furthermore, complications, such as neurological defects and the need for reoperation, are minimal with this method.

Incidence of Nerve Palsies During Pavlik Harness Treatment for Developmental Dysplasia of the Hip: A Retrospective Cohort Study.

The Pavlik harness has been used for approximately a century to treat developmental dysplasia of the hip (DDH). Femoral nerve palsy is a documented complication of Pavlik harness use, with an incidence ranging from 2.5% to 11.2%. Rare reports of brachial plexus palsy have also been documented. The primary purpose of the current study was to evaluate the incidence of various nerve palsies in patients undergoing Pavlik harness treatment for DDH. Secondary aims were to identify patient demographic or hip characteristics associated with nerve palsy. We performed a retrospective review of patients diagnosed with DDH and treated with a Pavlik harness from February 1, 2016, to April 1, 2023, at a single tertiary care orthopaedic hospital. Hip laterality, use of a subsequent rigid abduction orthosis, birth order, breech positioning, weight, and family history were collected. The median (and interquartile range [IQR]) or mean (and standard deviation [SD]) were reported for all continuous variables. Independent 2-sample t tests and Mann-Whitney U tests were conducted to identify associations between the variables collected at the initiation of Pavlik harness treatment and the occurrence of nerve palsy. Three hundred and fifty-one patients (547 hips) were included. Twenty-two cases of femoral nerve palsy (4% of all treated hips), 1 case of inferior gluteal nerve palsy (0.18%), and 2 cases of brachial plexus palsy (0.37%) were diagnosed. Patients with nerve palsy had more severe DDH as measured by the Graf classification (p < 0.001) and more severe DDH as measured on physical examination via the Barlow and Ortolani maneuvers (p = 0.003). Nerve palsies were associated with more severe DDH at the initiation of Pavlik harness use. Upper and lower-extremity neurological status should be scrutinized at initiation and throughout treatment to assess for nerve palsies. The potential for femoral, gluteal, and brachial plexus palsies should be included in the discussion of risks at the beginning of treatment. Families may be reassured that nerve palsies associated with Pavlik harness can be expected to resolve with a short break from treatment. Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Risk factors for postoperative avascular necrosis of the femoral head in children with developmental dysplasia of the hip.

To investigate factors associated with postoperative avascular necrosis of the femoral head (ANFH) in developmental dysplasia of the hip (DDH) patients, and if or how the associations varied among different subpopulations of age, sex and surgical method. Patients with DDH were enrolled between October 31, 2016 and July 15, 2020 in this retrospective cohort study. The average follow-up time was 21.42 ± 10.02 months. The outcome was postoperative ANFH. The main study variables were the DDH classification, Tonnis grade, International Hip Dysplasia Institute (IHDI) classification, and preoperative traction. Multivariate logistic regression was employed to assess the associations between main study variables and postoperative ANFH. Subgroup analysis was carried out based on age at reduction, sex and surgical method. Odds ratio (ORs) and 95% confidence intervals (CIs) were calculated. A total of 427 children with DDH were included, with 92 (21.55%) in the ANFH group, and 335 (78.45%) in the non-ANFH group. DDH classification was positively correlated with the risk of postoperative ANFH (OR = 4.14, 95% CI, 1.08-15.77, P = 0.038). Children with preoperative traction had a significantly decreased risk of postoperative ANFH in contrast to those without preoperative traction (OR = 0.37, 95% CI, 0.22-0.61, P < 0.001). Children aged 1-3 years who received preoperative traction has a significantly reduced risk of postoperative ANFH than those who did not receive preoperative traction (OR = 0.28, 95% CI, 0.15-0.51, P < 0.001). For children aged >3 years, positive association was found between DDH classification and the risk of postoperative ANFH (OR = 3.75, 95% CI, 1.51-9.31, P = 0.004). Girls with a more severe DDH type had a significantly higher risk of postoperative ANFH (OR = 3.80, 95% CI, 1.80-8.02, P < 0.001). Receiving preoperative traction was associated with a significantly decreased risk of postoperative ANFH in girls (OR = 0.37, 95% CI, 0.22-0.61, P < 0.001). For children undergoing open reduction, DDH classification was positively associated with the risk of postoperative ANFH (OR = 3.01, 95% CI, 1.65-5.50, P < 0.001), and those with preoperative traction had a lower risk of postoperative ANFH compared with those without preoperative traction (OR = 0.35, 95% CI, 0.20-0.61, P < 0.001). DDH classification and preoperative traction were associated with the risk of postoperative ANFH, and these associations varied across DDH patients with different ages, sexes and surgical methods.

