Scoliosis-specific exercises: A state of the Art Review.

Scoliosis-specific exercises are prescribed and used by many centers either primarily or as an adjunct to other treatment methods for adolescent idiopathic scoliosis of mild to moderate severity. These methods are often used in Europe and are being used in the United States with increasing frequency. There are few publications about these methods in the spine or orthopedic literature. We have performed a review of the relevant literature from the PubMed database to evaluate the evidence for effectiveness of these treatments. We found no studies that provide valid evidence that an exercise method prevents progression of AIS (adolescent idiopathic scoliosis) in patients during their peak growth period. We report our review of selected relevant efforts and provide a description of many of the available treatment programs.

Probing cortical excitability using rapid frequency tagging.

Frequency tagging has been widely used to study the role of visual selective attention. Presenting a visual stimulus flickering at a specific frequency generates so-called steady-state visually evoked responses. However, frequency tagging is mostly done at lower frequencies (<30 Hz). This produces a visible flicker, potentially interfering with both perception and neuronal oscillations in the theta, alpha and beta band. To overcome these problems, we used a newly developed projector with a 1440 Hz refresh rate allowing for frequency tagging at higher frequencies. We asked participants to perform a cued spatial attention task in which imperative pictorial stimuli were presented at 63 Hz or 78 Hz while measuring whole-head magnetoencephalography (MEG). We found posterior sensors to show a strong response at the tagged frequency. Importantly, this response was enhanced by spatial attention. Furthermore, we reproduced the typical modulations of alpha band oscillations, i.e., decrease in the alpha power contralateral to the attentional cue. The decrease in alpha power and increase in frequency tagged signal with attention correlated over subjects. We hereby provide proof-of-principle for the use of high-frequency tagging to study sensory processing and neuronal excitability associated with attention.

Correction: Pomalidomide, bortezomib, and low-dose dexamethasone in lenalidomide-refractory and proteasome inhibitor-exposed myeloma.

Following the publication of this article, the authors noted that the pomalidomide dose for the additional SC cohort in Fig. 1 was incorrectly listed. The correct dose for pomalidomide in the additional SC cohort should be the maximum tolerated dose of 4 mg/day, not 2 mg/day as listed in the original Fig. 1. The authors apologize for any inconvenience caused.

Pomalidomide, bortezomib and low-dose dexamethasone in lenalidomide-refractory and proteasome inhibitor-exposed myeloma.

This phase 1 dose-escalation study evaluated pomalidomide, bortezomib (subcutaneous (SC) or intravenous (IV)) and low-dose dexamethasone (LoDEX) in lenalidomide-refractory and proteasome inhibitor-exposed relapsed or relapsed and refractory multiple myeloma (RRMM). In 21-day cycles, patients received pomalidomide (1-4 mg days 1-14), bortezomib (1-1.3 mg/m2 days 1, 4, 8 and 11 for cycles 1-8; days 1 and 8 for cycle ⩾9) and LoDEX. Primary endpoint was to determine the maximum tolerated dose (MTD). Thirty-four patients enrolled: 12 during escalation, 10 in the MTD IV bortezomib cohort and 12 in the MTD SC bortezomib cohort. Patients received a median of 2 prior lines of therapy; 97% bortezomib exposed. With no dose-limiting toxicities, MTD was defined as the maximum planned dose: pomalidomide 4 mg, bortezomib 1.3 mg/m2 and LoDEX. All patients discontinued treatment by data cutoff (2 April 2015). The most common grade 3/4 treatment-emergent adverse events were neutropenia (44%) and thrombocytopenia (26%), which occurred more frequently with IV than SC bortezomib. No grade 3/4 peripheral neuropathy or deep vein thrombosis was reported. Overall response rate was 65%. Median duration of response was 7.4 months. Pomalidomide, bortezomib and LoDEX was well tolerated and effective in lenalidomide-refractory and bortezomib-exposed patients with RRMM.

