Lupus nephritis at the University Hospital of the West Indies, Kingston, Jamaica: a 10-year experience

The case records of 75 patients who had systemic lupus erythematosus (SLE) and renal involvement on renal histology were reviewed. There were 70 females and 5 males, with a ratio of 14:1. The majority of the patients were in their second and third decades of life. SLE nephritis was distinctly uncommon in patients below 10 years of age and above 50 years of age. In nearly 80% of the patients, SLE nephritis was diagnosed within the year prior to presentation. The most common presenting clinical features were arthritis in 78% and a rash in 48%. The two most common renal features were proteinuria in 60% and renal failure in 55%. Of these patients, 20%, 12%, 48%, and 17% fell into Class II (mesangial GN), Class III (focal GN), Class IV (diffuse GN), and Class V (membranous GN), respectively, when the renal histological abnormalities were categorized according to the World Health Organization classification. The remaining 3% of patients had sclerosis and as a result were unclassifiable. The two major clinical manifestations in the Class IV patients were an acute nephritis syndrome in 83% and renal failure in 61%. The nephrotic syndrome was seen in 62% of Class V (membranous GN) cases. There was 100% survival in Classes II (mesangial GN) and V (membranous GN), and 62% percent in Classes III (focal GN) and IV (diffuse GN) combined.     

Class V lupus nephritis: a clinicopathologic study in 152 patients

BACKGROUND: Class V lupus nephritis (LN) can be divided into two subgroups according to the 1995 WHO modified classification, but the difference in clinical characteristics between these subgroups is not well known. METHODS: We classified 152 patients with Class V LN, confirmed by renal biopsy, into two subgroups (61 Class Va, 91 Class Vb), and enrolled 488 patients with Class IV as controls. The clinical manifestations, serologic results and prognosis were compared for Classes Va and Vb. RESULTS: The incidence of hypertension and anemia in Class Vb patients was significantly higher than in Class Va (38.5% vs 21.3%, 72.5% vs 52.5%, p<0.05). The incidence of hematuria and renal insufficiency in Class Vb was 64.8%and 15.4%, which was higher than Class Va (44.2% and 3.3%), but lower than Class IV (89.1% and 35%), p<0.05. The percentage of patients with positive anti-dsDNA antibody and hypocomplementemia in Class Vb tended to be higher than Class Va (35.2% vs 26.2%, 50.6% vs 31.2%). Repeated renal biopsies in 24 patients (11 Class Va, 13 Class Vb) showed that eight Class Vb patients had "transformed" to Class IV LN, while only two Class Va patients did (p<0.05). In three Class Va patients serum creatinine doubled during follow-up, but none of them progressed to end-stage renal disease (ESRD). In Class Vb serum creatinine doubled in ten patients, and three progressed to ESRD. CONCLUSIONS: The renal injury and extrarenal manifestations of Class Vb patients were severer than Class Va. Class Vb patients were more likely to shift to Class IV LN, and the prognosis was poorer than for Class Va.     