Developmental retardation of femoral head size and femoral head ossification in mild and severe developmental dysplasia of the hip in infants: a preliminary cross-sectional study based on ultrasound images

For infants up to 6 months, ultrasound (US) screening of developmental dysplasia of the hip (DDH) is recommended. This cross-sectional study investigated the developmental data of femoral head size and femoral head ossification in mature infant hips and the impact of mild and severe DDH on femoral head development based on US images. We reviewed all hip US studies performed from January 2018 to December 2019 to evaluate DDH in infants younger than 6 months at West China Hospital, Sichuan University. The femoral head diameter (FHD) and femoral head ossification center type of each hip were recorded. A total of 1,037 normal participants with 2,074 mature hips and 367 DDH participants with 456 dysplastic hips were included in this study. For normal mature hips (Graf I), the FHD of mature male hips was significantly larger than that of female hips from the age of 2 months to 6 months (all P values <0.01), and the femoral head ossification center of males occurred significantly later than that of females at the same age from 3 months to 6 months (all P values <0.05). Compared with the matched mature hips, the FHDs of Graf IIa (-), IIb, IIc, and D, III or IV hips were significantly smaller (1.64 vs. 1.72 cm, 1.75 vs. 1.79 cm, 1.65 vs. 1.73 cm, 1.51 vs. 1.71 cm, respectively; all P values <0.05), and the occurrence of the femoral head ossification center was delayed in Graf IIa (-) and D, III or IV hips (both P values <0.05). However, no significant developmental retardation of the femoral head was observed in Graf IIa (+) hips. We identified a relatively normal range for the development of infants' hips from 1 month old to 6 months old and found significant developmental retardation of the femoral head in Graf IIa (-), IIb, IIc, and D, III or IV hips. This is a preliminary study of the developmental impact of DDH on the femoral head, and we will continue the follow-up study after treatment.

Direct anterior versus posteriorlateral approachs for clinical outcomes after total hip arthroplasty in the treatment of severe DDH

BackgroundThe total hip arthroplasty (THA) has gained popularity in in the treatment of severe developmental dysplasia of the hip (DDH). the posterior lateral approach (PLA) has good clinical efficacy and has been confirmed by the majority clinicians. Nevertheless, controversy exists regarding longer-term benefits of the direct anterior approach (DAA). The objective of this study was to investigate the clinical efficacy and placement of S-ROM prosthesis in the treatment of severe DDH by The total hip arthroplasty (THA) with different surgical approaches.MethodsA retrospective analysis was performed on 42 patients with severe DDH admitted to our hospital from August 2015 to February 2022, who were treated with S-ROM prosthesis for total hip arthroplasty and subtrochanteric osteotomy of the femur. They were divided into DAA group and PLA group according to different surgical approaches. Perioperative indicators and imaging data were collected.ResultsThe surgery time, intraoperative blood loss, and creatine kinase difference in DAA group and PLA group was without a statistically significant difference (P > 0.05). The postoperative length of hospitalization was shorter in the DAA group than in the PLA group (6.50 ± 3.15 vs 9.18 ± 4.93, P = 0.045). The acetabular abduction angles、the acetabular anteversion angles, the safe area ratio, The difference of femoral eccentricity, and the vertical difference of rotation center in DAA group and PLA group, there was no statistical significance (P > 0.05). Statistically significant differences were detected the horizontal difference of rotation center (P = 0.044).ConclusionsTotal hip arthroplasty with S-ROM prosthesis is a feasible procedure for severe dysplastic DDH. The clinical efficacy and prosthesis placement parameters of DAA approach are advantage to those of PLA approach.

Behandlung der Hüftgelenkdysplasie beim jungen Erwachsenen

Developmental dysplasia of the hip (DDH) is one of the most common disorders of hips in children. The deformity can remain asymptomatic into adolescence and adulthood; however, it is considered to be a form of prearthritis and is the main cause of premature osteoarthritis of the hip. The deformity affects the acetabulum but can also be accompanied by changes in the shape of the proximal femur. If conservative treatment for mild DDH is insufficient, or in cases of moderate to severe DDH, operative treatment should be carried out, for example by corrective osteotomy of the pelvis and/or the proximal femur and hip arthroscopy may be considered adjunctively in order to resolve the prearthritis and prevent premature osteoarthritis of the hip. This manuscript elucidates the deformity, the diagnostic measures required to make the diagnosis and the treatment options available for prevention of arthritis.