A glimpse into Texas Scottish Rite Hospital's educational, clinical care, and research development.

This article describes four areas of pediatric orthopaedic education, research, and clinical care undertaken at Texas Scottish Rite Hospital (TSRH) over the past 25 years. These areas include the weekly preoperative conferences, the evolution of the limb lengthening and deformity correction program, the development of the TSRH instrumentation system, and the evolution of the clubfoot treatment program.

Perfusion MRI in Early Stage of Legg-Calvé-Perthes Disease to Predict Lateral Pillar Involvement: A Preliminary Study.

BACKGROUND: Current radiographic classifications for Legg-Calvé-Perthes disease cannot be applied at the early stages of the disease. The purpose of this study was to quantify the perfusion of the femoral epiphysis in the early stages of Legg-Calvé-Perthes disease with use of perfusion magnetic resonance imaging (MRI) and to determine if the extent of epiphyseal perfusion can predict the lateral pillar involvement at the mid-fragmentation stage. METHODS: Twenty-nine patients had gadolinium-enhanced perfusion MRI at the initial stage or early fragmentation stage of Legg-Calvé-Perthes disease and were followed prospectively. The percent perfusion of the whole epiphysis and its lateral third was measured by four independent observers using image analysis software. The radiographs obtained at the mid-fragmentation stage were used for the lateral pillar classification. Intraclass correlation coefficient (ICC) and logistic regression analyses were performed. RESULTS: The mean age (and standard deviation) at diagnosis was 7.7 ± 1.7 years (range, 5.3 to 11.3 years). The mean interval between the MRI and the time of maximum fragmentation was 8.2 ± 5.5 months. The interobserver ICC for the percent perfusion of the lateral third of the epiphysis was 0.90 (95% confidence interval [CI]: 0.83 to 0.95). The mean percent perfusion of the lateral third of the epiphysis was 92% ± 2%, 68% ± 18%, and 46% ± 12% for the hips in which the lateral pillar was later classified as A, B, and C, respectively (p = 0.001). When the perfusion level was ≥90% in the lateral third of the epiphysis, the odds ratio of the lateral pillar being later classified as group A, as opposed to B or C, was 72.0 (CI: 3.5 to 1476). With a perfusion level of ≤55% in the lateral third of the epiphysis, the odds ratio of the lateral pillar being later classified as group C, as opposed to A or B, was 33.3 (CI: 2.8 to 392). Similar results were obtained for the whole epiphysis. CONCLUSIONS: Perfusion MRI measurements of the total epiphysis and its lateral third obtained at the early stages of Legg-Calvé-Perthes disease were predictive of lateral pillar involvement at the mid-fragmentation stage of the disease. LEVEL OF EVIDENCE: Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence.

Bracing for idiopathic scoliosis: how many patients require treatment to prevent one surgery?

BACKGROUND: Although the efficacy of bracing for adolescent idiopathic scoliosis has been debated, recent evidence indicates a strong dose-response effect with respect to preventing curve progression of ≥6°. The purpose of this study was to investigate whether bracing, prescribed with use of current criteria, prevents surgery and how many patients must be treated with bracing to prevent one surgery. METHODS: Of 126 patients with adolescent idiopathic scoliosis measuring between 25° and 45° and with a Risser sign of ≤2, 100 completed a prospective study in which they were managed with a Boston brace fitted with a heat sensor that measured brace wear. Noncompliant patients were compared both with highly compliant patients and with the entire cohort, with the end point of progression to surgery. The absolute risk reduction (ARR) was calculated and used to calculate the number needed to treat (NNT) to prevent one surgery. RESULTS: Bracing was not effective in preventing surgery unless the patient was highly compliant with brace wear. For patients who were considered to be highly compliant, based on the hours per day that they wore the brace, the NNT was 3 (95% confidence interval [CI], 2 to 7). CONCLUSIONS: Within the limitations of a nonrandomized prospective study design, bracing for adolescent idiopathic scoliosis was found to substantially decrease the risk of curve progression to a range requiring surgery when patients were highly compliant with brace wear. Since many patients avoid surgery without wearing a brace, current indications appear to lead to marked overtreatment. Bracing appears to decrease the risk of curve progression to a magnitude requiring surgery, but current bracing indications include many curves that would not have progressed to a magnitude requiring surgery even if the patient had not worn the brace, and overall compliance with brace wear is low. Identifying these lower-risk patients and improving the compliance of those likely to have curve progression could substantially improve bracing results.

