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INTRODUCTION: Mucopolysaccharidoses (MPSs) are a group of heredity storage diseases, transmitted in an autosomal recessive manner, associated with the accumulation of glycosaminoglycans (GAGs) in various tissues and organs. The concerned patients have multiple concomitant hereditary anomalies. Considering the craniofacial abnormality in these patients, airway management may be difficult for anesthesiologists. In these patients, preanesthetic assessment is necessary and performed with the accurate assessment of airways, consisting of the physical exam and radiography, MRI or CT of head and neck. An anesthesiologist should set up a "difficult intubation set" with a flexible fiber-optic bronchoscope and also, it may be necessary to discuss with an ear-nose and throat (ENT) specialist if required, for unpredicted emergency situations. CASE PRESENTATION: In this case-report we presented a 2-year-old boy with known MPSs with psychomotor retardation, bilateral corneal opacities, impaired hearing and vision, inguinal hernia, severe mitral regurgitation, micrognathia, coarse facial feature, stiff and short neck and restricted mouth opening. He scheduled for left inguinal hernia repair surgery. DISCUSSION: The patient's difficult airway was managed successfully and the anesthesia of his surgical procedure had an uneventful course.
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BACKGROUND: Evaluation of operational risk is a consequential goal in perioperative management of patients in cardiac surgery. Preoperative total lymphocyte count (PTLC) is a prognostic criterion of adverse major cardiovascular outcomes. OBJECTIVES: The purpose of this study was to investigate the prognostic value of PTLC as an independent predictor of postoperative morbidity and mortality in cardiac surgery. PATIENTS AND METHODS: Of 1604 patients scheduled for cardiac surgery between September, 2012 and March, 2013, a total of 1171 consecutive patients underwent elective primary valvular heart surgery and coronary artery bypass grafting. The patients were divided to three groups according to their PTLCs. The baseline characteristics and postoperative mortality and morbidity of the patients as well as the intensive care unit (ICU) stay according to the PTLCs were recorded and analyzed. The only inclusion criterion was a preoperative complete blood count. Exclusion criteria included: ages under 18 or over 80 years old, emergency surgery, adult patients with congenital heart disease and previous open heart surgery, and patients with any bacterial or viral infection during two weeks before the surgery. Protocol of anesthetic medications was used in all the patients similarly and according to standard. All the patients were admitted to the ICU after the surgery. RESULTS: A PTLC < 1500 cells/µL was associated with significantly high mortality and morbidity (P = 0.0001). In-hospital mortality and major composite morbidity were 9.65% and 28.4%, respectively. Low PTLC was associated with more frequent need for inotropic and intra-aortic balloon pump (IABP) support (P < 0.001), dialysis-dependent acute renal failure (P = 0.0001), postoperative superficial wound infections (P = 0.0001) and prolong ICU stay (P = 0.0001). CONCLUSIONS: Our study results showed that low PTLC was an independent, valuable prognostic criterion, with high sensitivity and specificity for evaluation of postoperative morbidity and mortality in cardiac surgery.
ABSTRACT
Now-a-days truncus arteriosus has been known as "common arterial trunk" (CAT) and is an uncommon congenital cardiac defect presenting in about 1-3% congenital heart disease. Environmental and genetic factors effects on incidence of CAT and other conotruncal anomalies. The majority patients with CAT and 22q11 deletion have other anomalies such as hypoplasia or aplasia of the thymus or parathyroid glands and immune deficits (T-cell deficiency), calcium metabolism disorder (hypocalcemia), palatal defects, learning and speech disorder, craniofacial anomalies, and neuropsychological abnormalities. CAT without surgical treatment frequently involves early severe pulmonary arterial hypertension (PAH) or early death from heart failure and associated conditions. Therefore, without corrective surgical repair, most CAT patients die in the initial years of life. In numerous centers early surgical repair associated with superior than 80% long-standing survival. Anesthesiologist must be performs comprehensive preoperative evaluation of infants or neonates with this disorder. In CAT patient exactly hemodynamic monitoring and suitable techniques to regulate pulmonary vascular resistance and systemic vascular resistance and cardiac function are more important than the select of a special anesthetic drug. Therefore, anesthetic drugs should be carefully administrated and titrate and under monitoring. Management of CAT after surgical repair depends on the adequacy of treatment, cardiac function, level of PAH, and degree of bleeding. Inotropic support is frequently necessary after the cardiac ischemia associated to the surgical repair. Pulmonary vasodilator drugs were used to PAH treatment.
ABSTRACT
Disseminated intravascular coagulation is a pathologic syndrome with different medical disorders. Diagnosis and treatment of this syndrome is one of the difficult managements in medical science. Thromboelastogram is the only guide for early diagnosis and precise management of this syndrome. We describe a patient who developed disseminated intravascular coagulation due to endocarditis and spleen abscess. She was diagnosed by thromboelastography and treated successfully.