Splint Duration and Not the Mode of Anesthesia Is the Main Factor Influencing Avascular Necrosis After Closed Reduction for Developmental Dysplasia of the Hip in Kosovo.

The aim of this study was to determine whether the use of analgesia and sedation (AS) as opposed to general anesthesia (GA) for closed reduction and spica casting of children with severe developmental dysplasia of the hip (DDH) influenced the long-term incidence of avascular necrosis (AVN). In a prospective, randomized, single-blinded clinical trial we investigated 100 pediatric patients with DDH type IIIa, IIIb, and IV (according to Graf classification), who were randomly assigned into the group receiving AS, and the group receiving GA. Baseline demographics, splint duration, and type of DDH were carefully assessed. The presence of AVN was assessed at the follow-up visits at 1 and 7 years after the end of treatment. The AS-group consisted of 50 patients (46 girls) with 76 hips affected (n = 11/Type-IIIa, n = 32/Type-IIIb, and n = 33/Type-IV). The GA-group consisted also of 50 patients (44 girls) with 78 hips involved (n = 15/Type-IIIa, n = 34/Type-IIIb, and n = 29/Type-IV). At 7-years follow-up, AVN was diagnosed in 9 of 154 hips (5.8%), 5 hips in the AS-group and 4 hips in the GA group. The logistic regression model showed no significant difference in AVN incidence between the AS and GA groups at 7-years follow-up (p = 0.27). The multivariate regression analysis showed that neither the type of DDH nor the age at diagnosis influenced the incidence of AVN (p = 0.48 and p = 0.28, respectively). Splint duration was identified as the only significant factor for the long-term incidence of AVN in the treatment of severe DDH. For every month of longer splint duration, the odds of AVN at 7-years follow-up increased by a factor of 3.81 (95%CI: 1.35–13.73, p = 0.02). Closed reduction and spica casting of children with severe DDH under AS can be considered a feasible alternative to management under GA. All efforts must be made to diagnose patients with DDH as early as possible and shorten the duration of splint treatment to prevent the development of AVN. Level of Evidence. Level II-1.

Robotic-arm assisted THA can achieve precise cup positioning in developmental dysplasia of the hip : a case control study.

AimsThis study aimed to evaluate the accuracy of implant placement with robotic-arm assisted total hip arthroplasty (THA) in patients with developmental dysplasia of the hip (DDH).MethodsThe study analyzed a consecutive series of 69 patients who underwent robotic-arm assisted THA between September 2018 and December 2019. Of these, 30 patients had DDH and were classified according to the Crowe type. Acetabular component alignment and 3D positions were measured using pre- and postoperative CT data. The absolute differences of cup alignment and 3D position were compared between DDH and non-DDH patients. Moreover, these differences were analyzed in relation to the severity of DDH. The discrepancy of leg length and combined offset compared with contralateral hip were measured.ResultsThe mean values of absolute differences (postoperative CT-preoperative plan) were 1.7° (standard deviation (SD) 2.0) (inclination) and 2.5° (SD 2.1°) (anteversion) in DDH patients, and no significant differences were found between non-DDH and DDH patients. The mean absolute differences for 3D cup position were 1.1 mm (SD 1.0) (coronal plane) and 1.2 mm (SD 2.1) (axial plane) in DDH patients, and no significant differences were found between two groups. No significant difference was found either in cup alignment between postoperative CT and navigation record after cup screws or in the severity of DDH. Excellent restoration of leg length and combined offset were achieved in both groups.ConclusionWe demonstrated that robotic-assisted THA may achieve precise cup positioning in DDH patients, and may be useful in those with severe DDH.Cite this article: Bone Joint Res 2021;10(10):629–638.

The Three-Dimensional Morphology of Femoral Medullary Cavity in the Developmental Dysplasia of the Hip.