Microparticles in health and disease.

Microparticles (MPs), small membrane-derived vesicles, are derived from many cell types and released into the circulation. Microparticles can express antigens, and contain cell surface proteins, cytoplasmic contents, and nuclear components from their cell of origin that determines their composition, characterization, and transfer of biologic information. Certain prompts for this release include shear stress, complement activation, proapoptotic stimulation, cellular damage, or agonist interaction with cell surface receptors. Release can be physiologic or pathologic and is associated with proinflammatory and procoagulant effects and has been implicated in thrombotic states. Microparticles also contribute to systemic inflammation and cardiovascular, hematologic, and oncologic disease states. The study of MPs in human medicine is rapidly advancing and extends into the physiology of health, the pathophysiology of disease, and the role of MPs in transfusion medicine. In veterinary medicine, published work on MPs has been limited to the area of inherited disorders, blood storage, and leukoreduction (LR). Microparticle research is still in its infancy, and this review should be seen as a snapshot of what is currently known. As research continues important limitations, including variations in preanalytic variables such as collection, storage, or centrifugation, and limitations of quantitation are coming to the forefront. Correlation of quantitation of MPs with assays of activity will hopefully shed light on the true nature of MPs in health and disease. This review will focus on the role of cellular exocytic vesiculation in health, disease, and transfusion medicine.

Source of protein supplementation during in vitro culture does not affect the quality of resulting blastocysts in the domestic cat.

The objective of this study was to assess and compare the quality of cat blastocysts produced in vitro using commercial blastocyst growth media supplemented with different sources of proteins (serum protein substitute from in vitro maturation through embryo development vs 4 mg/ml of bovine serum albumin for maturation and 5% foetal calf serum for fertilization and embryo development). Impact was specifically examined on the proportion of blastocyst formation, total number of blastomeres, proportion of inner cell mass and expression of pluripotency marker proteins NANOG and OCT-4. Blastocyst formation per total cleaved embryos was similar (p > 0.05) regardless of the protein supplementation. There were no differences (p > 0.05) between culture conditions regarding average number of blastomeres and proportion of inner cell mass in each embryo. Presence of OCT-4 protein was detected in nuclei of both trophectoderm and inner cell mass region, with a stronger signal in the latter regardless of the culture medium. NANOG protein also was present in the inner cell mass regardless of the in vitro culture condition. We therefore demonstrated that serum protein substitute was as good as semi-defined protein sources for the production of good-quality blastocysts and embryonic stem cells. In addition, a single defined medium could be successfully used for cat oocyte maturation, in vitro fertilization and embryo development.

Posterior spinal fusion in patients with Ehlers-Danlos syndrome: a report of six cases.

PURPOSE: There is a paucity of literature describing posterior spinal fusion (PSF) in the Ehlers-Danlos syndrome (EDS) patient. The vast majority of these studies diagnosed EDS clinically. The purpose of this study is to discuss the management and complications of EDS patients with scoliosis treated with PSF at a single institution. METHODS: Clinical and radiographic data are presented describing six patients who had PSF for EDS. The diagnoses were confirmed by a geneticist. RESULTS: All of the patients in the current cohort underwent posterior fusion only, with no anterior approach. Neuromonitoring was also used in the majority. Half of our patients experienced complications. One patient had a hemoperitoneum that was initially treated nonoperatively but, unfortunately, they expired 1 month after discharge from abdominal bleeding. Another patient suffered neuropathic pain attributed to the type of implant used. A third underwent a total of seven procedures beginning at the age of 3 years in a different era of spinal surgery. The mean major curve, percentage correction, and estimated blood loss of the current cohort are similar to previous studies. CONCLUSION: The fragility of the EDS patient population cannot be overlooked. Despite a conservative surgical approach, half of our patients experienced complications. The surgeon choosing to operate on EDS patients must do so with extreme caution.

