A long-standing conundrum: an unusual case of 2:1 atrioventricular block with conflicting findings.

A 2:1 atrioventricular (AV) block can occur anywhere within the conduction system, and noninvasive measurements may not always predict the exact site of the block. Although localization of the block is critical for deciding any treatment, patients should also be carefully questioned about symptoms both at rest and during exertion. A case of 2:1 AV block that was symptomatic only during exertion, appeared infranodal by noninvasive diagnostic methods, but was found to be intranodal on electrophysiological study is reported.

Flap loss in head and neck reconstruction: Is there a singular cause for failure?

Despite the high success rates reported in head and neck reconstruction, free flap failures continue to persist. Understanding the factors associated with flap loss and improving overall success are paramount. This study aimed to comprehensively assess the factors influencing flap revision and free tissue transfer survival in head and neck reconstruction. The study included 70 patients with defects in the lower two-thirds of the head and neck region and underwent reconstruction using free flaps. Patient age, gender, smoking status, comorbidities and data on the location and aetiology of the defect, the specific type of flap employed, the recipient artery and vein chosen, instances of revision and the overall success of the flap were collected. The investigation aimed to establish correlations between these variables as well as flap success and revision rates. No statistically significant differences were observed in arterial and venous anastomosis revision rates, or flap survival, in relation to variables such as age, gender, flap type, smoking status, comorbidities, recipient artery or vein and the number of vein anastomoses. The malignant tumour group exhibited a lower requirement for arterial revision and a higher flap survival rate compared to the benign tumour group. This study underscores the comparable safety profiles of perforator-based and conventional flaps in head and neck reconstruction. Furthermore, it reveals that patient characteristics are not contraindications for free tissue transfer. Additionally, the quantity of the veins and choice of recipient vessels are flexible and do not significantly impact flap success. The higher rates of flap success in patients with malignant aetiology requires further investigation.

Primary Neurorraphy of the Frontotemporal Branch of the Facial Nerve in Pediatric Patients.

LEVEL OF EVIDENCE: Level III-case-control study.

Could Acute Myeloid Leukemia Have Presented Even Worse? "Uncommon Cause of Concurrently Multivessel Thrombosis". / A Leucemia Mieloide Aguda Poderia ter se Apresentado Ainda Pior? "Causa Incomum de Trombose Multiarterial Concomitante".

Acute promyelocytic leukemia (APL) is a subgroup of acute myeloid leukemia (AML). Although it is known that hemorrhagic complications are common, thrombotic complications are not as rare as thought. However, myocardial infarction and ischemic stroke incidence are very rare during AML. Here, we present the astonishing case of APL diagnosed with pancytopenia in its presentation with acute myocardial infarction and ischemic stroke.

A leucemia promielocítica aguda (LPA) é um subgrupo da leucemia mieloide aguda (LMA). Embora se saiba que as complicações hemorrágicas são comuns, as complicações trombóticas não são tão raras quanto se pensa. No entanto, infarto do miocárdio e incidência de acidente vascular cerebral isquêmico são muito raros durante a LMA. Aqui, apresentamos o caso surpreendente de LPA diagnosticada com pancitopenia em sua apresentação com infarto agudo do miocárdio e acidente vascular cerebral isquêmico.

A Leucemia Mieloide Aguda Poderia ter se Apresentado Ainda Pior? "Causa Incomum de Trombose Multiarterial Concomitante" / Could Acute Myeloid Leukemia Have Presented Even Worse? "Uncommon Cause of Concurrently Multivessel Thrombosis"

Resumo A leucemia promielocítica aguda (LPA) é um subgrupo da leucemia mieloide aguda (LMA). Embora se saiba que as complicações hemorrágicas são comuns, as complicações trombóticas não são tão raras quanto se pensa. No entanto, infarto do miocárdio e incidência de acidente vascular cerebral isquêmico são muito raros durante a LMA. Aqui, apresentamos o caso surpreendente de LPA diagnosticada com pancitopenia em sua apresentação com infarto agudo do miocárdio e acidente vascular cerebral isquêmico.