ObjectiveThis study aimed to assess the morphology of the femoral medullary canal in subjects with developmental dysplasia of the hip (DDH) with the intent of improving the design of femoral stems in total hip arthroplasty.MethodsComputed tomography images of 56 DDH hips, which were classified into Crowe I to Crowe IV, and 30 normal hips were collected and used to reconstruct three-dimensional morphology of the femoral medullary cavity. Images of twenty-one cross sections were taken from 20 mm above the apex of the lesser trochanter to the isthmus. The morphology of femoral cavity was evaluated on each cross section for the longest canal diameter, the femoral medullary torsion angle (FMTA), and the femoral medullary roundness index (FMRI).ResultsThe Crowe IV group displayed the narrowest medullary canal in the region superior to the end of the lesser trochanter, but then gradually aligned with the medullary diameter of the other groups down to the isthmus. The FMTA along the femoral cavity increased with the severity of DDH, but the rate of variation of FMTA along the femoral canal was consistent in the DDH groups. The DDH hips generally showed a larger FMRI than the normal hips, indicating more elliptical shapes.ConclusionA femoral stem with a cone shape in the proximal femur and a cylindrical shape for the remainder down to the isthmus may benefit the subjects with severe DDH. This design could protect bone, recover excessive femoral anteversion and facilitate the implantation in the narrow medullary canal.

Tübingen hip flexion splint more successful than Pavlik harness for decentred hips after the age of three months.

The objective of this study was to evaluate the clinical and radiological outcomes of patients younger than six months of age with developmental dysplasia of the hip (DDH) managed by either a Pavlik harness or Tübingen hip flexion splint. Records of 251 consecutive infants with a mean age of 89 days (SD 47), diagnosed with DDH between January 2015 and December 2018, were retrospectively reviewed. Inclusion criteria for patients with DDH were: younger than 180 days at the time of diagnosis; ultrasound Graf classification of IIc or greater; treatment by Pavlik harness or Tübingen splint; and no prior treatment history. All patients underwent hip ultrasound every seven days during the first three weeks of treatment and subsequently every three to four weeks until completion of treatment. If no signs of improvement were found after three weeks, the Pavlik harness or Tübingen splint was discontinued. Statistical analysis was performed. The study included 251 patients with Graf grades IIc to IV in 18 males and 233 females with DDH. Mean follow-up time was 22 months (SD 10). A total of 116 hips were graded as Graf IIc (39.1%), nine as grade D (3.0%), 100 as grade III (33.7%), and 72 as grade IV (24.2%). There were 109 patients (128 hips) in the Pavlik group and 142 patients (169 hips) in the Tübingen group (p = 0.227). The Tübingen group showed a 69.8% success rate in Graf III and Graf IV hips while the success rate was significantly lower in the Pavlik group, 53.9% (p = 0.033). For infants older than three months of age, the Tübingen group showed a 71.4% success rate, and the Pavlik group a 54.4% success rate (p = 0.047). The Tübingen splint should be the preferred treatment option for children older than three months, and for those with severe forms of DDH such as Graf grade III and IV, who are younger than six months at time of diagnosis. The Tübingen hip flexion splint is a valid alternative to the Pavlik harness for older infants and those with more severe DDH. Cite this article: Bone Joint J2021;103-B(5):991-998.

Clinical evaluation of direct anterior approach total hip arthroplasty for severe developmental dysplasia of the hip

It is challenging to treat developmental dysplasia of the hip (DDH) classified Crowe III-IV using direct anterior approach (DAA) total hip arthroplasty (THA), and very little is known on its outcome. This study aimed to investigate the clinical result in this defined disorder with DAA versus posterolateral approach. Twenty-three consecutive hips with Crowe III-IV DDH who underwent DAA were retrospectively evaluated from 2016 through 2018. Outcomes were primarily assessed by HHS, WOMAC, and SF-12 physical scales. The second evaluations included leg length discrepancy, hip muscle strength, radiographic review, complications, and limp recovery. Results were compared to a control cohort of 50 hips underwent posterolateral THA concurrently within the observational period. At last follow-up (DAA 28.5 months; PLA 39.0 months), the mean increase of the HHS for DAA was 48.2 and 30.3 for PLA (p = 0.003). The improvement in WOMAC score in DAA cohort was 15.89 higher that of the PLA cohort after adjusting preoperative difference [R2 = 0.532, P = 0.000, 95% CI (10.037, 21.735)]. DAA had more rapid recovery of hip abductor strength at 1-month (p = 0.03) and hip flexor strength at 3 months (p = 0.007) compared to PLA. No significant differences were found in the radiographic analysis with the exception of increased acetabular anteversion in the DAA cohort (p = 0.036). Satisfactory improvement in limp, indicated by the percentage of limp graded as none and mild to the total, was much higher in DAA cohort (97.6%), compared to that of PLA cohort (90.0%, p = 0.032). DAA for high-dislocated dysplasia demonstrate a significant improvement in clinical result comparable to posterolateral approach. Improved clinical outcome in terms of increased HHS and WOMAC scores, rapid recovery of hip abductor and flexor strength, and enhanced limp recovery without an increased risk in complications, could be acquired when the surgeons were specialized in this approach.