Surgical treatment of occipital lobe epilepsy

OBJECT: Occipital lobe epilepsy (OLE) accounts for a small percentage of extratemporal epilepsies and only few and mostly small patient series have been reported. Preoperative findings, surgical strategies, histopathological bases, and postoperative outcomes for OLE remain to be elucidated. METHODS: A group of 54 patients with occipital lobe involvement were identified from a prospective epilepsy surgery database established in 1989. Medical charts, surgical reports, MR imaging, and histopathology data were reviewed, and patients with additional temporal and/or parietal involvement were categorized separately. Seizure outcome was classified according to the Engel classification scheme (Classes I-IV). Two patients were excluded due to incomplete data sets. Fifty-two patients with intractable epilepsy involving predominantly the occipital lobe were included in the study, comprising 17.8% of 292 patients undergoing operations for extratemporal epilepsies. RESULTS: In nearly all cases (50 [96.2%] of 52), a structural lesion was visible on preoperative MR imaging. Of these cases, 29 (55.8%) had "pure" OLE with no temporal or parietal lobe involvement. Most patients (83%) had complex partial seizures, and 60% also had generalized seizures. All patients underwent occipital lesionectomies or topectomies; 9 patients (17.3%) underwent additional multiple subpial transections. Histopathology results revealed 9 cortical dysplasias (17.3%), 9 gangliogliomas (17.3%), 6 other tumors (11.5%), 13 vascular malformations (25%), and 15 glial scars (28.8%). Visual field deficits were present in 36.4% of patients preoperatively, and 42.4% had new or aggravated visual field deficits after surgery. After a mean follow-up of 80 months, 36 patients were seizure free (69.2% Engel Class I), 4 rarely had seizures (7.7% Engel Class II), 8 improved more than 75% (15.4% Engel Class III), and 4 had no significant improvement (7.7% Engel Class IV). Multifactorial logistic regression analysis revealed that early age at epilepsy manifestation (p = 0.031) and shorter epilepsy duration (p = 0.004) were predictive of better seizure control. All other clinical and surgical factors were not significant in predicting outcome. CONCLUSIONS: Occipital lobe epilepsy is an infrequent but significant cause of extratemporal epilepsy. Satisfactory results (Engel Class I or II) were obtained in 77% of patients in our series. Postoperative visual field deficits occurred in a significant proportion of patients. In the modern MR imaging era, lesions should be investigated in patients with OLE and lesionectomies should be performed early for a better outcome.     

Interobserver reproducibility and application of the ISN/RPS classification of lupus nephritis-a UK-wide study

We sought to assess the interobserver variation of the new International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of lupus nephritis when compared with the previous World Health Organization classification, when used by pathologists throughout the UK. We also sought differences in how the 2 classifications were applied to a single set of biopsies. Twenty unselected renal biopsies showing lupus nephritis were circulated to pathologists in the UK National Renal Pathology External Quality Assessment Scheme, before the ISN/RPS scheme was published, with a request to apply the World Health Organization classification. The same slides were recirculated approximately 1 year later with a request to apply the ISN/RPS classification. A significant improvement in interobserver reproducibility was demonstrated by the new classification (kappa 0.53 vs. 0.44, P = 0.002). The reproducibility of the assessment of disease activity and chronicity remains suboptimal (kappa = 0.33). The new classification tends to produce more diagnoses of Class IV lupus nephritis, with fewer diagnoses of Classes III and V. The improvement in interobserver reproducibility indicates that an important aim of the new classification has been achieved. Further work is needed to determine whether the increase in diagnosis of Class IV nephritis represents an improvement in biopsy interpretation or a divergence from the previous classification, as the latter could undermine attempts to relate results from the new system to treatment strategies based on clinical trials which used the old.     

Practical classification of chin deformities

A visually pleasing chin is an important component of facial harmony. This study was undertaken to introduce a practical classification of chin dysmorphology, which can be used to guide the surgeon toward the appropriate surgical approach to chin repair. Analysis of life-size photography, based on standard anthropometric measurements, was completed on 2,879 patients from 1981 to 1991. Six hundred eighty-four patients were noted to have normal occlusion with some form of chin deformity. Analysis of this group of patients identified seven categories of chin dysmorphism: Class I, macrogenia (n = 170, 24.9%); Class II, microgenia (n = 435, 63.6%); Class III, combination of macrogenia in one direction and microgenia in the other direction (n = 54, 7.9%); Class IV, asymmetry (n = 4, 0.6%); Class V, soft tissue ptosis (witch's chin) (n = 13, 1.9%); Class VI, pseudomacrogenia (normal skeletal symphysis menti with excess soft tissue covering) (n = 5, 0.73%); and Class VII, pseudomicrogenia (long-face deformity producing clockwise rotation of the normal mandible) (n = 3, 0.4%). Having diagnosed the dysmorphism, logical surgical recommendations for lower face improvement were postulated. Only patients with Class II chin deformity can be corrected with augmentation. Classes I, III, and IV require an osteotomy of the chin. Classes V and VI benefit from soft tissue correction. Class VII deformity can be corrected with a maxillary osteotomy.     