How much varus is optimal with proximal femoral osteotomy to preserve the femoral head in Legg-Calvé-Perthes disease?

BACKGROUND: Although proximal femoral varus osteotomy is an established operative treatment for Legg-Calvé-Perthes disease, there is a lack of data on how much varus at the osteotomy is optimal for preserving the spherical shape of the femoral head. The purpose of this study was to determine if there is a correlation between the amount of varus used and the Stulberg radiographic outcome at maturity and to determine if the varus angulation improved over time. METHODS: The database and the radiographs of fifty-two patients treated with proximal femoral varus osteotomy from a multicenter prospective study were analyzed. The neck-shaft angles were measured before the operation, after the operation, and at the time of final follow-up, and the amount of varus placed was correlated with the Stulberg outcome at skeletal maturity. Spearman correlations and logistic regression were used for statistical analysis. RESULTS: All patients were six years of age or older at the time of diagnosis (mean 8.0 ± 1.4 years), and all were skeletally mature at the time of follow-up (mean age [and standard deviation] at the time of follow-up, 16.5 ± 2.0 years). The mean neck-shaft angle was 138° ± 7° preoperatively and 115° ± 11° postoperatively with a mean varus change of 23° ± 10°. No significant correlation was observed between the postoperative neck-shaft angle at maturity and the Stulberg outcome (r = -0.15, p = 0.17) and between the amount of varus placed and the Stulberg outcome (r = 0.14, p = 0.36). However, when the lateral pillar groups were analyzed separately with use of logistic regression, a significant relationship was observed for the lateral pillar group B (p = 0.025), with a higher postoperative neck-shaft angle having a greater probability of being associated with a Stulberg class-I or II outcome. At maturity, the mean neck-shaft angle had improved from 115° ± 11° following the initial surgery to 124° ± 8°. However, nineteen (37%) of the fifty-two patients showed no improvement (defined as a change of >5°) and fifteen (29%) of the fifty-two patients had an overriding greater trochanter. CONCLUSIONS: Contrary to the conventional belief, greater varus angulation does not necessarily produce better preservation of the femoral head following proximal femoral varus osteotomy. Given the results, our recommendation is to achieve 10° to 15° of varus correction when performing proximal femoral varus osteotomy on hips that are in the early stages of Legg-Calvé-Perthes disease.

Brace wear control of curve progression in adolescent idiopathic scoliosis.

BACKGROUND: The efficacy of brace treatment for patients with adolescent idiopathic scoliosis remains controversial, and effectiveness remains unproven. We accurately measured the number of hours of brace wear for patients with this condition to determine if increased wear correlated with lack of curve progression. METHODS: Of 126 patients with adolescent idiopathic scoliosis curves measuring between 25 degrees and 45 degrees , 100 completed a prospective study in which they were managed with a Boston brace fitted with a heat sensor that measured the exact number of hours of brace wear. Orthopaedic teams prescribed either sixteen or twenty-three hours of brace wear and were blinded to the wear data. At the completion of treatment, the number of hours of brace wear were compared with the frequency of curve progression of > or =6 degrees and with curve progression requiring surgery. RESULTS: The total number of hours of brace wear correlated with the lack of curve progression. This effect was most significant in patients who were at Risser stage 0 (p = 0.0003) or Risser stage 1 (p = 0.07) at the beginning of treatment and in patients with an open triradiate cartilage at the beginning of treatment. Logistic regression analyses showed a "dose-response" curve in which the greater number of hours of brace wear correlated with lack of curve progression. Brace wear to school and immediately afterward was most successful. Curves did not progress in 82% of patients who wore the brace more than twelve hours per day, compared with only 31% of those who wore the brace fewer than seven hours per day (p = 0.0005). The number of hours of brace wear also correlated inversely with the need for surgical treatment (p = 0.0005). The number of hours of wear were similar for the patients who were advised to wear the brace sixteen or twenty-three hours daily. CONCLUSIONS: The Boston brace is an effective means of controlling curve progression in patients with adolescent idiopathic scoliosis when worn for more than twelve hours per day.

