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Harald Stein - One of the best experts on this subject based on the ideXlab platform.
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plasmacellular differentiation in extranodal marginal zone b cell lymphomas of the ocular Adnexa an analysis of the neoplastic plasma cell phenotype and its prognostic significance in 136 cases
British Journal of Ophthalmology, 2005Co-Authors: Sarah E. Coupland, Ioannis Anagnostopoulos, Martin Hellmich, Claudia Auwhaedrich, Harald SteinAbstract:Aim: To determine (a) the expression of plasma cell related antigens in extranodal marginal zone B cell lymphomas (EMZL) of the ocular Adnexa; and (b) the prognostic value of plasmacellular differentiation in these tumours. Methods: A consecutive case series of 136 ocular Adnexal EMZL obtained from three ocular pathology centres over 20 years was analysed retrospectively. An extensive immunohistochemical panel, including the plasma cell related antigens VS38c, CD38, CD138, multiple myeloma oncogene-1-protein (MUM1/IRF4), and CREB binding protein (CBP) was performed. EMZL were defined as “plasmacellular differentiated” on the basis of morphological features, evidence of cytoplasmic immunoglobulin, negativity for BSAP/PAX5, and expression of at least one of the investigated plasma cell related antigens. Controls included normal or hyperplastic lymphatic tissues. Detailed clinical data were collected for most patients, and compared with the results of immunohistochemistry. The end points considered for statistical analysis were development of local tumour recurrence, development of systemic disease, and lymphoma related death. Results: 57 (42%) of the 136 ocular Adnexal EMZL showed a plasmacellular differentiation; 45 of these plasmacytoid cases were primary tumours. In contrast with most admixed normal plasma cells, which displayed co-expression of MUM1/IRF4, Vs38c, CD38, CD138, and CBP, the plasmacellular differentiated EMZL tumour cells demonstrated co-expression of all five plasma cell related antigens in only six of 57 (11%) plasmacellular differentiated ocular Adnexal EMZL. The most commonly expressed plasma cell related antigen was MUM1/IRF4, immunoreactivity being seen in 56/57 (98%) plasmacellular differentiated EMZL examined. Although the association of plasmacellular differentiation in primary ocular Adnexal EMZL and disseminated disease was statistically significant on univariate analysis (p = 0.042), this was weaker on multivariate analysis. Conclusion: Plasmacellular differentiated tumour cells in EMZL demonstrate an aberrant immune profile for plasma cell related antigens when compared with normal plasma cells. On multivariate analysis, plasmacellular differentiation in ocular Adnexal EMZL was not significantly associated with local recurrence, the development of systemic disease, or with lymphoma related death.
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immunoglobulin vh gene expression among extranodal marginal zone b cell lymphomas of the ocular Adnexa
Investigative Ophthalmology & Visual Science, 1999Co-Authors: Sarah E. Coupland, Ioannis Anagnostopoulos, Hans Dieter Foss, Michael Hummel, Harald SteinAbstract:PURPOSE. Most lymphomas of the ocular Adnexa are primary extranodal non-Hodgkin's lymphomas of the B-cell type, with the most common lymphoma subtype being the extranodal marginal-zone B-cell lymphoma (EMZL). Analysis of somatic mutations in the variable (V) region of the Ig heavy (H)-chain gene segment suggests that EMZL development in other locations is dependent on antigen stimulation. The purpose of this study was to analyze the presence of somatic hypermutations in clonally rearranged Ig H-chain V genes of this lymphoma entity in the ocular Adnexa and to estimate whether the mutation pattern is compatible with antigen selection. METHODS. Twenty-six eases of EMZL of the ocular Adnexa were diagnosed on the basis of morphology, histology, and immunohistology. A nested polymerase chain reaction (PCR) was performed on DNA extracted from paraffin sections. The isolated PCR products were sequenced and compared with published V H germline segments to determine the number of somatic mutations in the complementarity-determining region (CDR) 2 and framework (FW) region 3. RESULTS. The number of somatic mutations in the cases of EMZL varied between 0 and 24: Five cases involved 0 to 3 somatic mutations, and the remaining 21 cases involved 4 to 24 mutations. Based on the ratio of replacement (R) to silent (S) mutations in the CDR2 or FW3 regions, antigen selection seems to have occurred in 60% of ocular Adnexal EMZL. The V H 3 family was the most commonly expressed germline V H family (54%), followed by V H 4 (23%), with biased usage of the latter. Some germline V H genes used included DP-8, DP-10, DP-53, DP-63 (V H 4.21), and DP-49, which are frequently used by autoantibodies (e.g., rheumatoid factors) and natural autoantibodies. CONCLUSIONS. EMZLs of the ocular Adnexa have an Ig H-chain mutation pattern that supports the concept that they represent a clonal expansion of post-germinal-center memory B-cells in most instances. In two thirds of cases, antigen selection may have occurred, and autoantibodies may have a role in their development.
