Citations

BACKGROUND Waldenström macroglobulinemia (WM) is preceded by asymptomatic WM (AWM), for which the risk of progression to overt disease is not well defined. METHODS We studied 439 patients with AWM, who were diagnosed and observed at Dana-Farber Cancer Institute between 1992 and 2014. RESULTS During the 23-year study period, with a median follow-up of 7.8 years, 317 patients progressed to symptomatic WM (72%). Immunoglobulin M 4,500 mg/dL or greater, bone marrow lymphoplasmacytic infiltration 70% or greater, β2-microglobulin 4.0 mg/dL or greater, and albumin 3.5 g/dL or less were all identified as independent predictors of disease progression. To assess progression risk in patients with AWM, we trained and cross-validated a proportional hazards model using bone marrow infiltration, immunoglobulin M, albumin, and beta-2 microglobulin values as continuous measures. The model divided the cohort into three distinct risk groups: a high-risk group with a median time to progression (TTP) of 1.8 years, an intermediate-risk group with a median TTP of 4.8 years, and a low-risk group with a median TTP of 9.3 years. We validated this model in two external cohorts, demonstrating robustness and generalizability. For clinical applicability, we made the model available as a Web page application ( www.awmrisk.com ). By combining two cohorts, we were powered to identify wild type MYD88 as an independent predictor of progression (hazard ratio, 2.7). CONCLUSION This classification system is positioned to inform patient monitoring and care and, for the first time to our knowledge, to identify patients with high-risk AWM who may need closer follow-up or benefit from early intervention.

Background: The morphologic effect of squamosal synostosis has not been clarified, due to its low occurrence rate, and its inclination to be combined with premature closure of other major sutures, especially in syndromic synostosis. This study's goal is to explore the morphological influence of squamosal synostosis in both syndromic and nonsyndromic craniosynostosis patients. Methods: Twenty-five computed tomography (CT) scans from nonsyndromic squamosal synostosis (NSS), Crouzon syndrome with squamosal synostosis (CSS), Crouzon syndrome without squamosal synostosis, and normal controls were included. Three-dimensional (3D) cephalometry, entire cranial volume and cranial fossa volume were measured using Materialise software. Results: The entire cranial volume of all groups was similar. The NSS developed a gradual reduction in the severity of reduced segmental volume from the anterior cranial fossa, to the middle cranial fossa, and to the unaffected volume of posterior cranial fossa. The CSS developed the most severe volume reduction of middle cranial fossa (12608.30 ± 2408.61 mm3 vs 26077.96 ± 4465.74 mm3, 52%), with proportionate volume reduction of the anterior (7312.21 ± 2435.97 mm3 vs 10520.63 ± 2400.43 mm3, 30%) and posterior cranial fossa (33487.29 ± 5598.93 mm3 vs 48325.04 ± 14700.44 mm3, 31%). The NSS developed a narrower anterior cranial fossa, a clockwise rotated Frankfort horizontal plane, and a retracted chin (pogonion). Conclusion: Squamosal synostosis may alter the development of cranial fossa volume, especially the volume of middle cranial fossa. However, the development of cranial base, and related facial features are determined largely by major vault sutures and associated syndromic conditions, rather than the squamosal suture alone. Level of Evidence: II

Background From infancy to adulthood, the mandible develops increased ramus height, prominence of the chin, and laterally widened gonial angles. In Crouzon and Apert syndromes, both relative retrognathia and prognathic jaws have been reported. Growth is influenced by a variety of factors, including the growth and relative position of the skull base, functional coordination, and the spatial influence of the laryngopharynx. Thus, this study aimed to explore in detail the evolution of the mandible in both syndromes and its relationship with the entire facial structure and skull base. Methods One hundred twenty-three preoperative computed tomographic scans (Crouzon, n = 36; Apert, n = 33; control, n = 54) were included and divided into 5 age subgroups. Computed tomographic scans were measured using Materialise software. Cephalometrics relating to the mandible, facial structures, and cranial base were collected. Statistical analyses were performed using t test and statistical power analysis. Results In Crouzon syndrome, the angle between the cranial base and gnathion was increased prior to 6 months of age by 10.29 degrees (P < 0.001) and by adulthood to 11.95 degrees (P = 0.003) compared with normal. After 6 months of age, the distance between bilateral mandibular condylions (COR-COL) was narrower by 15% (P < 0.001) in Crouzon syndrome compared with control subjects. Before 6 months of age, Apert COR-COL decreased 16% (P < 0.001) compared with control subjects and 13% (P = 0.006) narrower than Crouzon. During 2 to 6 years of age, Apert mandibular ramus height caught up to, and became longer than, Crouzon by 12% (P = 0.011). The nasion-sella-articulare angle of the Apert skull was 5.04 degrees (P < 0.001) less than Crouzon overall. Conclusions In Crouzon syndrome, the changes of the spatial relationship of the mandible to the cranial base develop earlier than the mandibular shape deformity, whereas in Apert syndrome, the spatial and morphological changes are synchronous. The morphological changes of the mandible are disproportional in 3 directions, initially significant shortening of the mandibular width and length, and, subsequently, reduced height. Crouzon has more shortening in mandibular height compared with Apert, reflecting the more shortened posterior cranial base length. The narrowed angle between the mandible and the posterior cranial base in Apert skulls is consistent with the more limited nasopharyngeal and oropharyngeal airway space.

