Allergic contact dermatitis from topical ophthalmic medications: keep an eye on it! Liesbeth Gilissen Lana Dedecker Toon Hulshagen An Goossens First published: 10 January 2019 https://doi.org/10.1111/cod.13209 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1111/cod.13209. PDFTOOLS SHARE Abstract Background Allergic contact dermatitis (ACD) from topical ophthalmic medications is often overlooked.
Objectives To study the demographic characteristics, lesion locations, and associated medical conditions of the patients with ACD from ophthalmic drugs, and to identify the most common allergenic culprits, as well as trends in frequencies over the years.
Methods From January 1990 until December 2016, 16 065 patients were investigated in our clinic; all patients with a positive patch‐test reaction to eye medication or its ingredient(s) having caused ACD were studied. For each allergen identified, the number of positive test results compared with the total number of those in the total population, as well as trends across three periods, namely 1990‐1998, 1999‐2007, and 2008‐2016 were studied.
Results 118 patients (0.7%) presented with positive patch‐test results to ingredients of, and/or topical ophthalmic medications. Aminoglycoside antibiotics, followed by corticosteroids, as pharmacologically active ingredients, as well as wool alcohols, thiomersal and benzalkonium chloride, as excipients were the most frequent culprits. Particularly chloramphenicol showed a decreasing trend in positive reactions over time, whereas reactions tobramycin were increasing.
Conclusion ACD from eye medication is mainly due to active principles, but other excipient ingredients, beside the products "as is", should be tested as well. https://onlinelibrary.wiley.com/doi/10.1111/cod.13209
Diagnosing lanolin contact allergy with lanolin alcohol and Amerchol L101 Jannet Knijp Derk P. Bruynzeel Thomas Rustemeyer First published: 09 January 2019 https://doi.org/10.1111/cod.13210 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1111/cod.13210. PDFTOOLS SHARE Summary Background The prevalence of lanolin contact allergy in dermatitis patients varies from 1.2% to 6.9%. Different lanolin derivatives are used in patch testing.
Objectives To determine which combination of lanolin derivatives is most effective in patch testing for diagnosing lanolin contact allergy.
Methods A retrospective analysis of patients patch tested between 2016 and 2017 was performed. Patients were eligible if tested with lanolin alcohol 30% pet., Amerchol L101 50% pet. and a supplementary series containing other lanolin derivatives. Lanolin alcohol and Amerchol L101 were tested in duplicate.
Results Out of 594 patients, 28.6% (95% confidence interval [CI]: 25.1%‐32.3%) had a positive patch test reaction to at least one lanolin derivative. Reactions were common to lanolin alcohol (14.7%, 95% CI: 11.3%‐18.2%) and Amerchol L101 (15.0%, 95% CI: 11.5%‐18.5%) in the routinely tested series. Reactions to other test preparations were significantly less frequent (P < 0.05). The addition of Amerchol L101 to lanolin alcohol significantly increased the number of positive cases (odds ratio 1.79, P < 0.001).
Conclusions The combination of lanolin alcohol and Amerchol L101 is effective in patch testing for diagnosing lanolin contact allergy. Routinely testing with other lanolin derivatives may not be worthwhile as it detects only few additional patients. https://onlinelibrary.wiley.com/doi/10.1111/cod.13210
Impaired antimicrobial response and mucosal protection induced by ibuprofen in the immature human intestine Emanuela Ferretti, Eric Tremblay, Marie-Pierre Thibault, Sepideh Fallah, David Grynspan, Karolina M. Burghardt, Marcos Bettolli, Corentin Babakissa, Emile Levy & Jean-François Beaulieu Pediatric Researchvolume 84, pages813–820 (2018) | Download Citation
Abstract Background The use of nonsteroidal anti-inflammatory drugs (NSAIDs) such as indomethacin (INDO) and ibuprofen (IBU) has been shown to be an effective therapy for the closure of patent ductus arteriosus (PDA). However, this treatment has been associated with an increased risk of developing enteropathies in neonates. Whether the use of IBU is safer than INDO for the immature intestine remains to be elucidated.
Methods The direct impact of IBU on the human immature intestinal transcriptome was investigated using serum-free organ culture. Differentially expressed genes were analyzed with Ingenuity Pathway Analysis software and compared with those previously reported with INDO. Validation of differentially expressed genes was confirmed by qPCR.
Results We identified several biological processes that were significantly modulated by IBU at similar levels to what had previously been observed with INDO, while the expression of genes involved in "antimicrobial response" and "mucus production" was significantly decreased exclusively by IBU in the immature intestine.
Conclusions Our findings indicate that IBU has a harmful influence on the immature intestine. In addition to exerting many of the INDO observed deleterious effects, IBU alters pathways regulating microbial colonization and intestinal epithelial defense. https://www.nature.com/articles/s41390-018-0201-y
Sodium laurylsulphate (SLS) induced contact dermatitis is a commonly used model for testing effects of different topical formulations. Volar forearms are preferred testing site by the guidelines, but other anatomical locations were used in previous research, especially upper back, as the clinically used site for testing different antigens.
Objectives
Aim of the present study was to investigate existence of anatomical variations of skin response to irritation and its' effects on response to treatment.
Methods
Irritation was induced with SLS on symmetrical sites on both forearms and sides of upper back with additional sites exposed to water as controls. Half of the sites were treated with emollient cream while the other half were left untreated. Irritation was assessed using bioengineering methods and clinical scoring.
Results
Upper back skin showed higher reactivity to irritants with stronger barrier disruption (measured by Tewameter, 80·2±18·3 vs 48·0±24·2 gm−2h−1), more pronounced erythema (measured by Mexameter, 186·5±88·4 vs 92·1±58·2 AU) and dryness (measured by Corneometer, ‐28·6±14·5 vs 2·7±16·9 AU). Skin recovery rates were also influenced by anatomical location with the upper back showing faster recovery (316·7±223·1 vs 156·2±198·5). Treatment didn't lead to improvement in measured parameters, regardless of anatomical location.
Conclusion
Skins' reaction to irritant and recovery were dependant on anatomical location. Location where testing was conducted should always be reported as treatments tested across different locations could not be directly compared to each other.
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Antimelanogenic peptides are potentially useful to treat hyperpigmentation, but many peptides have limited application because of high cost and/or low activity.