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lymphoproliferative lesions of the ocular Adnexa analysis of 112 cases
Ophthalmology, 1998Co-Authors: Sarah E. Coupland, L Krause, Henrijacques Delecluse, Ioannis Anagnostopoulos, Hans Dieter Foss, Michael Hummel, Norbert Bornfeld, William R Lee, Harald SteinAbstract:Abstract Objective Lymphoproliferative lesions of the ocular Adnexa were analyzed to examine (1) the suitability of the Revised European-American Lymphoma (REAL) classification for the subtyping of the lymphomas in these sites; (2) the predictive value of the REAL classification for the evolution of these tumors; and (3) the frequency and prognostic impact of tumor type, location, proliferation rate (Ki-67 index), p53, and CD5 positivity and the presence of monoclonality within these tumors. Design Retrospective review. Methods The clinical, histomorphologic, immunohistochemical, and molecular biologic (polymerase chain reaction [PCR]) features of lymphoid proliferations of the ocular Adnexa were studied. Study materials The ocular Adnexal lymphoproliferative lesions were located as follows: orbit in 52 patients (46%), conjunctiva in 32 patients (29%), eyelid in 23 patients (21%), and caruncle in 5 patients (4%). Results Reactive lymphoid hyperplasia was diagnosed in 12 cases and lymphoma in 99 cases; 1 case remained indeterminate. The five main subtypes of lymphoma according to the REAL classification were extranodal marginal-zone B-cell lymphoma (64%), follicle center lymphoma (10%), diffuse large cell B-cell lymphoma (9%), plasmacytoma (6%), and lymphoplasmocytic lymphoma (5%). Age, gender, and anatomic localization of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years (mean, 3.3 years). Extent of disease at time of presentation was the most important clinical prognostic factor: advanced disease correlated with increased risk ratios of having persistent disease at the final follow-up and with lymphoma-related death ( P P P P P Conclusion The REAL classification is suitable for the subdivision of the ocular Adnexal lymphomas. The MIB-1 proliferation rate and p53 positivity may aid the prediction of disease stage and disease progression, whereas PCR can support the diagnosis and reduce the number of histologically indeterminate lesions.
Steven H Swerdlow - One of the best experts on this subject based on the ideXlab platform.
