Langerhans cell histiocytosis of the scapula - diagnosis & treatment options / Histiocitose de células de langerhans da escápula - diagnóstico e opções de tratamento / Histiocitosis de células de langerhans de la escápula - diagnóstico y opciones de tratamiento

ABSTRACT Langerhans cell histiocytosis (LCH) is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presentation of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single system disease, so conservative management was performed, and the patent improved clinically.

RESUMO A histiocitose de células de Langerhans (HCL) caracteriza-se por acúmulo anormal de histiócitos em tecidos como pulmão, baço, medula óssea, pele, sistema nervoso central, fígado e linfonodos, causando efeitos focais ou sistêmicos. Nenhuma apresentação clínica e radiográfica específica da HCL está descrita na literatura. Isso impõe um dilema diagnóstico para os cirurgiões. A escápula é o local de 3% dos tumores ósseos, ao passo que é o lugar menos comum para a HCL. Em um menino de 10 anos de idade, com lesão isolada na escápula e sem outro envolvimento sistêmico, sem achados específicos na RM ou na TC da escápula, o diagnóstico foi confirmado pela biópsia. A divisão entre doença isolada e de múltiplos sistemas é fundamental para o tratamento, considerando-se que este caso é uma doença de um só sistema. O paciente teve melhora clínica no acompanhamento de dois anos. A escápula é um dos locais mais raros de ocorrência da HCL, e como as lesões mimetizam umas às outras, sempre é preciso realizar biópsia por imuno-histoquímica para CD68 e S-100. Esta doença atingiu apenas um sistema, levando ao tratamento conservador e o paciente apresentou melhora clínica.

RESUMEN La histiocitosis de células de Langerhans (HCL) se caracteriza por la acumulación anormal de histiocitos en tejidos como pulmón, bazo, médula ósea, piel, sistema nervioso central, hígado y linfonodos, causando efectos focales o sistémicos. Ninguna presentación clínica y radiográfica específica de la HCL está descrita en la literatura. Eso impone un dilema diagnóstico para los cirujanos. La escápula es el local de 3% de los tumores óseos, al paso que es el lugar menos común para la HCL. En un niño de 10 años de edad, con lesión aislada en la escápula y sin otro compromiso sistémico, sin hallazgos específicos en la RM o en la TC de la escápula, el diagnóstico fue confirmado por la biopsia. La división entre enfermedad aislada y de múltiples sistemas es fundamental para el tratamiento, considerándose que este caso es una enfermedad de un único sistema. El paciente tuvo mejora clínica en el acompañamiento de dos años. La escápula es uno de los locales más raros de ocurrencia de la HCL, y como las lesiones mimetizan unas a otras, siempre es preciso realizar biopsia por inmunohistoquímica para CD68 y S-100. Esta enfermedad alcanzó a sólo un sistema, llevando al tratamiento conservador y el paciente presentó mejora clínica.

Transposition of Great Arteries with Intramural Coronary Artery: Experience with a Modified Surgical Technique.

OBJECTIVE: Transposition of the great arteries is a common congenital heart disease. Arterial switch is the gold standard operation for this complex heart disease. Arterial switch operation in the presence of intramural coronary artery is surgically the most demanding even for the most experienced hands. We are presenting our experience with a modified technique for intramural coronary arteries in arterial switch operation. METHODS: This prospective study involves 450 patients undergoing arterial switch operation at our institute from April 2006 to December 2013 (7.6 years). Eighteen patients underwent arterial switch operation with intramural coronary artery. The coronary patterns and technique used are detailed in the text. RESULTS: The overall mortality found in the subgroup of 18 patients having intramural coronary artery was 16% (n=3). Our first patient had an accidental injury to the left coronary artery and died in the operating room. A seven-day old newborn died from intractable ventricular arrhythmia fifteen hours after surgery. Another patient who had multiple ventricular septal defects with type B arch interruption died from residual apical ventricular septal defect and sepsis on the eleventh postoperative day. The remainder of the patients are doing well, showing a median follow-up duration of 1235.34±815.26 days (range 369 - 2730). CONCLUSION: Transposition of the great arteries with intramural coronary artery is demanding in a subset of patients undergoing arterial switch operation. We believe our technique of coronary button dissection in the presence of intramural coronary arteries using coronary shunt is simple and can be a good addition to the surgeons' armamentarium.

Transposition of Great Arteries with Intramural Coronary Artery: Experience with a Modified Surgical Technique

Abstract Objective: Transposition of the great arteries is a common congenital heart disease. Arterial switch is the gold standard operation for this complex heart disease. Arterial switch operation in the presence of intramural coronary artery is surgically the most demanding even for the most experienced hands. We are presenting our experience with a modified technique for intramural coronary arteries in arterial switch operation. Methods: This prospective study involves 450 patients undergoing arterial switch operation at our institute from April 2006 to December 2013 (7.6 years). Eighteen patients underwent arterial switch operation with intramural coronary artery. The coronary patterns and technique used are detailed in the text. Results: The overall mortality found in the subgroup of 18 patients having intramural coronary artery was 16% (n=3). Our first patient had an accidental injury to the left coronary artery and died in the operating room. A seven-day old newborn died from intractable ventricular arrhythmia fifteen hours after surgery. Another patient who had multiple ventricular septal defects with type B arch interruption died from residual apical ventricular septal defect and sepsis on the eleventh postoperative day. The remainder of the patients are doing well, showing a median follow-up duration of 1235.34±815.26 days (range 369 - 2730). Conclusion: Transposition of the great arteries with intramural coronary artery is demanding in a subset of patients undergoing arterial switch operation. We believe our technique of coronary button dissection in the presence of intramural coronary arteries using coronary shunt is simple and can be a good addition to the surgeons' armamentarium.