The Classification of Axial Deformity in Patients with Basilar Invagination

ObjectiveTo summarize the variation types of the axis in patients with basilar invagination (BI), then propose a classification scheme of the axis deformity.MethodsFrom December 2013 to September 2020, 92 patients (male 42, female 50) who were diagnosed with BI were studied retrospectively. Based on the imaging data of CT, the width and height of the axis pedicle and the sagittal diameter of the lateral mass were measured in each patient. According to the development of axis pedicle and lateral mass, the types of axis variation were summarized, and then the classification scheme of axis deformity was put forward.ResultsAll cases were analyzed and axis deformities were divided into four types. Type I: the axis is basically normal (53 cases, 57.6%). Type II: axis lateral mass is dysplasia (eight cases, 8.7%), which includes two subtypes: type IIA, the axis unilateral lateral mass is dysplasia (three cases); type IIB, the axis bilateral lateral masses are all dysplasia (five cases). Type III: axis pedicle is dysplasia (11 cases, 12%), which is subdivided into two subtypes: type IIIA, the axis unilateral pedicle is dysplasia (six cases); type IIIB, the axis bilateral pedicles are all dysplasia (five cases). Type IV: axis pedicle and lateral mass are all dysplasia (20 cases, 21.7%), this type contains the following four subtypes: type IVA, the unilateral axis pedicle and unilateral lateral mass (contralateral or ipsilateral) are all hypoplasia (four cases); type IVB, the unilateral axis pedicle and bilateral lateral masses are all hypoplasia (five cases); type IVC, the bilateral axis pedicles and unilateral lateral mass are all dysplasia (seven cases); type IVD, the bilateral axis pedicles and bilateral lateral masses are all dysplasia (four cases). The left and right abnormal lateral mass sagittal diameter (Type II) was (7.23 ± 1.39) mm and (5.96 ± 1.37) mm, respectively, the left and right abnormal pedicle width (Type III) was (2.61 ± 1.01) mm and (3.23 ± 0.66) mm, respectively, left and right abnormal pedicle height (Type III) was (5.43 ± 2.19) mm and (4.92 ± 1.76) mm, respectively. Moreover, the classification scheme has good repeatability and credibility.ConclusionsThe classification about axis deformity could provide personalized guidance for axis screw placement in the BI and other upper cervical surgery, and axis screw placement errors would be effectively avoided.     

Cornu cutaneum in a patient of African descent: implications and management of an uncommon lesion

Cornu cutaneum, or giant cutaneous horn (GCH), is an uncommon but disfiguring lesion that may cause the patient considerable distress. The aesthetic implications of such lesions are obvious. The etiology and treatment of GCH are more obscure, both to patients and physicians alike. We present a female patient of African descent with a GCH of the scalp, which represents the first such case to our knowledge outside Africa. The etiology and management of GCH in all races are reviewed.     