Objectives
To identify small and potent peptide inhibitors of cellular melanin synthesis useful for cosmetic and medical applications.
Methods
A positional scanning synthetic tetrapeptide combinatorial library was used for screening of potentially active peptides. Antimelanogenic activities of the peptide pools and individual peptides were evaluated in B16‐F10 melanoma cells and human epidermal melanocytes (HEMs) treated with alpha‐melanocyte‐stimulating hormone (α‐MSH).
Results
The predicted active tetrapeptide sequences were R‐(F/L)‐(C/W)‐(G/R)‐NH2. Of the individual tetrapeptides tested, D3 (RFWG‐NH2) and D5 (RLWG‐NH2) exhibited high antimelanogenic activities. Tetrapeptide D9 (FRWG‐NH2) with a sequence identical to that of a portion of α‐MSH also showed antimelanogenic activity. Of the tripeptides tested, E5 (FWG‐NH2), E6 (LWG‐NH2), and E7 (RWG‐NH2) were relatively more active. Dipeptide F1 (WG‐NH2) and monopeptide G1 (G‐NH2, glycinamide) retained activity, but G2 (Ac‐G‐NH2) and G3 (glycine) did not. The antimelanogenic activities of peptides D3, E5, F1, and G1 were verified in α‐MSH‐stimulated HEMs. Commercially available G‐NH2·HCl suppressed the phosphorylation levels of cAMP‐responsive element binding protein, protein levels of microphthalmia‐associated transcription factor and tyrosinase, L‐tyrosine hydroxylase activity of tyrosinase, and the melanin levels in stimulated cells.
Conclusions
Small peptides, including glycinamide and tryptophanyl glycinamide, are potent antimelanogenic agents with potential value for the treatment of skin hyperpigmentation.
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Cutaneous lupus erythematosus (CLE) is a potentially disfiguring, chronic autoimmune disease with extremely variable skin manifestations, negatively affecting quality of life (QoL) of patients. Patient‐reported outcome (PRO) measures used in assessing QoL in CLE patients have been either generic or developed without input from patients with CLE.
Objectives
To demonstrate the reliability and validity of a disease‐specific QoL measure for CLE – the cutaneous lupus erythematosus quality of life (CLEQoL).
Methods
A total of 101 patients with a clinical diagnosis of CLE were recruited, and each patient was asked to complete the CLEQoL. Internal consistency was used as a measure of reliability. Validity was measured in two ways – structural validity via exploratory factor analysis and convergent validity via Spearman correlations between CLEQoL and the Short Form 36 (SF‐36). Patient demographic and disease characteristics were collected. Data was analyzed using SPSS and significance was set to p<0·05.
Results
The average age of our CLE patients was 48±13 with discoid lupus (n=72, 71·3%) being the most predominant CLE subtype. Patients were mostly female (n=88, 87·1%) and African‐American/Black (n=59, 58·4%). Internal consistency ranged from 0·67 to 0·97. A total of five domains, functioning, emotions, symptoms, body image/cosmetic effects and photosensitivity, were extracted with a total explained variance of 71·06%. CLEQoL‐related domains correlated with SF‐36 domains (r ranging from ‐0·39 to ‐0·65).
Conclusion
The CLEQoL was found to be a valid and reliable PRO measure for assessing QoL in patients with CLE. Demonstrating that the CLEQoL has strong psychometric properties is an important step towards the development of a disease‐specific PRO measure that future clinical trials can use.
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Clinicopathologic traits and prognostic factors associated with pediatric sinonasal rhabdomyosarcoma Sana H. Siddiqui BA Emaad Siddiqui BS Rich D. Bavier BA Nirali M. Patel BA Suat Kiliç MD Soly Baredes MD, FACS Wayne D. Hsueh MD Jean Anderson Eloy MD, FACS, FARS First published: 10 January 2019 https://doi.org/10.1002/alr.22267 Potential conflict of interest: None provided. Presented orally at the 64th Annual Meeting of the American Rhinologic Society, Atlanta, GA, October 5‐6, 2018. Read the full text PDFTOOLS SHARE Abstract Background Pediatric sinonasal rhabdomyosarcoma (RMS) is an aggressive and rare malignancy. This is the first multi‐institutional study on the prognostic factors associated with outcomes in this population.
Methods The National Cancer Database was queried for the period from 2004 to 2013 for all cases of malignant sinonasal RMS in the pediatric population. The impact of patients' demographics, tumor characteristics, and Intergroup Rhabdomyosarcoma Study Group (IRSG) staging on survival was assessed using chi‐square test, Fisher's exact test, Kaplan‐Meier test, and Cox regression analyses.
Results A total of 157 cases of pediatric sinonasal RMS were identified. Mean age at diagnosis was 9.38 years and male patients comprised 48.4% of the cohort. The nasal cavity (31.8%) and maxillary sinus (30.6%) were the most common primary sites. Alveolar was the most common histology (49.7%), followed by embryonal type (32.5%). The majority of patients received solely chemoradiation (52.9%), followed by surgery with adjuvant chemoradiation (30.6%). Five‐year overall survival (OS) was 55.2% (±4.5%). Metastatic disease was associated with a poorer 5‐year OS rate (24.4% vs 61.5%; p = 0.010). Maxillary sinus site was associated with an improved survival (71.8% vs 47.6%; p = 0.009). On multivariate analysis, chemoradiation with or without surgery was an additional prognostic factor. Although IRSG clinical stages did not correlate with survival, high‐risk patients in the IRSG clinical risk groups were associated with poorer survival on multivariate analysis (hazard ratio [HR], 2.005; 95% confidence interval, 1.007‐3.993; p = 0.048).