Characterization of edible film fabricated with channel catfish (Ictalurus punctatus) gelatin extract using selected pretreatment methods.

Farm-raised catfish are important to the economy of the southeastern states in the United States, and catfish processing produces about 55% of by-products for inexpensive sale. Therefore, the utilization of catfish by-products is of great interest to the catfish industry. The objectives of this research were to determine the optimum pretreatment method to extract catfish gelatin for edible film application, and to characterize physical, mechanical, and barrier properties of edible films fabricated with catfish skin gelatin. Catfish skins obtained from a local plant were treated with 6 selected pretreatment methods. The main extraction was performed with deionized water at 50 degrees C after pretreatment. The gelatin yield was calculated and sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) was performed to characterize molecular weight (MW) profile. Color, tensile strength (TS), elongation, and water barrier property were determined to characterize the fabricated catfish gelatin films. From the results of gelatin yield, color, SDS-PAGE, as well as mechanical and barrier properties of the film, the pretreatment method with 0.25 M NaOH and 0.09 M acetic acid, followed by extraction at 50 degrees C for 3 h, was determined as the optimum extraction method. The catfish gelatin exhibited higher MW fractions than commercial mammalian gelatin. The catfish gelatin extracts possessed film-forming properties determined by TS, elongation, and water vapor permeability (WVP) comparable to those of commercial mammalian gelatin. The selected formula for catfish gelatin film was determined as 1% gelatin and 20% glycerol, resulting in greatest TS and lowest WVP.

Mutations responsible for Larsen syndrome cluster in the FLNB protein.

BACKGROUND: A gene for Larsen syndrome was recently described, and mutations were reported in five cases. OBJECTIVE: To test whether mutations in this gene, FLNB, could explain the disease in our independent collection of sporadic and dominant Larsen syndrome cases; and to test whether mutations occurred in a non-random pattern. RESULTS: Missense mutations were found in each of five cases. Four of the five were new; one was reported in a sporadic case in the original Larsen syndrome study of five cases. All mutations from the two studies were compiled. Clustered mutations were observed within three filamin B protein domains: the calponin homology 2 domain, repeat 14, and repeat 15. This suggested that as few as five (of the total of 46) coding exons of FLNB could be screened to detect Larsen syndrome mutations. Four of these exons were screened in a sixth (sporadic) case and a previously reported G1691S substitution mutation detected. CONCLUSIONS: Mutations in FLNB may be responsible for all cases of Larsen syndrome. They appear to occur in specific functional domains of the filamin B protein. This should simplify diagnostic screening of the FLNB gene. Analyses in larger patient series are warranted to quantify this. The study confirmed the extreme variability in clinical presentation and the presence of unaffected carriers. A molecular screen would be valuable for diagnosis and genetic counselling.

External beam radiation therapy after radical prostatectomy: efficacy and impact on urinary continence.