Clinical Experience with the Smeloff-Cutter Prosthesis: 1- to 12-Year Follow-up

To determine the long-term results of aortic valve replacement with the Smeloff-Cutter prosthesis, the fate of 358 of 459 (78%) consecutive patients was determined 1 to 12 years postoperatively. There were 319 male patients (70%). Mean age at operation was 57 years (range, 15 to 84 years). Aortic stenosis was the dominant lesion in 267 patients (58%) and aortic regurgitation in 133 patients (29%). Fifty-nine patients (13%) had both aortic stenosis and regurgitation. In addition to aortic valve replacement, 93 patients (20%) had coronary artery bypass, 30 (6.5%) had mitral commissurotomy, 23 (5%) had mitral valve replacement, and 41 (9%) had other procedures. Preoperative status by New York Heart Association Functional Class was: Class I, 3 (1%); Class II, 39 (8%); Class III, 148 (32%); and Class IV, 269 (59%). Operative (30-day) mortality was 8.5% (39 out of 459).Functional improvement was obtained in all postoperative survivors: 345 (82%), Class I; 63 (15%), Class II; and 12 (3%), Class III. A perivalvular leak developed in 6 patients (1%) and subacute bacterial endocarditis in five (1%). Actuarial long-term survival was 80% at 5 years and 71% at 8 years. Thromboembolism occurred in 34 patients (9.5%). The incidence of thromboembolism per 100 patient-years for patients receiving no anticoagulants was 5.4; antiplatelet agents, 2.9; and Coumadin (sodium warfarin), 2.6. Major thromboembolism was uncommon in patients on a regimen of sodium warfarin but major morbidity from bleeding was significant.     

Multiple subpial transections: outcome and complications in 20 patients who did not undergo resection

OBJECT: The authors describe patient characteristics, surgical methods, complications, and outcome over time in a cohort of patients who underwent multiple subpial transection (MST) without concomitant cortical resection. METHODS: Twenty consecutive patients in whom drug-resistant epilepsy had been diagnosed a mean of 16 +/- 9 years earlier (mean +/- standard deviation [SD]) were treated with MST without cortical resection. The mean follow-up period was 49.3 +/- 18.3 months (mean +/- SD, median 58 months). At 12 months of follow up, two of the 20 patients were Engel Class I, one was Class II, six were Class III, and 11 were Class IV. At latest follow up, one patient was Engel Class I, one was Class II, seven were Class III, and 11 were Class IV. According to an alternative five-tiered classification system, two outcomes were excellent, seven were good, one was fair, nine were poor, and one was worse. Outcome was found to be better in patients with no lesions observed on magnetic resonance (MR) imaging, and worse in those with large MST areas. Outcome had a tendency to change (this occurred in 13 of 20 cases). Five patients (25%) improved and seven (35%) deteriorated in Engel outcome class, and in one (5%) both developments occurred over time. Most outcome class changes occurred before the end of the 2nd year (nine), and four were observed in the 5th year. There where seven transient neurological deficits and four surgical complications. There was no permanent significant morbidity, and there were no deaths. CONCLUSIONS: Forty-five percent of patients achieved a worthwhile improvement after pure MST, if Engel outcome Class III is deemed a worthwhile improvement. The alternative five-tiered classification resulted in 50% with worthwhile improvement (excellent, good, or fair outcome), 45% with poor, and 5% with worse outcome. Lesions that are detectable on MR imaging, and large MST areas are predictive of worse results. Significant intraoperative problems may arise, but this happens infrequently. There is a notable rate of transient morbidity but the rate of permanent morbidity is not significant.     

Early and late risk of mitral valve replacement. A 12 year concomitant comparison of the porcine bioprosthetic and prosthetic disc mitral valves