Conclusion To date, this is the largest study on pediatric sinonasal RMS. IRSG clinical risk groups may be useful in stratifying high‐risk patients with poor prognosis. https://onlinelibrary.wiley.com/doi/10.1002/alr.22267
Formation of papillary mucosa folds and enhancement of epithelial barrier in odontogenic sinusitis Yuan Zhang MD, PhD Feng Lan MD, PhD Ying Li BS Chengshuo Wang MD, PhD Luo Zhang MD, PhD First published: 08 January 2019 https://doi.org/10.1002/alr.22277 Funding sources for the study: National Key R&D Program of China (2016YFC20160905200); the National Natural Science Foundation of China (81570895, 81420108009, 81400444, 81470678, and 81630023); Changjiang Scholars and Innovative Research Team (IRT13082); Special Fund of Capital Health Development (2011‐1017‐06, 2011‐1017‐02); Special Fund of Sanitation Elite Reconstruction of Beijing (2009‐2‐007); Beijing Health Bureau Program for High Level Talents (2011‐3‐043); Beijing Municipal Administration of Hospitals' Mission Plan (SML20150203); Capital Citizenry Health Program (z161100000116062). Potential conflict of interest: None provided. Read the full text PDFTOOLS SHARE Abstract Background Odontogenic sinusitis (OS) presents more satisfactory therapeutic effect after endoscopic surgery compared with chronic rhinosinusitis (CRS) of other origin. The aim of the present study was to investigate the clinical characteristics, morphological features, and epithelial barrier function of sinus mucosa of OS and discuss the possible relationship with good prognosis.
Methods A total of 25 subjects with OS, 7 CRS without nasal polyps (CRSsNP), 10 CRS with nasal polyps (CRSwNP), and 9 control subjects were recruited. The biopsy specimens were stained with hematoxylin and eosin for general observation of cytomorphologic features. Epithelial tight junctions (TJs) protein claudin‐4 expression was determined to evaluate the epithelial barrier integrity by using immunofluorescence and Image‐Pro Plus software analysis. The representative cytokine profiles regarding T helper 1 (Th1) (interferon [IFN]‐γ), Th2 (interleukin [IL]‐5), and Th17 (IL‐17) were examined by reverse transcription–polymerase chain reaction (RT‐PCR).
Results Extensively small papillary protrusions could be seen in the maxillary sinus mucosa of OS patients under nasal endoscopy, similar to the morphological behavior, which also presented as papillary folds in the surface of the epithelium. The epithelium in OS kept an increased claudin‐4 expression compared with that seen in CRSsNP, CRSwNP, and control subjects. The inflammatory pattern analysis demonstrated that OS belonged to the lymphocyte and plasma cell‐dominant cellular phenotypes, whereas IL‐17 was dominant compared with IFN‐γ as well as IL‐5.
Conclusion The odontogenic infections might induce the formation of papillary mucosa folds and enhance the epithelial TJ barrier function. OS exhibited as lymphocyte and plasma cell–dominant cellular phenotypes and Th17 cytokine profiles. https://onlinelibrary.wiley.com/doi/10.1002/alr.22277
Broncho‐Vaxom® (OM‐85 BV) soluble components stimulate sinonasal innate immunity Vasiliki Triantafillou BS Alan D. Workman MD Neil N. Patel BA, BS Ivy W. Maina BA Charles C. L. Tong MD Edward C. Kuan MD, MBA David W. Kennedy MD … See all authors First published: 07 January 2019 https://doi.org/10.1002/alr.22276 Funding sources for the study: National Institutes of Health (National Institute on Deafness and Other Communication Disorders [NIDCD] R01DC013588 to N.A.C.); Veterans Affairs Merit Review (CX001617 to N.A.C.). Potential conflict of interest: None provided. Presented orally at the ARS Meeting at the annual Combined Otolaryngology Spring Meetings (COSM) on April 18‐22, 2018, National Harbor, MD. Read the full text PDFTOOLS SHARE Abstract Background Broncho‐Vaxom® (OM‐85 BV) is an extract of infectious respiratory bacteria that is used as an immunostimulant outside of the United States for the prevention and treatment of bronchitis and rhinosinusitis. Prior studies have shown that use of OM‐85 BV is associated with reduction in frequency of respiratory infection and decreased duration of antibiotic usage. However, the effects of OM‐85 BV on respiratory mucosal innate immunity are unknown.
Methods Human sinonasal epithelial cells were grown at an air‐liquid interface (ALI). Ciliary beat frequency (CBF) and nitric oxide (NO) production in response to stimulation with OM‐85 BV was measured in vitro. Pharmacologic inhibitors of bitter taste receptor (T2R) signaling were used to determine if this pathway was taste‐receptor–mediated.
Results Apical application of OM‐85 BV resulted in an NO‐mediated increase in CBF (p < 0.05) and increased NO production (p < 0.0001) when compared to saline‐stimulated control cultures. ALI pretreatment with taste receptor pathway inhibitors blocked OM‐85 BV–induced increases in NO.
Conclusion OM‐85 BV has ciliostimulatory and immunogenic properties that may be partially responsible for its observed efficacy as a respiratory therapeutic. These responses were NO‐dependent and consistent with T2R activation. Further work is necessary to elucidate specific component‐receptor signaling relationships. https://onlinelibrary.wiley.com/doi/10.1002/alr.22276
Antineoplastic drugs (AD) have been increasingly used, but the disposal of their wastes in the environment via hospital effluent and domestic sewage has emerged as an environmental issue. The current risks posed to these animals and effects of pollutants on the reptiles' population level remain unknown due to lack of studies on the topic. The aim of the present study was to evaluate the mutagenicity of neonate Podocnemis expansa exposed to environmental concentrations (EC) of cyclophosphamide (Cyc). The adopted doses were EC-I 0.2 μg/L and EC-II 0.5 μg/L Cyc. These doses correspond to 1/10 and ¼ of concentrations previously identified in hospital effluents. Turtles exposed to the CyC recorded larger total number of erythrocyte nuclear abnormalities than the ones in the control group after 48-h exposure. The total number of abnormalities for both groups (EC-I and EC-II) 96 h after the experiment had started was statistically similar to that of animals exposed to high Cyc concentration (positive control 5 × 104 μg/L). This outcome confirms the mutagenic potential of Cyc, even at low concentrations. On the other hand, when the animals were taken to a pollutant-free environment, their mutagenic damages disappeared after 240 h. After such period, their total of abnormalities matched the basal levels recorded for the control group. Therefore, our study is the first evidence of AD mutagenicity in reptiles, even at EC and short-term exposure, as well as of turtles' recovery capability after the exposure to Cyc.
Source: Journal of the American Academy of Dermatology, Volume 80, Issue 2
Author(s): Vaianu Leroy, Pauline Henrot, Thomas Barnetche, Muriel Cario, Anne-Sophie Darrigade, Pauline Manicki, Marie-Sylvie Doutre, Estibaliz Lazaro, Joel Constans, Damien Barcat, Jean-Philippe Vernhes, Christophe Richez, Alain Taieb, Marie-Elise Truchetet, Julien Seneschal, Fédération Hospitalo-Universitaire–Aquitaine's Care and Research Organisation for Inflammatory and Immune-Mediated Diseases
Background
Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker.