CLINICAL COURSE OF SCHIMKE IMMUNO-OSSEOUS DYSPLASIA AFTER KIDNEY TRANSPLANTATION

Schimke immuno-osseous dysplasia (SIOD) is one of the rare causes of steroid-resistant nephrotic syndrome. Extra-renal symptoms of this autosomal recessive disease include spondyloepiphyseal dysplasia, disproportional growth failure and defective cellular immunity. Kidney transplantation (KT) is indicated in these patients when their kidney problems progress to end-stage renal disease (ESRD). Due to the rarity of this condition, clinical course of SIOD after KT is not well known yet. Here we present our experience of SIOD after KT. Since 2014 three children with focal segmental glomerulosclerosis (FSGS) confirmed by biopsy were diagnosed as SIOD through genetic analysis. Onset age of proteinuria was 7, 5, 3 years, respectively. All of them had disproportional growth failure and typical facial feature. They reached ESRD in about two years after diagnosis of proteinuria. At their age of 9, 7, 9 years, these patients had living donor KT from their parents. After KT they suffered from multiple infections. Case 1 had PCP pneumonia requiring ventilator care 2 months after KT, then in 3 months disseminated adenovirus infection occurred along with CMV, EBV, and JC virus infection. The patient succumbed to the infection in 2 months despite aggressive management. Case 2 had CMV infection and PCP pneumonia after 3 months of KT. She suffered from skin infection and otitis. Case 3 suffered from respiratory failure due to CMV infection after 5 months of KT. Recurrent hospitalization was required due to various infections. Allograft has survived for 3.5 years in case 3 despite low-dose tacrolimus single medication (< 5ng/mL), but case 1 lost his graft function along with disseminated adenovirus infection. Case 2 had graft failure after 5 years of KT from acute rejection, probably due to insufficient dosage of tacrolimus and poor compliance. As Extra-renal presentation case 1 had renal vein thrombosis extended to IVC and pulmonary hypertension before KT, lowering his oxygen saturation to 80-90% in room air. Case 2 had severe developmental dysplasia of the hip requiring orthopedic surgery at the age of 5. She had sitting height and leg length ratio of 0.96, vertebral flattening and lordosis on spine x-ray suggesting spondyloepiphyseal dysplasia. After KT she suffered constant headache and Moyamoya syndrome was dignosed. She had an encephaloduroarteriosynangiosis when she was 10yrs 4month old. Case 3 suffered from spontaneous bacterial peritonitis and recurrent rhabdomyolysis along with neutropenia and thrombocytopenia before KT. In three years of KT his neutropenia and thrombocytopenia aggravated and bone marrow study suggested aplastic anemia. Hematopoietic stem cell transplantation is under consideration. In summary, SIOD is a rare systemic disease presenting as FSGS. While KT is indicated for their ESRD, their extra-renal symptoms progress regardless of KT. With its defective cellular immunity multiple infections complicate the clinical course after KT in SIOD.

Postoperative radiographic evaluation and simulation study for optimal cup placement in high-hip centre total hip arthroplasty.

Achieving favourable outcomes in high-hip centre reconstruction in hip dysplasia requires the optimal cup height and size, which can provide sufficient bone coverage for stable cup fixation that fits the anteroposterior acetabular rim without increasing the cup height more than necessary. We retrospectively reviewed 214 patients who underwent primary total hip arthroplasty (THA) and identified 30 hips with Crowe II (n = 15) or III (n = 15) developmental dysplasia of the hip (DDH). We measured the cup-centre-edge angle (cup-CE) and the vertical and horizontal distances from the teardrop. In a simulation study, we examined the cup-CE and optimal cup size by changing the cup height in 5-mm increments over a distance of 15-40 mm above the inter-teardrop line using a 3-dimensional template system. Postoperative radiographic evaluation revealed a mean cup-CE of 19.9° in Crowe II hips and 15.2° in Crowe III hips; the respective mean vertical distances were 26.6 mm and 27.6 mm (p = 0.511). There was no evidence of cup loosening or lateralisation at a minimum of 7 years (7-11 years) follow-up. Simulation showed that the hip centre needed to be elevated to 20 mm to acquire a cup-CE of more than 0°. More than 10° of cup-CE could be expected by elevating the hip centre to 25 mm in both Crowe II and III. Even in severe DDH, a high-hip centre positioned approximately 25 mm superior to the inter-teardrop line was sufficient to achieve optimal bone coverage, which could lead to more secure cup fixation.