Abstract Acute promyelocytic leukemia (APL) is a subgroup of acute myeloid leukemia (AML). Although it is known that hemorrhagic complications are common, thrombotic complications are not as rare as thought. However, myocardial infarction and ischemic stroke incidence are very rare during AML. Here, we present the astonishing case of APL diagnosed with pancytopenia in its presentation with acute myocardial infarction and ischemic stroke.

Comparison of Cromolyn Sodium, Montelukast, and Zafirlukast Prophylaxis for Capsular Contracture.

BACKGROUND: Capsular contracture is the most common complication following breast augmentation. Recently, prophylaxis studies aiming to inhibit the release of profibrotic substances to prevent capsular contracture have gained in importance. This study investigated the effects of cromolyn sodium, montelukast, and zafirlukast on capsular contracture in a rat model. METHODS: Thirty female Wistar albino rats were randomly divided into five groups: control, sham, cromolyn sodium, montelukast, and zafirlukast. Intraperitoneal injections were administered daily to the sham (1 ml per day), cromolyn sodium (10 mg/kg per day), montelukast (10 mg/kg per day), and zafirlukast (1.25 mg/kg per day) groups 1 month before surgery. Miniature breast implants were then placed on the backs of the rats in each group. Injections were continued for the next 3 months. The rats were subsequently killed, and the capsules were harvested and assessed histopathologically. The histopathologic outcomes were acute inflammation status, inflammation severity, synovial metaplasia, foreign body reaction, mast cell count, and capsular thickness. RESULTS: The cromolyn sodium, montelukast, and zafirlukast groups had less acute inflammation and lower mean inflammation severity scores, foreign body reaction occurrence, mast cell counts, and capsular thickness than the control and sham groups ( p < 0.05). These parameters were better in the cromolyn sodium group than in the montelukast and zafirlukast groups ( p < 0.05). CONCLUSIONS: Cromolyn sodium appears to inhibit capsular contracture more efficiently than montelukast and zafirlukast. This report may be a pioneer study for the prophylactic use of cromolyn sodium in capsular contracture. CLINICAL RELEVANCE STATEMENT: The prophylactic administration of cromolyn sodium appears to reduce capsular contracture more efficiently than that of montelukast and zafirlukast. This report might constitute a pioneer study for the prophylactic use of cromolyn sodium in capsular contracture.

Relation Between Frailty and 1-Year Outcomes After Implantation of a Left Ventricular Assist Device.

Frailty has been associated with morbidity and mortality in patients with heart failure and those who underwent cardiac surgery. We aimed to study the effect of frailty on cardiovascular outcomes and the reversibility of frailty after the left ventricular assist device (LVAD) implantation. A total of 51 consecutive patients (44 men; aged 54 ± 10 years) scheduled to undergo LVAD implantation were assessed for frailty (Fried's phenotype, frail ≥3/5), cognitive function (using Mini-Cog), and depression (utilizing Patient Health Questionnaire-9) before the surgery and 3 months afterward. Patients were observed for mortality and adverse events [all-cause readmission, bleeding, renal dysfunction, and ventricular fibrillation (VF)/sustained ventricular tachycardia (VT)] for 12 months. More than half of the patients (54%) were designated as frail. Although there was no statistical difference in mortality among frail and nonfrail patients, frail ones were more likely to have a prolonged length of stay [adjusted odds ratio (AOR) 14.9, 95% confidence interval 1.6 to 132.5, p = 0.01]. At the 3-month reassessment after operation, frailty and cognition rates were better (frailty score [lower is better]: 3 vs 1.5, p <0.0001; cognition score [higher is better]: 4.5 vs 5, p = 0.001), and patients had less depression (Patient Health Questionnaire-9 score [lower is better]: 8 vs 4, p <0.0001). Of the secondary outcomes, only postoperative VF/sustained VT reached statistical significance in being more common among frail patients than nonfrail ones (p = 0.02). Although frailty was not associated with mortality at 1 year, prolonged length of stay occurred more with frail LVAD patients. Frailty status, cognitive function, and depressive mood all improved in most patients after LVAD.