Objectives
To assess the association between pigmentation disorders and systemic involvement in patients with SSc.
Methods
A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation.
Results
A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89).
Limitations
This was a single-center retrospective study of a cohort of patients with SSc.
Conclusion
Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc.
Source: Journal of the American Academy of Dermatology, Volume 80, Issue 2
Author(s): Anne Lynn S. Chang, Duy C. Tran, John G.D. Cannon, Shufeng Li, Mark Jeng, Roma Patel, Lindsay Van der Bokke, Alana Pague, Richard Brotherton, Kerri E. Rieger, Ansuman T. Satpathy, Kathryn E. Yost, Sunil Reddy, Kavita Sarin, A. Dimitrios Colevas
Source: Journal of the American Academy of Dermatology, Volume 80, Issue 2
Author(s): Catherine C. Motosko, Anna K. Ault, Laura L. Kimberly, George A. Zakhem, M. David Gothard, Roger S. Ho, Alexes Hazen
Background
Spin—reporting that distorts the interpretation of results—is not unusual within scientific literature.
Objective
To appraise strategies of spin among placebo-controlled double-blind clinical trials of topical treatments for photoaged skin.
Methods
A systematic review of the literature was performed to identify placebo-controlled double-blind clinical trials of topical treatments for photoaged skin. A survey of spin strategies was developed and applied to the cohort of identified studies.
Results
The systematic review led to the identification of 20 studies in which various types of spin strategies, broadly classified as either inappropriate statistical analyses or inappropriate interpretation of results, were used. The most commonly used strategies included use of multiple primary outcomes (95%), inappropriate extrapolation of results from specific outcomes to global improvements (95%), focus on within-group comparisons (75%), and focus on interim analyses to give more weight to nonsignificant findings (65%).
Limitations
Classification of spin strategies was subjective and might not encompass all the methods used in the published literature.
Conclusion
Findings in this study inform efforts to reduce spin in the dermatologic literature.
Source: Journal of the American Academy of Dermatology, Volume 80, Issue 2
Author(s): Huilin Tang, Weilong Shi, Yiqing Song, Jiali Han
Background
Current evidence about the association between voriconazole and risk of cutaneous squamous cell carcinoma (SCC) remains inconsistent.
Objective
To assess the association between voriconazole use and risk of SCC.
Methods
We systematically searched PubMed and Embase and performed a random effects model meta-analysis to calculate the pooled relative risk (RR) with a 95% confidence interval (CI).
Results
Of the 8 studies involving a total of 3710 individuals with a lung transplant or hematopoietic cell transplant that were included in the qualitative analysis, 5 were included in the meta-analysis. Use of voriconazole was significantly associated with increased risk of SCC (RR, 1.86; 95% CI, 1.36-2.55). The increased risk did not differ according to type of transplantation or adjustment for sun exposure. Longer duration of voriconazole use was found to be positively associated with risk of SCC (RR, 1.72; 95% CI, 1.09-2.72). Voriconazole use was not associated with increased risk of basal cell carcinoma (RR, 0.84; 95% CI, 0.41-1.71).
Limitations
There were some heterogeneities in the retrospective observational studies.
Conclusions
Our findings support an increased risk of SCC associated with voriconazole in individuals with a lung transplant or hematopoietic cell transplant. Routine dermatologic surveillance should be performed, especially among individuals at high risk of developing SCC.
Source: Journal of the American Academy of Dermatology, Volume 80, Issue 2
Author(s): Fan Di Xia, Bart S. Ferket, Victor Huang, Robert S. Stern, Peggy A. Wu
Background
Treatments for early-stage mycosis fungoides (MF) include topical steroids, topical nitrogen mustard, topical bexarotene, narrowband ultraviolet B (NBUVB), psoralen plus ultraviolet A (PUVA), and local radiation. The relative cost-effectiveness of each treatment given the differences in treatment failure, disease progression, and therapy escalation is not established.
Objective
To compare the cost-effectiveness (CE) of treatment options for stage IA MF.
Methods
A state-transition model was constructed with health states of stage IA to stage IV disease, no MF, and death. Treatment-specific remission and relapse rates were obtained from the literature. Lifetime costs were calculated by accounting for medications, office visits, laboratory monitoring, related procedures, work absences, and travel.
Results
The order of CE of the study treatments was determined to be as follows: local radiation, $225,399 for 15.40 life-years (LYs); NBUVB, $344,728 for 15.17 LYs; PUVA, $371,741 for 15.07 LYs; topical corticosteroids, $469,354 for 14.65 LYs; topical nitrogen mustard, $951,662 for 14.29 LYs; and topical bexarotene, 11,892,496 for 13.55 LYs. Sensitivity analyses confirmed the CE rankings.
Limitations
We assumed a constant probability of response, relapse rates, and 3-month treatment intervals.
Conclusions
Local radiation is the most cost-effective treatment for limited local disease, whereas phototherapy (NBUVB or PUVA) is cost-effective for generalized disease. Our findings can serve to inform future studies and recommendations regarding selection of therapy for stage IA MF.
Source: Journal of the American Academy of Dermatology, Volume 80, Issue 2
Author(s): Feras M. Ghazawi, Janelle Cyr, Rami Darwich, Michelle Le, Elham Rahme, Linda Moreau, Elena Netchiporouk, Andrei Zubarev, Osama Roshdy, Steven J. Glassman, Denis Sasseville, Ivan V. Litvinov
Background
The incidence of cutaneous malignant melanoma (CMM) is on the rise in many parts of the world. However, there is limited knowledge on the epidemiology of CMM in Canada.
Objective
To conduct a comprehensive population-based study of CMM in Canada.
Methods
We examined patient clinical and pathologic characteristics as well as the incidence and mortality trends of CMM in Canada using 3 independent population-based registries.
Results
In total, 72,565 Canadian patients were given CMM diagnoses during 1992-2010; 47.5% were women. Average age at the time of diagnosis was 56.5 years for women and 60.4 years for men. We report a steady increase in CMM incidence and mortality rates in both sexes. The overall incidence rate of CMM in Canada was 12.29 cases/100,000 person-years. We also report important differences in the incidence and mortality rates between Canadian provinces and territories; the highest incidence of this cancer was documented in Nova Scotia and Prince Edward Island.