Preoperative Planning for Total Hip Arthroplasty for Neglected Developmental Dysplasia of the Hip.

Developmental dysplasia of the hip (DDH) is accompanied by morphological alterations on both the acetabular and the femoral side. Total hip arthroplasty (THA) provides effective treatment in cases of neglected DDH but requires elaborate preoperative planning. To determine the morphological changes resulting from the dysplasia, the anatomic acetabular position, the height of the femur head dislocation, the height of the femur head dislocation, and the combined anteversion must all be established. In addition, a vital and complicated process of strategizing leg length balance must be conducted in cases of severe DDH. Each type of leg length discrepancy (LLD), including bony and functional and anatomical LLD, should be evaluated in the context of the presence or absence of a fixed pelvic tilt. Moreover, with severe unilateral dislocated hips, a more inferior change in the original rotational center of the hip must be accounted for. Due to these multiple morphological changes, the accurate size of the prosthesis and the cup position are difficult to predict. In comparison with other methods, CT scan‐based 3‐dimensional templating provides the best accuracy. Despite the presence of anatomic alterations, various types of acetabular and femoral prostheses have been developed to treat hip dysplasia. Both cemented and cementless cups are used in DDH cases. In DDH accompanied by insufficient acetabular bone stock, a cemented cup combined with bone graft provides a reliable treatment. Monoblock stems can be used when the combined anteversion is less than 55°, and a modular stem system when this parameter is greater than 55°. Customized stems can be designed for DDH coupled with severe proximal femoral distortion. A ceramic‐on‐ceramic bearing is considered optimal for young DDH patients.

Inflammatory and fibrosis infiltration in synovium associated with the progression in developmental dysplasia of the hip.

Developmental dysplasia of the hip (DDH) is a common musculoskeletal disorder characterized by progressive joint soreness and limited mobility. The aim of the present study was to investigate the pathological changes and inflammatory infiltration in the hypertrophic synovium of the hip joint associated with the progression of DDH. Synovial biopsies in the hip joint are obtained from patients with moderate DDH and severe DDH during surgery. These biopsies are processed for histological and immunohistochemical (IHC) analysis and investigation of the pathological processes in a synovium, including types of inflammatory cell infiltration, synovial angiogenesis and fibrosis, neuron endings and neuropeptide invasion. Correlation analysis was performed between the mean optical density (MOD) of each antibody, and Harris hip score (HHS) and visual analogue score (VAS) using the Spearman correlation test. Chronic inflammation in the synovium was observed via the positive IHC staining of inflammatory cells, such as T cells, B cells, macrophages and leukocytes. Excessive staining of vimentin and α smooth muscle actin in the synovium of severe DDH represented significant fibrosis and angiogenesis. These targets were also significantly correlated with HHS in severe DDH. The MOD levels of CD68 (indicators of macrophage) indicated apparent correlations with HHS and VAS in patients with severe DDH. The labels of nerve fibers and pain transmission indicators were as follows: Neurofilament‑200 and substance P. Calcitonin gene‑related peptide was upregulated in the synovium of severe DDH in contrast to that in the synovium of moderate DDH. The MOD levels of NF‑200, SP and CGRP were correlated with VAS in severe DDH. The pathology of DDH includes chronic inflammatory cell infiltration corresponding with nerve fibers and fibroblastic proliferation, which might contribute to arthritis progression and joint soreness in DDH.