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use of similar immunoglobulin vh gene segments by malt lymphomas of the ocular Adnexa
Modern Pathology, 2009Co-Authors: David W Bahler, Philippe Szankasi, Sucheta Kulkarni, Raymond R Tubbs, James R Cook, Steven H SwerdlowAbstract:Extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue type (MALT lymphomas) develop from acquired reactive infiltrates directed against external or autoantigens. Although some European cases of ocular Adnexal MALT lymphoma have been associated with Chlamydia psittaci infections, C. psittaci has not been detected in large studies of US-based cases. To evaluate whether the growth of US-based ocular Adnexal MALT lymphomas may be promoted by a similar antigen, we identified and analyzed the expressed immunoglobulin VH genes in 10 cases. Interestingly, the VH genes in two cases used the same VH1 family V1-2 gene segment, and three cases used the same VH4 family V4-34 gene segment. The other five cases all used different gene segments V4-31, V5-51, V3-23, V3-30, and V3-7. All of the VH genes were mutated from germ line, with percent homologies ranging between 96.9 and 89.0%. The distribution of replacement and silent mutations within the VH genes was nonrandom consistent with the maintenance of immunoglobulin function and also strongly suggestive of antigen selection in the six VH genes with highest mutation loads. The CDR3 sequences in two of three VH-34 cases were the same size (15 amino acids) and had similar sizes in the two VH1-2 cases (18 and 16 amino acids). In conclusion, US-based MALT lymphomas of the ocular Adnexa preferentially express a limited set of VH gene segments not frequently used by other MALT lymphomas and consistent with some recognizing similar antigens. Analysis of somatic mutations present within the VH genes is also consistent with antigen binding stimulating the growth of these lymphomas.
Gabriel Oelsner - One of the best experts on this subject based on the ideXlab platform.
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Minimal surgery for the twisted ischaemic Adnexa can preserve ovarian function
Human Reproduction, 2003Co-Authors: Gabriel Oelsner, Shlomo B. Cohen, David Soriano, Dahlia Admon, Shlomo Mashiach, Howard CarpAbstract:BACKGROUND: Recently detorsion has replaced salpingo-oophorectomy as treatment for the twisted ischaemic Adnexa. This paper asssess whether the ovary resumes normal function after preservation by detorsion. METHODS: The results of detorsion performed between January 1988 and December 2001 were retrospectively analysed. Post-operative complications and subsequent ovarian function were assessed including: ultrasound monitoring of follicular development, Adnexal appearance during subsequent surgery, and the outcome of IVF. RESULTS: A total of 102 detorsions were performed; 67 by laparoscopy, 35 by laparotomy. No patient developed thromboembolism. Post-operative fever occurred in 15% of patients after laparoscopy and 29% after laparotomy (P < 0.01). Patients were hospitalized for a mean (6 SD) of 2.1 6 1.2 and 7.4 6 1.5 days after laparoscopy and laparotomy respectively (P < 0.001). Ultrasound showed normal follicular development in 93 and 91% of patients after detorsion by laparoscopy and laparotomy respectively. At subsequent surgery, the Adnexa appeared normal in nine out of nine patients after laparoscopy and in four out of five patients after laparotomy. Four patients of the laparoscopy group and two patients of the laparotomy group underwent subsequent IVF. In all six patients oocytes retrieved from the previously ischaemic ovary were fertilized. CONCLUSIONS: Detorsion with Adnexal sparing is the treatment of choice for twisted ischaemic Adnexa, and preferably performed by laparoscopy.