Limitations
Data on race, clinical disease stage, and Breslow depth of CMM was not available.
Conclusion
This study, for the first time, defines the disease burden of CMM in Canada and highlights important longitudinal, geographic, and spatial differences in the distribution of CMM in this country.
Source: Journal of the American Academy of Dermatology, Volume 80, Issue 2
Author(s): Andrew N. Hanna, Andrew J. Sinnamon, Robert E. Roses, Rachel R. Kelz, David E. Elder, Xiaowei Xu, Barbara A. Pockaj, Jonathan S. Zager, Douglas L. Fraker, Giorgos C. Karakousis
Background
There is large variability in the risk of sentinel lymph node (SLN) positivity among patients with intermediate thickness melanoma (ITM), with a subgroup of patients exhibiting a low risk of nodal disease.
Objective
To identify a group of patients with ITM for whom the risk of nodal disease is low.
Methods
A retrospective cohort of patients with ITM who underwent wide excision and nodal evaluation from 2010 to 2013 was identified by using the National Cancer Database and analyzed for the presence of nodal disease. Classification and regression tree analysis identified the most important factors used in a model to identify groups at low risk of SLN positivity.
Results
Of 23,440 patients, 14.7% were found to have nodal metastasis. On classification and regression tree analysis, patients older than 55 years without lymphovascular invasion and with a lesion thickness less than 1.7 mm had an SLN positivity rate of 4.9%. A model using age and thickness in nonulcerated patients identified a low-risk subgroup with a corresponding SLN positivity rate of 4.7%.
Limitations
This was a retrospective study, and the model developed requires prospective validation.
Conclusions
Patient age is an important factor in estimating risk of SLN in patients with ITM and may help identify patients without ulceration who may be safely spared an SLN biopsy.
This study explores the role of anti‐Mullerian hormone (AMH) and LH/FSH ratio in diagnosis of polycystic ovary syndrome (PCOS).
Methods
In this multicenter cross‐sectional descriptive study, a total of 863 infertile women between 18‐ 45 years were evaluated at 3 infertility centres in Vietnam and were recruited from June 2016 to June 2017. The patients were classified into two groups: Group I included 441 patients with PCOS (based on Rotterdam criteria consensus) and Group II included 422 non‐PCOS women. Diagnosis of PCOS was established based on Rotterdam 2003 consensus and exclusion criteria were ovarian disease (ovary cyst/tumor), history of ovarian surgery, and ovarian failure.
Results
At an optimum cut‐off level of 32.79 pmol/L, AMH showed sensitivity and specificity of 78.50% and 75.83%, respectively with the AUC 0,852 (95%CI:0.826‐0.875). The LH/FSH ratio had a similar AUC at the optimum cut‐off of 1.33 (AUC=0.867, 95%CI 0.842‐0.889), which demonstrated a similar diagnosis value to AMH (p=0.340). By using multiple logistic regression analysis, one ng/ml increase in AMH levels was associated with an increased risk of PCOS (OR=1.63, 95%CI: 1.506 ‐ 1.764; p<0.001). Similarly, one unit increase in LH/FSH ratio was associated with 14.433 time increased (95%CI: 9.302 ‐22.395; p<0.001) risk of PCOS. There were no significant differences between values of AMH and LH/FSH ratio in PCOS diagnosis, as the difference between the two AUCs was 0.013, 95%CI: ‐0.024 ‐ 0.028 and p=0.897.
In conclusion
value of serum AMH concentration has been found not significantly superior to LH/FSH ratio in PCOS diagnosis. Although these biomarkers separately are not adequate for PCOS diagnosis based on their own value, the combination of different endocrine factors including AMH, LH, and LH/FSH ratio together with BMI and other anthropometric and clinical characteristics may offer extra value to establish the diagnosis of PCOS.
This article is protected by copyright. All rights reserved.
Natural history, prevalence, and pathophysiology of cervical spondylotic myelopathy Highly accessed articlep. 5
Gomatam Raghavan Vijay Kumar, Dibyendu Kumar Ray, Rupant Kumar Das
DOI:10.4103/isj.isj_48_18
This study is a narrative review performed to summarize the current knowledge about the epidemiology, natural history and pathogenesis of cervical spondylotic myelopathy (CSM). A comprehensive search was undertaken to look at all available articles between January 1, 1956 to May 1, 2018, on PubMed and the Cochrane Collaboration Library. The natural history of CSM is variable. The main determinants of the clinical course of CSM are the extent of neurological impairment, age, cervical instability, abnormalities of cord conduction, canal diameter, congenitally stenotic spinal canal and the extent of involvement and tract disruption on diffusion tensor imaging (DTI) imaging. There is little data on the true incidence and prevalence of CSM across the globe and none from India. The pathoanatomic basis of CSM is cord compression, either dynamic or static. The biological events that are thought to play a significant role in the development of CSM are ischemia, derangement of the blood-spinal cord barrier, chronic neuronal inflammation, and apoptosis. Emerging knowledge about the molecular biology holds promise for potential intervention, both for prevention and for cure, of this common and debilitating condition.
Clinical spectrum and importance of evaluation systems in degenerative cervical myeloradiculopathyp. 13
Ganesh Swaminathan, Vetrivel Muralidharan, Baylis Vivek Joseph
DOI:10.4103/isj.isj_61_18
Degenerative cervical myelopathy includes facet joint arthropathy and/or intervertebral disc prolapse, as well as aberration (hypertrophy, calcification, or ossification) in the ligamentum flavum, and/or posterior longitudinal ligament. Cervical spondylotic myelopathy and ossification of posterior longitudinal ligament are two major conditions under this spectrum. Patients with degenerative changes of the cervical spine can present with wide spectrum of symptoms and signs ranging from axial neck pain, radiculopathy or myelopathy. A combination of history, physical examination, and provocative tests such as Spurling's sign, shoulder abduction test, neck distraction test, Valsalva maneuver, Elvey's upper limb tension/brachial plexus tension test increase the likelihood of diagnosis of cervical radiculopathy. Myelopathy can manifest in the early stage as subtle changes in the upper limb dexterity or mild walking difficulty and in late stage with severe spasticity and flexor spasms. Clinicians are increasingly using quantitative or semi-quantitative scales of neurological impairment. However, there is no gold standard evaluation systems that can reliably assess disease severity.