A consecutive series of 706 mitral valve replacements was performed from January, 1972, to January, 1984. The follow-up ranged from 6 to 150 months with a mean of 50 and a median of 43 months. Seven percent (50) of the patient were lost to follow-up. There were 243 men and 463 women, whose ages ranged from 17 to 86 years (mean 58). A porcine bioprosthetic valve was implanted in 528 patients (514 Hancock and 14 Carpentier-Edwards valves) and a prosthetic disc valve in 178 patients (102 standard disc Bj?rk-Shiley, 34 Beall, and 42 Harken disc valves). Seven patients were in Functional Class II, 325 in Class III, and 374 in Class IV. A concomitant operative procedure was performed in 253 of the 706 patients (36%). Mitral regurgitation was the primary hemodynamic lesion in 363 and mitral stenosis in 343. Operative mortality figures were as follows: 77 of 706 (11%) for the overall group, 34 of 453 (7.5%) for isolated mitral valve replacement, 30 of 169 (17.5%, p = 0.001) for mitral replacement plus coronary bypass, 49 of 528 (9%) for the bioprosthetic valve group, and 28 of 178 (16%) for the prosthetic disc valve group (p = 0.01). After the operation, 262 patients were in Functional Class I, 99 in Class II, and 18 in Class III. The long-term survival rate was significantly lower in patients who had an associated procedure (45% +/- 6%), who had mitral regurgitation rather than mitral stenosis (53% +/- 5% versus 67% +/- 4%) (p = 0.002), who were in Functional Class IV rather than Classes I to III (51% +/- 4% versus 70% +/- 4%) (p = 0.001), and who received a prosthetic disc valve rather than a bioprosthesis (40% +/- 6% versus 67% +/- 4%) (p = 0.001). Thromboembolic rates were significantly higher with prosthetic valves than with bioprosthetic valves (4.6% +/- 0.22% versus 2.4% +/- 0.5% per patient-year of follow-up), and the incidence of anticoagulant-related hemorrhage was significantly higher in the prosthetic valve group (1.65% versus 0.43% per patient-year). Primary valve dysfunction was significantly more common in the bioprostheses (1.23% versus 0.40% per patient-year).(ABSTRACT TRUNCATED AT 400 WORDS)     

A modified classification for the maxillectomy defect

BACKGROUND: At present no widely accepted classification exists for the maxillectomy defect suitable for surgeons and prosthodontists. An acceptable classification that describes the defect and indicates the likely functional and aesthetic outcome is needed. METHODS: The classification is made on the basis of the assessment of 45 consecutive maxillectomy patients derived prospectively from the database (September 1992) and retrospectively from 1989. RESULTS: The classification of the vertical component is as follows: Class 1, maxillectomy without an oro-antral fistula; Class 2, low maxillectomy (not including orbital floor or contents); Class 3, high maxillectomy (involving orbital contents); and Class 4, radical maxillectomy (includes orbital exenteration); Classes 2 to 4 are qualified by adding the letter a, b, or c. The horizontal or palatal component is classified as follows: a, unilateral alveolar maxillectomy; b, bilateral alveolar maxillectomy; and c, total alveolar maxillary resection. CONCLUSION: This practical classification attempts to relate the likely aesthetic and functional outcomes of a maxillectomy to the method of rehabilitation.     

骨盆部分切除术治疗骨盆肿瘤的部位分型与术式选择

探针对骨盆部肿瘤控其发生部位进行分型,选择相应的手术方式,以最大限度保留患侧肢体的功能。方法通过对１９８０年－１９９５年２０例骨盆腔肿瘤手术的总结,提出按肿瘤及部位即骶髂关节及髋关节是否被丰 坏将骨盆腔为四型     

Surgical management of acne keloidalis

Summary Acne keloidalis is an uncommon disease that predominantly affects post-pubertal males. The condition is primarily a chronic folliculitis complicated by hypertrophic scarring and multiple draining abscesses. Many patients present with advanced stages, despite prolonged non-operative treatments. Advanced stages of the disease can be easily treated with excisional cure followed by primary closure. Tissue expansion of the posterior scalp is a treatment option in very large lesions, yet simple primary closure offers an equally successful cosmetic and functional result, in lesions up to 11.5 cm in width.     

