Dataset Information

Total Hip Arthroplasty in A Young Patient with Bernard-Soulier Syndrome.

ABSTRACT: The management of patients with coagulopathic disorders undergoing orthopaedic surgery requires a dedicated, multi-disciplinary team with detailed perioperative planning. Bernard-Soulier Syndrome (BSS) is an extremely rare disorder, affecting 1 in 1 million individuals worldwide. It is caused by a deficiency in glycoprotein 1b-V-IX which is required for normal platelet-mediated clot formation. The deficiency results in prolonged bleeding time with high risk of spontaneous bleeds. Few reports exist in the clinical literature of BSS patients undergoing major surgery.A 40 year old, female with known BSS and developmental dysplasia of her left hip (DDH) was referred to us for consideration of left total hip arthroplasty (THA). Consultation with her Haematologist for pre-operative optimization of platelets and related clotting times together with detailed discussions of her intended anaesthesia protocol and surgery resulted in a successful operation with less than anticipated blood loss. She entered our rehabilitation program just one week after surgery.BSS is an extremely rare bleeding disorder that puts patients at very high risk of blood loss following surgery. This is the first report that we are aware of describing a BSS patient undergoing a THA. A cohesive, highly specialized, multi-disciplinary team is crucial to the success of these patients.

SUBMITTER: Bisland S

PROVIDER: S-EPMC4719372 | biostudies-other | 2014 Apr-Jun

REPOSITORIES: biostudies-other

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Total Hip Arthroplasty in A Young Patient with Bernard-Soulier Syndrome.

Bisland Stuart S Smith Frank F

Journal of orthopaedic case reports 20140401 2

<h4>Introduction</h4>The management of patients with coagulopathic disorders undergoing orthopaedic surgery requires a dedicated, multi-disciplinary team with detailed perioperative planning. Bernard-Soulier Syndrome (BSS) is an extremely rare disorder, affecting 1 in 1 million individuals worldwide. It is caused by a deficiency in glycoprotein 1b-V-IX which is required for normal platelet-mediated clot formation. The deficiency results in prolonged bleeding time with high risk of spontaneous bl ...[more]

PMID: 27298957

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Project description:BackgroundMany risk factors have been described for dislocation following total hip arthroplasty (THA), yet a patient-specific risk assessment tool remains elusive. The purpose of this study was to develop a high-dimensional, patient-specific risk-stratification nomogram that allows dynamic risk modification based on operative decisions.MethodsIn this study, 29,349 THAs, including 21,978 primary and 7371 revision cases, performed between 1998 and 2018 were evaluated. During a mean 6-year follow-up, 1521 THAs were followed by a dislocation. Patients were characterized, through individual-chart review, according to non-modifiable factors (demographics, indication for THA, spine disease, prior spine surgery, and neurologic disease) and modifiable operative decisions (operative approach, femoral head diameter, and type of acetabular liner [standard, elevated, constrained, or dual-mobility]). Multivariable regression models and nomograms were developed with dislocation as a binary outcome at 1 year and 5 years postoperatively.ResultsDislocation risk, based on patient-specific comorbidities and operative decisions, was wide-ranging-from 0.3% to 13% at 1 year and from 0.4% to 19% at 5 years after primary THA, and from 2% to 32% at 1 year and from 3% to 42% at 5 years after revision THA. In the primary-THA group, the direct anterior approach (hazard ratio [HR] = 0.27) and lateral approach (HR = 0.58) decreased the dislocation risk compared with the posterior approach. After adjusting for the approach in that group, the combination of a ≥36-mm-diameter femoral head and an elevated liner yielded the largest decrease in dislocation risk (HR = 0.28), followed by dual-mobility constructs (HR = 0.48). In the patients who underwent revision THA, the adjusted risk of dislocation was most markedly decreased by the use of a dual-mobility construct (HR = 0.40), followed by a ≥36-mm femoral head and an elevated liner (HR = 0.88). The adjusted risk of dislocation after revision THA was decreased by acetabular revision (HR = 0.58), irrespective of whether other components were revised.ConclusionsOur patient-specific dislocation risk calculator, which was strengthened by our use of a robust multivariable model that accounted for comorbidities associated with instability, demonstrated wide-ranging patient-specific risks based on comorbidity profiles. The resultant nomograms can be used as a screening tool to identify patients at high risk for dislocation following THA and to individualize operative decisions for evidence-based risk mitigation.Level of evidencePrognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Dataset Information

Total Hip Arthroplasty in A Young Patient with Bernard-Soulier Syndrome.

Publications

Total Hip Arthroplasty in A Young Patient with Bernard-Soulier Syndrome.

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