INTRODUCTION AND OBJECTIVES: The efficacy of adjuvant and salvage external beam radiation (AXRT+SXRT) for prostate cancer after radical prostatectomy (RP) has been debated because of the inability to rule out systemic occult metastasis, uncertainty that radiation eradicates residual local disease and the potential of exacerbating impotency and incontinence. To characterize the effectiveness and treatment morbidity a retrospective review was performed. METHODS: In all, 38 patients received AXRT and 91 received SXRT. The SXRT group was stratified by PSA level, age, race, pathologic stage, margin status, worst Gleason sum, radiation dose and pelvic field. Complications evaluated were impotence and incontinence. Median follow-up was 60.2 months. RESULTS: The 5-y disease-free survival (DFS) rate was 61.3% for AXRT and 36.3% for SXRT. Multivariate analysis of the SXRT cohort showed Gleason score, pathologic stage and pre-XRT PSA to be predictors of disease recurrence. After XRT 26% had worsened continence. CONCLUSIONS: Patients who recur after RP whose pathologic stage is pT2 or pT3c, Gleason score of 8 or higher or pre-XRT PSA is >2.0 ng/dl may have microscopic metastatic disease and a decreased chance of cure with SXRT alone. Continence was further impaired after XRT.

Complete embedding and close step-sectioning of radical prostatectomy specimens both increase detection of extra-prostatic extension, and correlate with increased disease-free survival by stage of prostate cancer patients.

The objectives of this work were to evaluate the efficacy of controlled close step-sectioned and whole-mounted radical prostatectomy specimen processing in prediction of clinical outcome as compared to the traditional processing techniques. Two-hundred and forty nine radical prostatectomy (RP) specimens were whole-mounted and close step-sectioned at caliper-measured 2.2-2.3 mm intervals. A group of 682 radical prostatectomy specimens were partially sampled as control. The RPs were performed during 1993-1999 with a mean follow-up of 29.3 months, pretreatment PSA of 0.1-40, and biopsy Gleason sums of 5-8. Disease-free survival based on biochemical or clinical recurrence and secondary intervention were computed using a Kaplan-Meier analysis. There were no significant differences in age at diagnosis, age at surgery, PSA at diagnosis, or biopsy Gleason between the two groups (P<0.05). Compared with the non-close step-sectioned group, the close step-sectioned group showed higher detection rates of extra-prostatic extension (215 (34.1%) vs, 128 (55.4%), P<0.01), and seminal vesicle invasion (50 (7.6%) vs 35 (14.7%), P<0.01). The close step-sectioned group correlated with greater 3-y disease-free survival in organ-confined (P<0.01) and specimen-confined (P<0.01) cases, over the non-uniform group. The close step-sectioned group showed significantly higher disease-free survival for cases with seminal vesicle invasion (P=0.046). No significant difference in disease-free survival was found for the positive margin group (P=0.39) between the close step-sectioned and non-uniform groups. The close step-sectioned technique correlates with increased disease-free survival rates for organ and specimen confined cases, possibly due to higher detection rates of extra-prostatic extension and seminal vesicle invasion. Close step-sectioning provides better assurance of organ-confined disease, resulting in enhanced prediction of outcome by pathological (TNM) stage.

Automatic lumbar vertebral identification using surface-based registration.

This work proposes the use of surface-based registration to automatically select a particular vertebra of interest during surgery. Manual selection of the correct vertebra can be a challenging task, especially for closed-back, minimally invasive procedures. Our method uses shape variations that exist among lumbar vertebrae to automatically determine the portion of the spinal column surface that correctly matches a set of physical vertebral points. In our experiments, we register vertebral points representing posterior elements of a single vertebra in physical space to spinal column surfaces extracted from computed tomography images of multiple vertebrae. After registering the set of physical points to each vertebral surface that is a potential match, we then compute the standard deviation of the surface error for each registration trial. The registration that corresponds to the lowest standard deviation designates the correct match. We have performed our current experiments on two plastic spine phantoms and two patients.

Efficacy of perioperative halo-gravity traction in the treatment of severe scoliosis in children.