Cementless Total Hip Arthroplasty in Crowe III and IV Dysplasia: High Hip Center and Modular Necks

BackgroundTotal hip arthroplasty (THA) in severe developmental dysplasia of the hip (DDH) is a challenging procedure. The most used techniques involve anatomic cup positioning, augmentation femoral osteotomy. However, anatomic cup positioning is not always feasible in severe DDH and osteotomy nonunion may ensue. The purpose of the study was to assess the survivorship, the hip score results, the radiological parameters (fixation, loosening, component position) of a large cohort of patients with Crowe III and IV DDH, treated with high hip center and modular necks THAs. MethodsEighty-four THAs in Crowe III and IV DDH were evaluated, achieving a final follow-up of 15.1 years. All the patients were treated with the same cementless implant (modular necks and ceramic-on-ceramic coupling) and the same approach (high cup placement with slight medialization). The patients were clinically evaluated (Harris Hip score and Merle d'Aubigne and Postel score). A radiographic evaluation was performed, analyzing the orientation of the cup. ResultsEighty patients were available at the last follow-up. The clinical scores were good at the final follow-up. Two sciatic lesions occurred: one patient fully recovered. The overall survivorship was 90.5% at 15 years: only 2 cases of aseptic loosening were reported. The mean center of rotation height was 33 ± 8 mm and the medialization was 30 ± 5 mm. ConclusionA high cup placement with slight medialization is a valid technique in DDH patients. A good restoration of the offset, ceramic-on-ceramic coupling and a porous socket may provide durable results, overcoming the effects of increasing joint reaction forces related to high cup placement.

Subtrochanteric shortening osteotomy during cementless total hip arthroplasty in young patients with severe developmental dysplasia of the hip

BackgroundThis retrospective study was designed to determine complications, functional and radiographic results of transverse subtrochanteric osteotomy during cementless, modular total hip arthroplasty (THA) in a series of active patients younger than 45 years with Crowe Type-III or IV developmental dysplasia of the hip (DDH).MethodsWe followed 49 patients (56 hips) with DDH who were treated with cementless THA, where the acetabular cup was positioned in the anatomic hip center and where a simultaneous transverse femoral osteotomy was performed. Complication rate evaluation and clinical outcomes were measured by validated clinical scores and radiographic evaluation were performed at a mean follow up of 10 years (range, 4.8–14.3 years).ResultsThe mean limb-length discrepancy was reduced from 4.2 cm to 1.1 cm (P < 0.01). The mean Harris hip score (HSS) significantly improved from 40.6 points to 87.4 points (P < 0.01). Similarly, severity of low back pain, modified MAP, HOOS, and SF-12 also showed significant improvement (P < 0.01). There were 3 cases of postoperative dislocation, 3 cases of transient nerve palsy, 2 cases of nonunion, and 4 cases of intraoperative fracture. At 10 years follow-up, the estimated survival rate with any component revision as end points was 92%.ConclusionThe cementless THA combined with transverse subtrochanteric osteotomy is a reliable technique with restoration of a more normal limb, satisfactory clinical outcomes, and mid-term survival of components.

Novel technique for intraoperative sciatic nerve assessment in complex primary total hip arthroplasty: a pilot study.

Sciatic nerve injury (SNI) is a potentially devastating complication after total hip arthroplasty (THA). Intraoperative neural monitoring has been found in several studies to be useful in preventing SNI, but can be difficult to implement. In this study, we examine the results of using a handheld nerve stimulator for intraoperative sciatic nerve (SN) monitoring during complex THA requiring limb lengthening and/or significant manipulation of the SN. A consecutive series of 11 cases (9 patients, 11 hips) with either severe developmental dysplasia of the hip (Crowe 3-4) or other underlying conditions requiring complex hip reconstruction involving significant leg lengthening and/or nerve manipulation. SN function was monitored intraoperatively by obtaining pre- and post-reduction thresholds during component trialing. The results of nerve stimulation were then used to influence intraoperative decision-making. No permanent postoperative SN complications occurred, with an average increase of 28.5 mm in limb length, range (6-51 mm). In 2 out of 11 cases, a change in nerve response was identified after trial reduction, which resulted in an alternate surgical plan (femoral shortening osteotomy and downsizing femoral head). In the remaining cases, the stimulator demonstrated a response consistent with the baseline assessment, assuring that the appropriate lengthening was achieved without SNI. 1 patient had a transient motor and sensory peroneal nerve palsy, which resolved within 2 weeks. The intraoperative use of a handheld nerve stimulator facilitates surgical decision-making and can potentially prevent SNI. The real-time assessment of nerve function allows immediate corrective action to be taken before nerve injury occurs.