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laparoscopy versus laparotomy for detorsion and sparing of twisted ischemic Adnexa
Jsls-journal of The Society of Laparoendoscopic Surgeons, 2003Co-Authors: Shlomo B. Cohen, Dahlia Admon, Shlomo Mashiach, Mordechai Goldenberg, Daniel S. Seidman, Arnaud Wattiez, Gabriel OelsnerAbstract:BACKGROUND Torsion of the ovary is an urgent event for fertile women. Until recent years, the common treatment for twisted ischemic ovaries was salpingo-oophorectomy. We have demonstrated in the past that the ovary can be salvaged provided detorsion is performed. We studied the outcome of women undergoing minimal surgery for ischemic ovaries versus the extended procedure including cystectomy in respect of ovarian function and fertility performance. METHODS We retrospectively studied 102 women who underwent surgery for torsion of the ovary in which the macroscopic appearance of black-bluish ischemic Adnexa was encountered during surgery. Detorsion without removal of the Adnexa or the ovary was performed by laparoscopy in 67 patients and by laparotomy in 35 patients. Patients' files were reviewed for immediate and late outcomes. Patients were examined postoperatively with vaginal ultrasound for ovarian follicular function. Data concerning patients' further surgeries or in vitro fertilization were retrieved from the charts as well. RESULTS Febrile morbidity was approximately 15% and 29% in the laparoscopy and laparotomy groups, respectively. Hospital stay was 2.1 +/- 1.2 and 7.4 +/- 1.5 days in the laparoscopy and laparotomy groups, respectively (P<0.001). Ultrasound follow-up was available in 60 of 67 patients who underwent laparoscopy and in 32 of 35 patients treated by laparotomy. Normal-sized ovaries with follicular development were encountered in the detorsed side in 93% and 91%, respectively. Normal macroscopic appearance of the Adnexa at subsequent surgeries was reported in 9 of 9 patients in the laparoscopy group and in 4 of 5 patients in the laparotomy group. Four patients from the laparoscopy group and 2 from the laparotomy group underwent subsequent in vitro fertilization. In all 6 patients, oocytes retrieved from the previously detorsed ovary were fertilized. In both groups, none of the patients developed clinical signs of pelvic or systemic thromboembolism. CONCLUSIONS Torsion of the ischemic Adnexa should be treated laparoscopically by detorsion and Adnexal sparing.
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Laparoscopic detorsion allows sparing of the twisted ischemic Adnexa.
Journal of The American Association of Gynecologic Laparoscopists, 1999Co-Authors: Shlomo B. Cohen, Gabriel Oelsner, Dahlia Admon, Shlomo Mashiach, Daniel S. Seidman, Mordechai GoldenbergAbstract:Abstract Study Objective. To determine the safety and outcome of laparoscopic detorsion in the management of the twisted ischemic, hemorrhagic Adnexa. Design. Retrospective chart review and prospective follow-up (Canadian Task Force classification II-2). Setting. University-affiliated hospital. Patients. Fifty-eight women with twisted black-bluish ischemic Adnexa encountered at laparoscopy. Intervention. Laparoscopic detorsion with Adnexal sparing. Measurements and Main Results. All patients had a benign immediate postoperative course. Transient temperature elevation occurred in seven women (12.1%). No signs of pelvic or systemic thromboembolism were detected in any patient. Long-term follow-up included transvaginal ultrasound, which revealed follicular development in the previously twisted Adnexa in 54 women; normal macroscopic appearance at incidental subsequent surgery in 9; and in vitro fertilization with retrieval of oocytes from the previously twisted side in 4. Conclusion. Laparoscopic detorsion of the twisted ischemic, hemorrhagic Adnexa is a safe procedure with minimal postoperative morbidity and a potential for the ovary to recuperate fully with preservation of normal function. Laparoscopic Adnexa-sparing procedures should be performed in place of traditional salpingo-oophorectomy in women with this disorder who desire future fertility.
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Long-term follow-up of the twisted ischemic Adnexa managed by detorsion.