Anterior surgical options for cervical spondylotic myelopathyp. 33
Andrei Fernandes Joaquim, John Alex Sielatycki, K Daniel Riew
DOI:10.4103/isj.isj_39_18
Cervical spondylotic myelopathy (CSM) is one of the most common among causes of spinal cord dysfunction worldwide. In this article, we provide a broad narrative review of the options to treat CSM from an anterior approach to the cervical spine. Anterior procedures are effective and safe, especially for one or two level disease (although can be used up to 7-8 levels). This approach can be used in patients with lordotic, neutral, or kyphotic cervical spine alignment and provide excellent access for direct neural decompression. The most common adverse effects of anterior cervical operations are dysphagia and dysphonia, but fortunately, these are mild and transient in the majority of cases. Severe complications, such as vertebral arterial injury, spinal cord injury or airway compromise, are rare but must be taken into consideration, especially when additional risk factors are present (multilevel procedures, revision surgeries, older, and infirm patients). The primary anterior cervical procedures for treating CSM are anterior cervical discectomy and fusion (ACDF), anterior cervical corpectomy and fusion (ACCF), oblique cervical corpectomy, and cervical disc arthroplasty. A combination (hybrid) of ACDF and ACCF is also utilized as an option to allow for wide decompression, deformity correction, and provide more surface area of exposed, and bleeding cancellous bone. More recently, the senior author (KDR) has utilized a hemi-corpectomy and fusion hybrid technique which will be described in this text. Advantages and disadvantages of each of these options are discussed in detail, as well as the need for posterior instrumentation supplementation in selected patients; such as those with concomitant cervical deformity, poor bone quality, or those at risk for pseudarthrosis following multilevel arthrodeses. The management of patients with cervical spinal cord compression without myelopathy or with mild symptoms is also discussed.
Posterior surgical options for spondylotic cervical myelopathyp. 42
Shankar Acharya, Nikhil Jain
DOI:10.4103/isj.isj_57_18
Cervical spondylotic myelopathy (CSM) is a common presentation in the middle-aged to elderly population. The cause of myelopathy is multifactorial, and cervical spondylosis is the most common cause. This review looks into the treatment options, timing of the surgery, and the advantages and disadvantages of the various posterior approaches for multilevel spondylotic myelopathy. CSM is a disabling disorder that should be addressed in its early phases. There are limited surgical options available, and each procedure has its advantages and disadvantages. Since the neurological and functional outcomes are the same for all well-performed decompressions, the choice of surgical approach depends on various other factors. Posterior approaches are good for multilevel disease as they make the surgery simpler, shorter and with reduced complications in comparison to multilevel anterior surgeries.
: Etiology, prevalence, progression, and surgical strategiesp. 52
Yoshiharu Kawaguchi
DOI:10.4103/isj.isj_41_18
Ossification of the posterior longitudinal ligament (OPLL) is characterized by replacement of the ligamentous tissue by ectopic new bone formation. OPLL often causes narrowing of the spinal canal and has been recognized as a cause of cervical myelopathy and/or radiculopathy. Although a clear inheritance of OPLL has not been identified, there is a strong genetic background for OPLL. A recent genome-wide association study using all Japan cohort reported that there were 6 susceptible loci for OPLL. In addition, there were several studies to seek the biomarkers of OPLL. OPLL is frequently found in the cervical spine. However, 53.4% had OPLL not only in the cervical spine, but also in other spinal regions in patients with cervical OPLL. Further, 65.2% with cervical OPLL had ossification of the ligamentum flavum (OLF) especially at the levels of the thoracic and the lumbar spine. There is no effective conservative treatment. Surgical decompression is considered in patients with severe and/or progressive myelopathy. Early surgical decompression of the spinal cord is recommended in patients with apparent myelopathy. Operative methods are divided into two procedures, anterior decompressive surgery and posterior decompressive surgery. The choice of the surgical procedure is determined according to several factors, such as local pathology of OPLL and spinal alignment.
K Venugopal Menon, Hood Al Saqri, Renjit Kumar, Maruti Kambali DOI:10.4103/isj.isj_8_18
Background: Wide exposure to the anterior part of the upper cervical spine is difficult due to anatomical constraints. The Labio-Mandibulo-Glossotomy (LMG) approach is considered a difficult approach with high morbidity. The objective of this study is to describe the authors experience with the approach and it's outcomes in six cases and offer tips and pearls to the surgical access. Methods: This is a retrospective review of a small series of six cases that were operated for upper cervical lesions by the LMG approach. Two had mandible fractures that needed fixation and in the others osteotomy of the mandible was performed. The patients were followed up for minimum two years or death (in malignancy). We specifically looked for cosmetic or functional problems related to osteotomy, glossotomy, and, hospital and ICU stay duration. Surgical access is described in detail. Results: The hospital stay was similar to other major spine trauma or tumour surgeries at our center (median 14 days) and mean ICU stay 2.8 days. There were no long-term issues related to the access. Several tips and tricks are offered to minimize intra and post-operative problems. Conclusions: The LMG approach, though apparently formidable, is quite a safe and simple procedure with few residual complications.
This study is a narrative review performed to summarize the current knowledge about the epidemiology, natural history and pathogenesis of cervical spondylotic myelopathy (CSM). A comprehensive search was undertaken to look at all available articles between January 1, 1956 to May 1, 2018, on PubMed and the Cochrane Collaboration Library. The natural history of CSM is variable. The main determinants of the clinical course of CSM are the extent of neurological impairment, age, cervical instability, abnormalities of cord conduction, canal diameter, congenitally stenotic spinal canal and the extent of involvement and tract disruption on diffusion tensor imaging (DTI) imaging. There is little data on the true incidence and prevalence of CSM across the globe and none from India. The pathoanatomic basis of CSM is cord compression, either dynamic or static. The biological events that are thought to play a significant role in the development of CSM are ischemia, derangement of the blood-spinal cord barrier, chronic neuronal inflammation, and apoptosis. Emerging knowledge about the molecular biology holds promise for potential intervention, both for prevention and for cure, of this common and debilitating condition.