Five-year outcomes after off-pump or on-pump coronary-artery bypass grafting

BACKGROUND: We previously reported that there was no significant difference at 30 days or at 1 year in the rate of the composite outcome of death, stroke, myocardial infarction, or renal failure between patients who underwent coronary-artery bypass grafting (CABG) performed with a beating-heart technique (off-pump) and those who underwent CABG performed with cardiopulmonary bypass (on-pump). We now report the results at 5 years (the end of the trial). METHODS: A total of 4752 patients (from 19 countries) who had coronary artery disease were randomly assigned to undergo off-pump or on-pump CABG. For this report, we analyzed a composite outcome of death, stroke, myocardial infarction, renal failure, or repeat coronary revascularization (either CABG or percutaneous coronary intervention). The mean follow-up period was 4.8 years. RESULTS: There were no significant differences between the off-pump group and the on-pump group in the rate of the composite outcome (23.1% and 23.6%, respectively; hazard ratio with off-pump CABG, 0.98; 95% confidence interval [CI], 0.87 to 1.10; P=0.72) or in the rates of the components of the outcome, including repeat coronary revascularization, which was performed in 2.8% of the patients in the off-pump group and in 2.3% of the patients in the on-pump group (hazard ratio, 1.21; 95% CI, 0.85 to 1.73; P=0.29)...

Off-pump or on-pump coronary-arterybypass grafting at 30 days

BackgroundThe relative benefits and risks of performing coronary-artery bypass grafting (CABG) with a beating-heart technique (off-pump CABG), as compared with cardiopulmonarybypass (on-pump CABG), are not clearly established.MethodsAt 79 centers in 19 countries, we randomly assigned 4752 patients in whom CABG was planned to undergo the procedure off-pump or on-pump. The first coprimary outcome was a composite of death, nonfatal stroke, nonfatal myocardial infarction, or new renal failure requiring dialysis at 30 days after randomization.ResultsThere was no significant difference in the rate of the primary composite outcomebetween off-pump and on-pump CABG (9.8% vs. 10.3%; hazard ratio for the offpump group, 0.95; 95% confidence interval [CI], 0.79 to 1.14; P = 0.59) or in any of its individual components. The use of off-pump CABG, as compared with on-pump CABG, significantly reduced the rates of blood-product transfusion (50.7% vs. 63.3%; relative risk, 0.80; 95% CI, 0.75 to 0.85; P<0.001), reoperation for perioperative bleeding(1.4% vs. 2.4%; relative risk, 0.61; 95% CI, 0.40 to 0.93; P = 0.02), acute kidney injury (28.0% vs. 32.1%; relative risk, 0.87; 95% CI, 0.80 to 0.96; P = 0.01), and respiratorycomplications (5.9% vs. 7.5%; relative risk, 0.79; 95% CI, 0.63 to 0.98; P = 0.03) but increased the rate of early repeat revascularizations (0.7% vs. 0.2%; hazard ratio, 4.01; 95% CI, 1.34 to 12.0; P = 0.01).ConclusionsThere was no significant difference between off-pump and on-pump CABG with respect to the 30-day rate of death, myocardial infarction, stroke, or renal failure requiring dialysis. The use of off-pump CABG resulted in reduced rates of transfusion, reoperation for erioperative bleeding, respiratory complications, and acute kidney injury but also resulted in an increased risk of early revascularization. (Funded by theCanadian Institutes of Health Research; CORONARY ClinicalTrials.gov number,NCT00463294.)

Rationale and design of the coronary artery bypass grafting surgery off or on pump revascularization study: a large international randomized trial in cardiac surgery

Background Uncertainty remains regarding the benefits and risks of the technique of operating on a beating heart(off pump) for coronary artery bypass grafting (CABG) surgery versus on-pump CABG. Prior trials had few events and relativelyshort follow-up. There is a need for a large randomized, controlled trial with long-term follow-up to inform both the short- andlong-term impact of the 2 approaches to CABG.Methods We plan to randomize 4,700 patients in whom CABG is planned to undergo the procedure on pump or offpump. The coprimary outcomes are a composite of total mortality, myocardial infarction (MI), stroke, and renal failure at30 days and a composite of total mortality, MI, stroke, renal failure, and repeat revascularization at 5 years. We will alsoundertake a cost-effectiveness analysis at 30 days and 5 years after CABG surgery. Other outcomes include neurocognitivedysfunction, recurrence of angina, cardiovascular mortality, blood transfusions, and quality of life.Results As of May 3, 2011, CORONARY has recruited N3,884 patients from 79 centers in 19 countries. Currently,patient's mean age is 67.6 years, 80.7% are men, 47.0% have a history of diabetes, 51.4% have a history of smoking, and34.4% had a previous MI. In addition, 20.9% of patients have a left main disease, and 96.6% have double or triplevessel disease.Conclusions CORONARY is the largest trial yet conducted comparing off-pump CABG to on-pump CABG. Its resultswill lead to a better understanding of the safety and efficacy of off-pump CABG. (Am Heart J 2012;163:1-6.)