An evaluation of Bosniak's radiological classification of cystic renal masses

OBJECTIVE: To determine the clinical usefulness of Bosniak's classification of cystic renal masses, the differentiation of which remains difficult despite significant advances in diagnostic imaging. PATIENTS AND METHODS: The computed tomography (CT) findings of all histopathologically examined cystic renal masses diagnosed at our institution were analysed retrospectively; 35 patients with cystic renal masses were treated between 1986 and 1998. Tissues surgically removed were examined pathologically and the final diagnosis compared with the preoperative CT category of Bosniak's classification. RESULTS: The histopathological examined showed cystic renal cell carcinoma in 21 patients, a benign renal cyst in 12, haemangiosarcoma in one and transitional cell carcinoma in one. Most of the 35 masses (26, 74%) were found incidentally during evaluation for an unrelated disease or a routine health check. All 11 masses of Bosniak category I were benign and one category II mass was malignant. All 10 masses of category III and 12 of category IV were malignant. CONCLUSIONS: Bosniak's classification is useful for differentiating category I, III and IV cystic renal masses. There were too few samples to allow meaningful conclusions to be drawn for category II renal masses. It is critical to differentiate between complicated cysts of category II and III because of the major implications for prognosis and clinical management.     

The outcome and a new ISN/RPS 2003 classification of lupus nephritis in Japanese

BACKGROUND: A considerable diversity in prognosis is seen with lupus glomerulonephritis (LGN). Hence, the clinical usefulness of a recent International Society of Nephrology/Renal Pathology Society (ISN/RPS) 2003 classification to judge the long-term outcome of human LGN has been investigated. METHODS: We studied retrospectively 60 subjects with LGN (7 males, 53 females, mean age of 33 years old) who underwent renal biopsies and were followed from 1 to 366 months, with a mean of 187 months. We diagnosed renal pathology as classes, active and sclerosing lesions, according to the new and WHO1995 classification of LGN, and analyzed the clinicopathologic factors affecting to the prognosis of LGN. RESULTS: New classification got much higher consensus in the judgment of classes (98% vs. 83%, P = 0.0084). The group of Class IV-S (N = 6) or IV-G (N = 17) at initial biopsies showed higher rate of end-stage renal failure (ESRF) compared with that of Class I, II, III or V (40.9% vs. 2.6%, P < 0.001). The mean 50% renal survival time of Class IV was 189 +/- 29 months, and patients with Class IV-S tended to have a poorer prognosis (95 +/- 22 months for IV-S vs. 214 +/- 35 months for IV-G, P = 0.1495). Class IV was also selected as the most significant risk factor for ESRF by stepwise model (P = 0.002). In subanalysis for ESRF in Class IV (-S or -G), treatment including methylprednisolone pulse therapy was only selected as a significant improving factor for primary outcome (P = 0.034). In addition, activity index was the significant risk factor of death and/or ESRF after initial renal biopsies (P = 0.043). As for actuarial patient death during all follow-up periods, complications with anti-phospholipid syndrome or nephrotic syndrome were significant risk factors (P = 0.013, P = 0.041, respectively). CONCLUSION: New ISN/RPS 2003 classification provided beneficial pathologic information relevant to the long-term renal outcome and the optimal therapy preventing ESRF and/or death in patients with LGN.     

Seizure and memory outcome following temporal lobe surgery: selective compared with nonselective approaches for hippocampal sclerosis

OBJECT: The aim of this study was to compare seizure and memory outcome in patients with medically refractory mesial temporal lobe epilepsy due to hippocampal sclerosis (MTLE/HS) treated using an anterior temporal lobectomy (ATL) or a selective amygdalohippocampectomy (SA). METHODS: Surgical outcome data were prospectively collected for 2 to 11 years in 161 consecutive patients with MTLE/ HS. Eighty patients underwent an ATL and 81 an SA. Seizure control achieved with each technique was compared using the Engel classification scheme. Postoperative memory testing was performed in 86 patients (53%). At the last follow up, 72% of the patients who had undergone an ATL (mean follow up 6.7 years) and 71% of those who had undergone an SA (mean follow up 4.5 years) were seizure free (Engle Class IA). Estimated survival in patients in Engel Classes I, IA, and I and II combined did not differ between the two surgical techniques. Preoperatively, 58% of the patients had verbal memory scores one standard deviation (SD) below the normal mean. One third of the patients with preoperative scores in the normal range worsened after surgery, although this outcome was not related to the surgical technique. In contrast, one third of those whose preoperative scores were less than -1 SD experienced improvement after surgery. Nine (18%) of the 50 patients whose left side had been surgically treated improved their verbal memory scores by more than one SD. Seven (78%) of these nine underwent an SA (p = 0.05). CONCLUSIONS: Both ATL and SA can lead to similar favorable seizure control in patients with MTLE/HS. Preliminary data suggest that postoperative verbal memory scores may improve in patients who undergo selective resection of a sclerotic hippocampus in the dominant temporal lobe.     