SUMMARY: Perioperative halo traction was used in the treatment of severe scoliosis in 19 children. Diagnoses included neuromuscular, idiopathic, and congenital scoliosis. Traction was transferable between the bed and a walker or wheelchair. Thirteen patients had prior spinal surgery, and most required osteotomy. Traction was used for 6 to 21 weeks. All patients underwent spinal fusion surgery after traction, with instrumentation used in 15 patients. Improvement was achieved in all patients. The Cobb angle improved 35% from an average 84 degrees before traction (range 63 degrees -100 degrees ) to 55 degrees preceding fusion. Trunk decompensation improved in all patients. Trunk height increased 5.3 cm in traction. Response to traction did not correlate with diagnosis, patient age, or prior surgery. There were no neurologic complications. Perioperative halo-gravity traction improves trunk balance and frontal and sagittal alignment in children with severe spinal deformity. Surgical fusion was enhanced by the improved alignment, and neurologic injury was avoided.

Mechanical testing of a single rod versus a double rod in a long-segment animal model.

This study involved the mechanical testing of single-rod segmental hook fixation and double-rod segmental hook fixation in a long-segment animal model. The goals were first to compare the flexibility of a single-rod scoliosis construct with that of a double-rod construct when tested in torsion, and second, to determine the effect of not using instrumentation with every vertebral segment for the single rod. Another study found that the single-rod construct was as stiff in torsion as the standard double-rod construct in a model of 10 vertebral segments. The amount of neutral zone (NZ) rotation was tested in five calf spines using an MTS (Material Testing System) machine. Five constructs were tested and included 1) a single rod with hooks at every level except the apex; 2) a single rod with two fewer hooks; 3) a single rod with four fewer hooks; 4) a double-rod construct; and 5) no instrumentation. The amount of NZ rotation between vertebral segments was measured over 12, 10, 8, 6, 4, and 2 vertebral segments. An analysis of variance with all constructs showed that the instrumented spines had significantly less movement than did the uninstrumented spine. Statistical comparison using analysis of variance of constructs (constructs 1 to 4) showed that over 12 vertebral segments (T4-L3), all single-rod constructs (constructs 1 to 3) allowed more NZ rotation than did the standard double-rod construct. This testing indicated that over 12 vertebral segments the single rod allowed more NZ rotation than a double-rod construct.

Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience.

PURPOSE: von Hippel-Lindau disease, hereditary papillary renal cell carcinoma, the Birt-Hogg-Dubé syndrome and familial renal oncocytoma are familial renal tumor syndromes. These hereditary disorders are noteworthy for the development of multiple bilateral renal tumors and the risk of new tumors throughout life. One management strategy is observation of solid renal tumors until reaching 3 cm, then performing parenchymal sparing surgery. We present a 5-year update on our experience. MATERIALS AND METHODS: From May 1988 to October 1998, 49 patients with hereditary renal cell carcinoma, including von Hippel-Lindau disease in 44, hereditary papillary renal cell carcinoma in 4 and the Birt-Hogg-Dubé syndrome in 1, and 1 with familial renal oncocytoma underwent exploration to attempt renal parenchymal sparing surgery. Patients were followed prospectively with periodic screening for recurrence, metastasis and loss of renal function. Median followup was 79.5 months (range 0.7 to 205). RESULTS: A total of 50 patients underwent 71 operations resulting in unilateral nephrectomy in 6, bilateral nephrectomy in 1 and partial nephrectomy in 65, with 1 to 51 tumors removed from each kidney (mean 14.7). Mean patient age was 39.5 years (range 18 to 70). Of the 65 (40%) partial nephrectomies 26 were performed with cold renal ischemia. Mean blood loss was 2.9 +/- 0.5 l (range 0.15 to 23). Postoperative complications included renal atrophy in 3 patients. Mean preoperative serum creatinine was 1.05 +/- 0.03 mg/dl (range 0.6 to 1.8), and postoperative creatinine was 1.06 +/- 0.04 mg/dl (range 0.6 to 2.0). No patient who underwent renal parenchymal sparing surgery required renal replacement therapy. Metastatic disease developed in 1 patient with a 4.5 cm renal tumor. CONCLUSIONS: Parenchymal sparing surgery with a 3 cm threshold in patients with hereditary renal cancer appears to be an effective therapeutic option to maximize renal function while minimizing the risk of metastatic disease.