Fertility and Sterility, 1993Co-Authors: Gabriel Oelsner, Dahlia Admon, David Bider, Mordechai Goldenberg, Shlomo MashiachAbstract:Objective To examine our assumption that although the twisted Adnexa appears ischemic-hemorrhagic, it can safely be revived by detorsion with preservation of ovarian function. Design Patients' records were obtained from a computerized database and reviewed. The preoperative diagnosis of Adnexal torsion was based upon patients' symptoms, clinical examination, laboratory investigations, and ultrasound scanning. Setting Gynecology department at a large teaching hospital receiving primary referrals of public sector patients. Patients In 40 patients who presented with signs and symptoms suggestive of ovarian torsion, "black-bluish" ischemic Adnexa were encountered at surgery. Interventions All patients were managed by unwinding of the Adnexa: laparotomy in 26 cases and operative laparoscopy in 14. In 13 patients detorsion only was performed, in 15 detorsion and cystectomy were carried out, and in 12 patients detorsion was done and ovarian cysts aspirated. Main Outcome Measures Postoperative course, mean hospitalization period, follow-up pelvic examination, ovarian folliculogenesis on ultrasound examination, and oocyte retrieval and fertilization. Results The size of the twisted ovary ranged from 4 to 20cm (mean, 9.5cm). The postoperative course was uneventful, except for transient temperature elevation in five patients. The mean hospitalization period was 6.5days (range 5 to 10days) after laparotomy and 1.8days (range 1 to 3days) after laparoscopy. Three patients were lost to follow-up. In 37 patients, pelvic examination was normal. A normal sized ovary, with follicular development, was demonstrated sonographically in 35 of 37 patients. In 6 of 7 patients, macroscopically normal Adnexa were visualized at subsequent laparotomy or laparoscopy. In two patients undergoing IVF, oocytes were retrieved and fertilized from the detorted ovary. The patency of the fallopian tube was demonstrated in four cases. Conclusions This new "Adnexal-sparing" approach should be applied instead of the traditional salpingo-oophorectomy in young women with twisted ischemic Adnexa.
Moty Pansky - One of the best experts on this subject based on the ideXlab platform.
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High risk of recurrent torsion in premenarchal girls with torsion of normal Adnexa
Fertility and Sterility, 2016Co-Authors: Noam Smorgick, Yaakov Melcer, Tal Sarig-meth, Ron Maymon, Zvi Vaknin, Moty PanskyAbstract:Objective To compare Adnexal torsion characteristics and torsion recurrence rates in a pre- and postmenarchal pediatric and adolescent population. Design Retrospective cohort study. Setting University-affiliated medical center. Patient(s) Females Intervention(s) Adnexal detorsion, cystectomy, salpingectomy, or salpingo-oophorectomy by laparoscopy or laparotomy. Oophoropexy using the utero-ovarian ligament plication technique was performed in cases of recurrent torsion of normal Adnexa. Main Outcome Measure(s) The clinical presentation, laboratory and ultrasound characteristics, surgical findings, surgical procedures, pathologic diagnosis, and torsion recurrence rates were analyzed and compared between pre- and postmenarchal patients. Result(s) Twenty premenarchal and 24 postmenarchal patients were identified. The clinical presentation, laboratory findings, and ultrasound characteristics were similar between the two groups, except for a higher prevalence of paraovarian cysts on preoperative ultrasound in the postmenarchal compared with in the premenarchal group (20.8% vs. 0%). For the whole cohort, torsion of normal Adnexa constituted 25.0% (11/44) of cases, while torsion of "pathologic" Adnexa constituted 75.0% (33/44) of torsion cases. Torsion recurrence was significantly more common among girls whose first torsion episode occurred in the premenarchal period compared with in postmenarche (35% vs. 4.2%). On multivariate logistic regression analysis, torsion recurrence was significantly associated with premenarchal status (odds ratio [OR] = 12.7; 95% confidence interval [CI], 1.1–152.0) and with torsion involving normal Adnexa (OR = 19.1; 95% CI, 2.3–154.5). Conclusion(s) Recurrent torsion is common in patients whose first torsion episode occurred in the premenarchal period and involved otherwise normal Adnexa. Ovarian fixation procedures may be considered in patients at risk for torsion recurrence.