Long-term seizure outcome following surgery for dysembryoplastic neuroepithelial tumor

OBJECT: Resection of dysembryoplastic neuroepithelial tumor (DNET) is thought to result in favorable seizure outcome, but long-term follow-up data are scarce. The authors present a review of 18 patients who underwent surgical removal of a DNET: 12 via temporal lobectomy and six via lesionectomy. METHODS: The mean long-term follow up was 10.8 years (median 10.4 years, range 7.8 to 14.8 years), and results obtained during this time period were compared with previously reported short-term (mean 2.7 years) seizure outcome data. In the current study, 66.7% patients had an Engel Class I outcome and 55.6% had an Engel Class IA outcome compared with 77.8% and 55.6%, respectively. Temporal lobectomy (Engel Class I, 83.3%; Engel Class IA, 66.7%) led to a better seizure outcome than lesionectomy (Engel Classes I and IA, 33.3%). Two patients (11.1%) required repeated operation and both had an incomplete lesionectomy initially. CONCLUSIONS: Results indicated that complete resection of a DNET leads to a favorable seizure outcome, with epilepsy cure in those who had experienced early postoperative seizure relief. Long-term seizure outcome after surgery is predictable based on the result of short-term follow up.     

The West China Hospital radiographic classification for fibrous dysplasia in femur and adjacent bones: A retrospective analysis of 205 patients

ObjectiveThis study aims to investigate the reliability and clinical outcome of a newly developed classification system for patients with fibrous dysplasia (FD) of the femur and adjacent bones, optimizing its evaluation and management.MethodsA total of 205 patients (121 female and 84 male) with FD in the femur and adjacent bones were included in this retrospective study. All affected femurs were measured and treated based on this classification at our institution between 2009 and 2019. Based on previous studies and extensive clinical follow‐up, we cautiously proposed the West China Hospital radiographic classification for FD in femur and adjacent bones following corresponding treatment options. There are five types with five radiographic features, including proximal femur bone loss, coxa vara, femoral shaft deformity, genu valgum, and hip arthritis. The intraobserver and interobserver reproducibility of this classification was assessed by four observers using the Cohen kappa statistic. The clinical outcome was evaluated using the criteria of Guille.ResultsAt a median follow‐up of 60 months (range 6–120), 205 patients (median 34.8 years old, range 18–73 years old) were categorized into the following five types: Type I 31.7%, Type II 30.2%, Type III 20.5%, Type IV 10.2%, and Type V 7.3%. The mean interobserver and intraobserver kappa scores were 0.85 (range 0.77–0.89) and 0.85 (range 0.79–0.92), respectively. For clinical outcomes, there was no significant difference in the postoperative Guille score for Type I patients (mean 9.01 ± 1.22). There was a significant increase in the postoperative Guille score in Type II, III, IV, and V, compared to the preoperative values (P < 0.01). For complications, two Type III patients reported pain, and one Type III patient had mild‐to‐moderate Trendelenburg gait. One Type IV patient had a mild Trendelenburg gait. And two Type V patients still had mild limping.ConclusionThis classification is reproducible and serves as a tool for evaluating and treating FD in the femur and adjacent bones. Therefore, we recommend this classification for the diagnosis and treatment of FD‐related deformities in the femur and adjacent bones.