AbstractBackgroundThe Goldilocks mastectomy procedure involves local contouring of completely autologous breast tissue created by preserving and de‐epithelializing the residual mastectomy flaps. The purpose of this study was to provide outcomes data for 96‐Goldilocks mastectomy procedures analyzing indications, complications, relevant comorbidities, and adjuvant cancer treatment impacting reconstructive and aesthetic outcomes.MethodsComprehensive review of every patient who underwent Goldilocks mastectomy from 2012 to 2018 included relevant medical and surgical comorbidities, as well as complication profiles. Aesthetic outcomes were also assessed in those with postoperative imaging available.ResultsA total of 53 patients (96 breasts) were included in this study. Bilateral cases consisted of 81.1% of the total cohort (n = 86 cases), and 18.9% (n = 10 cases) were unilateral procedures. Mean age at the time of reconstruction was 55.8 (33‐77) years. Mean body mass index (BMI) at the time of reconstruction was 33.7 (19.2‐54.6). The overall complication rate was 9.38% (seroma = 2, hematoma = 1, cellulitis = 2, wound dehiscence = 3, and operating room take back = 1).ConclusionsGoldilocks breast reconstruction is a safe, effective option in patients with higher than average BMI or excess local breast tissue, or in patients meeting these criteria preferring a single‐stage reconstruction. This study qualifies its use in patients with higher than average risk factors for more extensive reconstructive modalities.

Abstract Background: Genicular nerve radiofrequency ablation (RFA) is an intervention to treat patients with chronic knee pain who have failed previous conservative, pharmacologic, and surgical interventions. Vascular complications following interventional procedures of the knee are extremely rare. A delay in diagnosis may be detrimental for the viability of the affected limb and may ultimately require amputation. Case Presentation: A 76-year-old man with a history of severe bilateral knee osteoarthritis and grade 4 chondromalacia presented to our clinic with refractory, severe bilateral knee pain and received a bilateral genicular nerve RFA. He returned 4 days later with right medial thigh pain and a magnetic resonance imaging study revealing a hematoma along the anteromedial aspect of the right distal femoral diaphysis measuring 13.3 × 4.5 × 3.0 cm. After collaboration between pain medicine and orthopedic surgery services, decision was made to treat patient conservatively with rest, compression, elevation, ice application, tramadol, and gabapentin, but with close follow-up and a low threshold to intervene with diagnostic and therapeutic angiography with embolization if bleeding worsened; he reported resolution of his pain after a 4-day and 1-month follow-up. Conclusion: This is the first report describing iatrogenic vascular injury in the knee after a genicular RFA procedure. Pain medicine physicians should be aware of the vascular anatomy of the knee, particularly paying close attention to variations after previous surgeries. Future trials should investigate modalities that minimize vascular complications including concomitant use of ultrasonography with fluoroscopy and other forms of RFA including pulsed or cooled RFA.

Background: Apert syndrome patients are different in clinical pathology, including obstructive sleep apnea, cleft palate, and mental deficiency. These functional deficiencies may be due to anatomic deformities, which may be caused by different forms of associated suture fusion. Therefore, a classification system of Apert syndrome based on the type of craniosynostosis pattern might be helpful in determining treatment choices. Methods: CT scans of 31 unoperated Apert syndrome and 51 controls were included and subgrouped as: class I. Bilateral coronal synostosis; class II. Pansynostosis; and class III. Perpendicular combination synostosis: a. unilateral coronal and metopic synostosis; b. sagittal with bilateral/unilateral lambdoid synostosis; and c. others. Results: Class I is the most common (55%) subtype. The cranial base angulation of class I was normal; however, the cranial base angulation on the cranium side of the skull in class II increased 12.16 degrees (P = 0.006), whereas the facial side cranial base angle of class IIIa decreased 4.31 degrees (P = 0.035) over time. The external cranial base linear measurements of class I showed more evident reduction in anterior craniofacial structures than posterior, whereas other subtypes developed more severe shortening in the posterior aspects. Conclusions: Bicoronal synostosis is the most common subtype of Apert syndrome with the normalized cranial base angulation. Combined pansynostosis patients have flatter cranial base, whereas the combined unilateral coronal synostosis have a kyphotic cranial base. Class I has more significant nasopharyngeal airway compromise in a vertical direction, whereas classes II and III have more limited oropharyngeal space.