Clinical spectrum and importance of evaluation systems in degenerative cervical myeloradiculopathy
p. 13
Ganesh Swaminathan, Vetrivel Muralidharan, Baylis Vivek Joseph DOI:10.4103/isj.isj_61_18
Degenerative cervical myelopathy includes facet joint arthropathy and/or intervertebral disc prolapse, as well as aberration (hypertrophy, calcification, or ossification) in the ligamentum flavum, and/or posterior longitudinal ligament. Cervical spondylotic myelopathy and ossification of posterior longitudinal ligament are two major conditions under this spectrum. Patients with degenerative changes of the cervical spine can present with wide spectrum of symptoms and signs ranging from axial neck pain, radiculopathy or myelopathy. A combination of history, physical examination, and provocative tests such as Spurling's sign, shoulder abduction test, neck distraction test, Valsalva maneuver, Elvey's upper limb tension/brachial plexus tension test increase the likelihood of diagnosis of cervical radiculopathy. Myelopathy can manifest in the early stage as subtle changes in the upper limb dexterity or mild walking difficulty and in late stage with severe spasticity and flexor spasms. Clinicians are increasingly using quantitative or semi-quantitative scales of neurological impairment. However, there is no gold standard evaluation systems that can reliably assess disease severity.
Rajavelu Rajesh, Shanmuganathan Rajasekaran, Sri Vijayanand DOI:10.4103/isj.isj_63_18
This is a narrative review. The objective of this study is to provide an overview on the imaging modalities and their utilization in cervical myelopathy (CM). Using PubMed, studies published on the "imaging modalities in CM," "cervical spondylotic myelopathy (CSM) imaging," "computed tomography (CT) and magnetic resonance imaging (MRI) in CM," "imaging in ossified posterior longitudinal ligament (OPLL)," "dural ossification in OPLL," "diffusion tensor imaging (DTI) in CSM," and "dynamic MRI, functional MRI, and magnetic resonance spectroscopy (MRS) in CSM" were evaluated. The review addresses the evaluation of CM with various imaging modalities ranging from radiographs, CT, and MRI to advanced imaging techniques such as DTI and MRS. Each investigation contributes specific detail to the disease process in a different dimension. Specific parameters for CSM and OPLL, and their influence on outcome are discussed. Imaging in CM plays an important role in analyzing the cause of myelopathy, defining the level of the lesion, parameters to assess the time of intervention and to predict the outcome.
Anterior surgical options for cervical spondylotic myelopathy
p. 33
Andrei Fernandes Joaquim, John Alex Sielatycki, K Daniel Riew DOI:10.4103/isj.isj_39_18
Cervical spondylotic myelopathy (CSM) is one of the most common among causes of spinal cord dysfunction worldwide. In this article, we provide a broad narrative review of the options to treat CSM from an anterior approach to the cervical spine. Anterior procedures are effective and safe, especially for one or two level disease (although can be used up to 7-8 levels). This approach can be used in patients with lordotic, neutral, or kyphotic cervical spine alignment and provide excellent access for direct neural decompression. The most common adverse effects of anterior cervical operations are dysphagia and dysphonia, but fortunately, these are mild and transient in the majority of cases. Severe complications, such as vertebral arterial injury, spinal cord injury or airway compromise, are rare but must be taken into consideration, especially when additional risk factors are present (multilevel procedures, revision surgeries, older, and infirm patients). The primary anterior cervical procedures for treating CSM are anterior cervical discectomy and fusion (ACDF), anterior cervical corpectomy and fusion (ACCF), oblique cervical corpectomy, and cervical disc arthroplasty. A combination (hybrid) of ACDF and ACCF is also utilized as an option to allow for wide decompression, deformity correction, and provide more surface area of exposed, and bleeding cancellous bone. More recently, the senior author (KDR) has utilized a hemi-corpectomy and fusion hybrid technique which will be described in this text. Advantages and disadvantages of each of these options are discussed in detail, as well as the need for posterior instrumentation supplementation in selected patients; such as those with concomitant cervical deformity, poor bone quality, or those at risk for pseudarthrosis following multilevel arthrodeses. The management of patients with cervical spinal cord compression without myelopathy or with mild symptoms is also discussed.
Cervical spondylotic myelopathy (CSM) is a common presentation in the middle-aged to elderly population. The cause of myelopathy is multifactorial, and cervical spondylosis is the most common cause. This review looks into the treatment options, timing of the surgery, and the advantages and disadvantages of the various posterior approaches for multilevel spondylotic myelopathy. CSM is a disabling disorder that should be addressed in its early phases. There are limited surgical options available, and each procedure has its advantages and disadvantages. Since the neurological and functional outcomes are the same for all well-performed decompressions, the choice of surgical approach depends on various other factors. Posterior approaches are good for multilevel disease as they make the surgery simpler, shorter and with reduced complications in comparison to multilevel anterior surgeries.
Ossification of the posterior longitudinal ligament: Etiology, prevalence, progression, and surgical strategies
p. 52
Yoshiharu Kawaguchi DOI:10.4103/isj.isj_41_18
Ossification of the posterior longitudinal ligament (OPLL) is characterized by replacement of the ligamentous tissue by ectopic new bone formation. OPLL often causes narrowing of the spinal canal and has been recognized as a cause of cervical myelopathy and/or radiculopathy. Although a clear inheritance of OPLL has not been identified, there is a strong genetic background for OPLL. A recent genome-wide association study using all Japan cohort reported that there were 6 susceptible loci for OPLL. In addition, there were several studies to seek the biomarkers of OPLL. OPLL is frequently found in the cervical spine. However, 53.4% had OPLL not only in the cervical spine, but also in other spinal regions in patients with cervical OPLL. Further, 65.2% with cervical OPLL had ossification of the ligamentum flavum (OLF) especially at the levels of the thoracic and the lumbar spine. There is no effective conservative treatment. Surgical decompression is considered in patients with severe and/or progressive myelopathy. Early surgical decompression of the spinal cord is recommended in patients with apparent myelopathy. Operative methods are divided into two procedures, anterior decompressive surgery and posterior decompressive surgery. The choice of the surgical procedure is determined according to several factors, such as local pathology of OPLL and spinal alignment.