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torsion of normal Adnexa in postmenarcheal women can ultrasound indicate an ischemic process
Ultrasound in Obstetrics & Gynecology, 2008Co-Authors: Noam Smorgick, Arie Herman, Ron Maymon, Sonia Mendelovic, Moty PanskyAbstract:Objective Torsion of normal Adnexa is a rare event involving steadily increasing congestion and ischemia of the ovary. We investigated whether this process can be characterized by sonographic features. Methods Twelve menstrually cycling women with 14 separate episodes of laparoscopic-proven torsion of normal Adnexa were identified retrospectively, and the results of their preoperative gray-scale ultrasound examinations and Doppler flow evaluations were analyzed. The cases were classified into ‘short-term’ ( 24 h; range, 1–10 days) duration of torsion according to the reported period of abdominal pain before admission. Absence of any additional Adnexal pathology was confirmed by both intraoperative inspection and postoperative follow-up ultrasound examinations. Results The median age of the cohort was 24.0 (interquartile range (IQR), 20.5–28.7) years, and parity ranged from 0 to 3. All affected ovaries were significantly enlarged compared with non-affected ones (median cross-sectional area, 18.1 (IQR, 12.4–26.4) cm2 vs. 4.3 (IQR, 2.9–6.2) cm2, P < 0.01). We could distinguish two distinct sonographic patterns of torted ovaries: there were numerous small peripheral follicles in the ovarian parenchyma in nine cases, and there was a solid-appearing mass with hypo- and hyperechogenic foci in five cases. Comparison of the ultrasound images of patients with short-term vs. long-term abdominal pain revealed that the solid-appearing ovary was more common in the latter group (0/6 vs. 5/8, P = 0.03), while there was no significant difference between groups in the presence of free pelvic fluid or median ovarian cross-sectional area. Intraovarian blood flow was diminished or absent in five of the eight patients in whom color Doppler imaging was performed. Conclusions Ultrasound images of twisted normal Adnexa may vary according to the duration of the condition, reflecting the pathological series of events of increased ovarian congestion and necrosis. Recognition of the different sonographic features of twisted normal Adnexa may assist in the correct diagnosis of these patients. Copyright © 2008 ISUOG. Published by John Wiley & Sons, Ltd.
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torsion of normal Adnexa in postmenarchal women and risk of recurrence
Obstetrics & Gynecology, 2007Co-Authors: Moty Pansky, Noam Smorgick, Arie Herman, David Schneider, Reuvit HalperinAbstract:OBJECTIVE To compare the incidence of recurrent torsion of normal Adnexa to recurrent torsion of abnormal Adnexa in postmenarchal women. METHODS All cases of surgically confirmed Adnexal torsion operated on in our department from January 2002 to April 2006 were retrospectively analyzed. Classification as torsion of pathologic Adnexa versus torsion of normal Adnexa was based on pre- and postoperative pelvic sonograms, operative findings, and pathologic diagnoses (when available). The torsion recurrence rates were evaluated by a telephone questionnaire, with 90.5% compliance. RESULTS Twelve women had twisted normal Adnexa and 50 women had twisted abnormal Adnexa. The women with twisted normal Adnexa were younger (25.5+/-7.9 years versus 31.4+/-11.4 years, P=.10) and had a lower median parity (0 versus 1, P=.06). Both groups had similar clinical presentations and a similar mean time from admission to surgery. The torsion recurrence rates were 63.6% (95% confidence interval [CI] 0.3-0.9) in the twisted normal Adnexa group and 8.7% (95% CI 0.02-0.2) in the twisted abnormal Adnexa group (P<.001). The retorsion risk of the pathologic Adnexa was especially low after cystectomy (5.3%, 95% CI 0.001-0.3) or salpingo-oophorectomy (0%). The Kaplan-Meier life table analysis also showed a higher retorsion risk for normal Adnexa (P=.008). CONCLUSION The current Adnexa-sparing laparoscopic management of Adnexal torsion by simply untwisting may predispose to recurrent torsion of normal Adnexa. The role of ovariopexy procedures in the prevention of recurrent torsion events remains uncertain. LEVEL OF EVIDENCE III.
