OBJECTIVE The purpose of this study was to correlate skeletal pathologic findings quantified by MRI-based bone marrow burden score with genotype and spleen status and other clinical parameters, including liver size and duration of enzyme replacement therapy, in patients with Gaucher’s disease. MATERIALS AND METHODS Two radiologists retrospectively reviewed MR images of 47 patients with Gaucher’s disease and determined bone marrow burden scores by consensus on the basis of previously published criteria. The bone marrow burden scores were correlated with genotype, liver volume, spleen status, age, and cumulative duration of enzyme replacement therapy. RESULTS Subjects with compound heterozygous N370S alleles had significantly higher overall, lumbar spinal, and femoral bone marrow burden scores than did N370S homozygotes. There was a significant positive correlation between an enlarged or surgically absent spleen and bone marrow burden score. There were no significant associations between bone marrow burden score and liver volume, age, cumulative duration of enzyme replacement therapy, or cumulative duration of untreated disease. Femoral and lumbar spinal bone marrow burden scores had a weak but significant positive correlation across all patients. CONCLUSION Skeletal pathologic findings in Gaucher’s disease encapsulated as bone marrow burden score correlate significantly with the number of copies of the N370S allele, which has an ameliorative effect on bone marrow disease. Splenectomy or splenomegaly is associated with greater risk of bone marrow disease. Femoral and lumbar spinal bone marrow burden scores, although only weakly correlated, independently illustrated both the protective role of the N370S allele and the unfavorable implication of splenectomy. This finding suggests that axial and appendicular bone marrow burdens are related but distinct and justifies multiple-compartment evaluation in Gaucher’s disease.
Purpose Adults with traumatic digital amputation (TDA) of the hand may be managed with replantation or revision amputation. To date, there is no systematic review evaluating patient reported outcomes (PROs) comparing replantation versus revision amputation. Methods Three databases (MEDLINE, EMBASE, and PubMed) were systematically searched in duplicate from inception until June 13, 2019 using Covidence software. Studies comparing replantation versus revision amputation outcomes were considered for inclusion. Methodological quality was assessed using Methodologic Index for Nonrandomized Studies (MINORS) criteria. Data were pooled in a random‐effects meta‐analysis model using Revman software. Certainty of evidence was evaluated using Grading of Recommendations, Assessment, Development, and Evaluations (GRADE). Results Of 4350 studies identified, 12 retrospective cohort studies met inclusion criteria and compared TDA outcomes for replantation (n = 717; 82.9% male; mean age 40.3) versus revision amputation (n = 1046; 79.8% male; mean age 41.7). The overall replantation survival rate was 85.3%. The average MINORS score was 57% (13.75/24). Replantation of the thumb had a superior Michigan Hand Questionnaire (MHQ) score (+11.88, 95% CI [7.78–15.99], I2 = 21%) compared with revision amputation. Replantation of single non‐thumb digits had a superior MHQ score (+5.31, 95% CI [3.10–7.51], I2 = 67%) and Disability of Arm, Shoulder, and Hand (DASH) score (−5.16, 95% CI [−8.27 to −2.06], I2 = 0%) compared with revision amputation. Most patients in the meta‐analysis were from Asian populations (87.9%). Conclusion There is low‐quality evidence that thumb replantation achieves superior PROs compared with revision amputation, which may be clinically important. Replantation of single non‐thumb digits also yielded superior PROs, which is likely not clinically important and based on very low‐quality evidence. Future studies with populations outside Asia are required to determine if PROs vary based on cultural differences toward digital amputation.
High-pressure delivery devices for paint and other substances can lead to severe injuries of the hand without immediate surgical debridement. We present a case of a high-pressure paint gun injury treated surgically with full resolution of function. A systematic review of the literature details outcomes of similar injuries.
Background: The success of salvage procedures for failing digital replants (FR) is poorly documented. We sought to evaluate the success of salvage procedures for FR and factors contributing to successes and failures of replants.Methods: Adult patients who presented to our center between January 1, 2000 and December 31, 2015, suffered ≥1 digital amputation(s), and underwent digital replantation were included.Preoperative, perioperative, and postoperative details were recorded. Digits were monitored postoperatively via nursing and physician assessments. The presumed reason for failure, details, and outcomes of salvage attempts were recorded for FR. Length of hospital stay and complications were also recorded.Results: Fifty-two patients and 83 digits were included. Fifty-two digits (63%) were compromised (arterial ischemia in 15 digits; venous congestion in 37 digits) and 48 digits had salvage therapy. Twenty-one FR (44%) were salvaged via operative (1 of 2; 50%), nonoperative (19 of 43; 44%), and combined (1 of 3; 33%) therapies. FR patients were more likely than those with successful replants to receive a blood transfusion (52 vs. 23%; p = .009) with more transfused units (3.45 AE 3.30 vs. 0.86 AE 0.95; p = .001). Length of stay was prolonged for FR patients (9 [range: 2-22] vs. 7 [range: 3-19] days; p = .039). Ultimately, 59% (49 of 83) of replants were successful, where 25% (21 of 83) were successfully salvaged.Conclusion: Nonoperative and operative salvage therapies improve the rate of replant survival.We suggest close postoperative monitoring of all replants and active salvage interventions for compromised replants in the postoperative period.
Joint denervation has been proposed as a less invasive option for surgical management of hand arthritis that preserves joint anatomy while treating pain and decreasing postoperative recovery times. The purpose of this systematic review was to investigate the efficacy and safety of surgical joint denervation for osteoarthritis in the joints of the hand. EMBASE, MEDLINE, and PubMed databases were searched from January 2000 to March 2019. Studies of adult patients with rheumatoid arthritis or osteoarthritis of the hand who underwent joint denervation surgery were included. Two reviewers performed the screening process, data abstraction, and risk of bias assessment (Methodological Index for Non-Randomized Studies). This review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and was registered with PROSPERO (#125811). Ten studies were included, 9 case series and 1 cohort study, with a total of 192 patients. In all studies, joint denervation improved pain and hand function at follow-up (M = 36.8 months, range = 3-90 months). Pooled analysis of 3 studies on the first carpometacarpal joint showed a statistically significant ( P < .001) reduction in pain scores from baseline (M = 6.61 ± 2.03) to postoperatively (M = 1.69 ± 1.27). The combined complication rate was 18.8% (n = 36 of 192), with neuropathic pain or unintended sensory loss (8.8%, n = 17 of 192) being the most common. This review suggests that denervation may be an effective and low-morbidity procedure for treating arthritis of the hand. Prospective, comparative studies are required to further understand the outcomes of denervation compared with traditional surgical interventions.
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