Clinical predictors of complications and outcomes in degenerative cervical myeloradiculopathy
p. 59
Jamie R F Wilson, Fan Jiang, Michael G Fehlings DOI:10.4103/isj.isj_60_18
Degenerative cervical myelopathy (DCM) is the leading cause of adult spinal cord dysfunction worldwide, and surgical decompression remains the mainstay treatment to arrest the progression of neurological deterioration. A number of clinical factors can predict and influence the outcomes of surgery, including patient demographics, baseline myelopathy severity, duration of symptoms, imaging characteristics, and types of surgical approach. Understanding the influence and relationship of these factors on surgical outcomes allows the treating clinician the ability to provide the patient with realistic expectations when discussing surgical intervention for DCM.
The spine clinics – Cervical spondylotic myelopathy – Clinical scenarios
p. 68
Ankur Nanda, KR Renjith, Abhinandan Mallepally, C S Vishnu Prasath, Ajoy P Shetty DOI:10.4103/isj.isj_67_18
This section of the symposium deals with different case scenarios related to cervical spondylotic myelopathy (CSM) which in our daily clinical practice not only act as diagnostic challenges but also test our decision-making abilities. These cases have been handled by different experts and hence help the readers in providing a wider perspective to the problem of cervical myelopathy and its management. This section ends with comments by the authors on key takeaway points from each case scenario, and some literature supported recommendations for the management of CSM.
Cell-based treatment strategies for intervertebral disc degeneration: An overview on potentials and shortcomings
p. 81
Prasanthi Sampara, Rajkiran Reddy Banala, Satish Kumar Vemurit, AV Gurava Reddy, G P V Subbaiah DOI:10.4103/isj.isj_21_17
The intervertebral discs (IVDs) are the cushioning pads of fibrocartilage, which are immeasurably vital for the uprightness of vertebral column and for its function. IVD provides flexibility, tensile strength to the spine, and also cope up with varied types of biomechanical stresses. IVD degeneration (IVDD) is one of the musculoskeletal disorders mostly seen in older population, and it is the foremost cause of low back pain and consequences of IVDD are disc herniation, spinal stenosis, and degenerative lumbar scoliosis. Yet the therapeutic options are restricted and the treatments given remain unsatisfactory putting more economical burden on world's population. IVDD is considered as a multifactorial disorder, due to the involvement of factors such as genetic inheritance, alterations in cellular composition, and anabolic and catabolic reactions, which could initiate degenerative process in the IVD. However, our conception on IVD genesis and the etiopathology of IVDD have given us an opportunity for exploring and formulate appropriate therapies to tackle IVDD. The cell therapy gives scope for sustained matrix synthesis, controlled inflammation, and prevention of osteophyte formation in IVD. The present review focuses on the existing issues related to current therapeutic approaches and about latest evidence on cell therapy-based regeneration of IVD and maintaining the microenvironment of cellular matrix which holds a promise for future therapeutic applications.
The median labio-mandibulo-glossotomy approach to the upper cervical spine: A personal series and tips and pearls
p. 92
K Venugopal Menon, Hood Al Saqri, Renjit Kumar, Maruti Kambali DOI:10.4103/isj.isj_8_18
Background: Wide exposure to the anterior part of the upper cervical spine is difficult due to anatomical constraints. The Labio-Mandibulo-Glossotomy (LMG) approach is considered a difficult approach with high morbidity. The objective of this study is to describe the authors experience with the approach and it's outcomes in six cases and offer tips and pearls to the surgical access. Methods: This is a retrospective review of a small series of six cases that were operated for upper cervical lesions by the LMG approach. Two had mandible fractures that needed fixation and in the others osteotomy of the mandible was performed. The patients were followed up for minimum two years or death (in malignancy). We specifically looked for cosmetic or functional problems related to osteotomy, glossotomy, and, hospital and ICU stay duration. Surgical access is described in detail. Results: The hospital stay was similar to other major spine trauma or tumour surgeries at our center (median 14 days) and mean ICU stay 2.8 days. There were no long-term issues related to the access. Several tips and tricks are offered to minimize intra and post-operative problems. Conclusions: The LMG approach, though apparently formidable, is quite a safe and simple procedure with few residual complications.
A novel surgical technique for hydatid cyst involving cervicothoracic anterior epidural space
p. 99
Bharat R Dave, Degulmadi Devanand, Ganesh Deshmukh DOI:10.4103/isj.isj_17_18
Spinal hydatid cyst comprises <1% of the total cases of hydatid disease. There is very little literature on the involvement of anterior epidural space by hydatid cyst and its management. This report presents a unique presentation of spinal hydatidosis in cervicothoracic anterior epidural space and a novel technique in surgical management.
Sacral chordoma with degenerative spondylolisthesis and upper lumbar disc herniation
p. 102
Shakti A Goel, Hitesh N Modi, Yatin J Desai, Bhavin Patel DOI:10.4103/isj.isj_24_17
Sacral chordoma is a rare condition requiring multidisciplinary approach for management. Here, we report a 72-year-old male patient who was diagnosed with sacral chordoma with L2–L3 disc herniation and L5–S1 degenerative spondylolisthesis and L1 body fracture. The patient was first managed by discectomy L2–L3 with D12–L3 decompression and fixation. Sacral chordoma excision was done 10 months later. The chordoma was excised by anterior laparoscopic resection and mobilization of tissues from the tumor followed by posterior sacrectomy with L5–S1 decompression and extension of fixation in a single stage. Proline mesh was used to support the colon posteriorly. This was further complicated by proximal junction fracture due to fall which was further managed by proximal extension of the rod-screw construct. The patient became symptom free without any radiotherapy or chemotherapy and is able to walk independently, two years following the primary surgery without recurrence of tumor.
Alkaptonuria is a rare metabolic, autosomal recessive disorder caused by the deficiency of homogentisic acid oxidase and it is characterized by bluish-black discoloration of cartilages, skin (Ochronosis), degenerative changes in the articular, extra-articular cartilages, intervertebral disc, other tissues causing pain in the joints and spinal column. Although intervertebral disc degeneration is common in these patients, those presenting with symptoms severe enough to warrant surgery are rare. Only a few patients have been treated surgically. We present a case of alkaptonuria presenting with radiculopathy and lumbar disc herniation. The case presented demonstrates that although lumbar disc herniation is rare in alkaptonuria, it should be sought in such patients and surgical treatment yields good functional outcome.