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Torsion of Normal Adnexa in Postmenarchal Women and Risk of Recurrence
Obstetrics & Gynecology, 2007Co-Authors: Moty Pansky, Noam Smorgick, Arie Herman, David Schneider, Reuvit HalperinAbstract:OBJECTIVE To compare the incidence of recurrent torsion of normal Adnexa to recurrent torsion of abnormal Adnexa in postmenarchal women. METHODS All cases of surgically confirmed Adnexal torsion operated on in our department from January 2002 to April 2006 were retrospectively analyzed. Classification as torsion of pathologic Adnexa versus torsion of normal Adnexa was based on pre- and postoperative pelvic sonograms, operative findings, and pathologic diagnoses (when available). The torsion recurrence rates were evaluated by a telephone questionnaire, with 90.5% compliance. RESULTS Twelve women had twisted normal Adnexa and 50 women had twisted abnormal Adnexa. The women with twisted normal Adnexa were younger (25.5+/-7.9 years versus 31.4+/-11.4 years, P=.10) and had a lower median parity (0 versus 1, P=.06). Both groups had similar clinical presentations and a similar mean time from admission to surgery. The torsion recurrence rates were 63.6% (95% confidence interval [CI] 0.3-0.9) in the twisted normal Adnexa group and 8.7% (95% CI 0.02-0.2) in the twisted abnormal Adnexa group (P
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Torsion of normal Adnexa in postmenarchal women and risk of recurrence.
Obstetrics and gynecology, 2007Co-Authors: Moty Pansky, Noam Smorgick, Arie Herman, David Schneider, Reuvit HalperinAbstract:To compare the incidence of recurrent torsion of normal Adnexa to recurrent torsion of abnormal Adnexa in postmenarchal women. All cases of surgically confirmed Adnexal torsion operated on in our department from January 2002 to April 2006 were retrospectively analyzed. Classification as torsion of pathologic Adnexa versus torsion of normal Adnexa was based on pre- and postoperative pelvic sonograms, operative findings, and pathologic diagnoses (when available). The torsion recurrence rates were evaluated by a telephone questionnaire, with 90.5% compliance. Twelve women had twisted normal Adnexa and 50 women had twisted abnormal Adnexa. The women with twisted normal Adnexa were younger (25.5+/-7.9 years versus 31.4+/-11.4 years, P=.10) and had a lower median parity (0 versus 1, P=.06). Both groups had similar clinical presentations and a similar mean time from admission to surgery. The torsion recurrence rates were 63.6% (95% confidence interval [CI] 0.3-0.9) in the twisted normal Adnexa group and 8.7% (95% CI 0.02-0.2) in the twisted abnormal Adnexa group (P<.001). The retorsion risk of the pathologic Adnexa was especially low after cystectomy (5.3%, 95% CI 0.001-0.3) or salpingo-oophorectomy (0%). The Kaplan-Meier life table analysis also showed a higher retorsion risk for normal Adnexa (P=.008). The current Adnexa-sparing laparoscopic management of Adnexal torsion by simply untwisting may predispose to recurrent torsion of normal Adnexa. The role of ovariopexy procedures in the prevention of recurrent torsion events remains uncertain. III.
Dedee F Murrell - One of the best experts on this subject based on the ideXlab platform.
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granulation tissue in the eyelid margin and conjunctiva in junctional epidermolysis bullosa with features of laryngo onycho cutaneous syndrome
Clinical and Experimental Ophthalmology, 2007Co-Authors: Edwi C Figueira, Anne Crotty, Christophe J Challino, Minas T Coroneo, Dedee F MurrellAbstract:Eye and Adnexal involvement in epidermolysis bullosa can range from symptoms of mild irritation resulting from conjunctival involvement to severe cicatrization of the ocular surface and Adnexa. We describe a unique case of granulation tissue in the eyelid margin and conjunctiva in a patient with junctional epidermolysis bullosa. The eyelid granulation tissue resembled granulomas that seen in laryngo-onycho-cutaneous syndrome, which is caused by a mutation in an isoform of the LAMA3 gene, LAMA3a. On investigation, our patient had a combination of a unique mutation in LAMA3 and the mutation I17N in LAMA3a, providing further evidence that laryngo-onycho-cutaneous syndrome is a variant of junctional EB.