Inferior ST-segment elevation due to metastatic cardiac tumor.

There are numerous causes for ST-segment elevation on ECG, the tumoral invasion of the heart being a rarer one. Because the management will differ one should always keep in mind the presence of such entity. Here we report a case of persistent ST-segment elevation due to a metastatic cardiac tumor.

Evaluation of versatility and outcomes of the first dorsal metacarpal artery flap in thumb defects.

BACKGROUND: It is clinically vital to determine the best technique to reconstruct thumb defects with satisfactory esthetic and functional outcomes. We aimed to quantitatively present the safety, versatility, limitations, advantages, and functional results of the first dorsal metacarpal artery flap (FDMAF) and evaluate its outcomes in thumb defect reconstruction by comparing it with the other current surgical options. METHODS: A total of 21 patients underwent thumb defect reconstruction. They were evaluated with the following parameters: Etiology, age, timing of reconstruction, flap vitality, Semmes-Weinstein monofilament (SWM) test, static two-point discrimination (2PD) test, pain, cortical reorientation, cold intolerance questionnaire, and Michigan hand outcomes questionnaire. Their functional outcomes were evaluated by comparing their scores with the other current surgical options published in the literature. RESULTS: The mean follow-up period was 22.3 months. The mean pain score of the flap was 0.4±0.6 and no patient had pain in the donor area (range, 0-10). The sensory outcome was 'good' (8.6 mm) based on the static 2PD test. The mean SWM test score was 4.02 g. Patient satisfaction was 4.6 according to the Michigan hand outcomes questionnaire (range, 0-5). The cold intolerance questionnaire scores showed that the patients had mild cold intolerance (mean, 10.5; range 0-100). Complete cortical reorientation was seen in 81% of the patients. CONCLUSION: Restoration of the innervation of thumb defects is possible with the FDMAF with high satisfaction rates based on our results. The absence of the microsurgical vessel or nerve repair facilitates the surgery, shortens its duration, and reduces morbidity, reserving the microsurgical options for more complicated cases. Therefore, it can be concluded that the FDMAF is an effective flap with great modifications for complicated thumb defects but surgeons should consider their clinical outcomes and prolonged surgery when choosing which technique to be used.

Aneurysmal Bone Cyst of Pisiform Bone: Diagnosis and Treatment of a Rare Entity: Case Report and a Review of the Literature.

Aneurysmal bone cyst (ABC) is a benign expansile bone tumor without metastasis capability. Only 3-4% of ABCs occur in the hand and they mainly take place in metaphysis' of long bones like metacarpals or phalanges. Carpal ABCs have been reported as individual case reports in the literature due to rarity. A patient presented with pain in her right wrist. Magnetic resonance imaging revealed a well circumscribed one cm sized mass in the pisiform bone that resembled an aneurysmal bone cyst. Total pisiformectomy was performed. Treatment options are total excision or curettaging in ABCs. But rarity of these lesions may delay the diagnosis process for the inexperienced surgeon.

Comparative assessment of fingertip replantation in paediatric and adult patients within a single institution.

The purpose of this retrospective study was to compare the outcomes of paediatric and adult fingertip replantation within a single institution. Our retrospective study found no significant difference in the survival rate between the paediatric (10/12) and adult (22/26) groups. At 6 months follow-up, there was no significant difference in sensory recovery between both groups, as measured with Semmes-Weinstein testing, but a significant difference in mean static two-point discrimination testing values between the paediatric (4.0 mm) and adult (6.2 mm) groups. Moreover, the mean time for regaining sensation was faster in paediatric patients (1.3 months) as compared with adult patients (4.1 months). Five children and four adults received erythrocyte transfusion. Paediatric fingertip replantation has similar survival rates, faster and better sensory nerve recovery as compared with adults despite a higher erythrocyte transfusion rate. Although technically demanding, paediatric fingertip replantation is recommended, whenever possible, because of the good outcomes achievable.Level of evidence: IV.

A hesitated approach: primary radiotherapy for keloids-a case series.

PURPOSE: To assess the efficacy and toxicity of hypofractionated radiotherapy (RT) alone in treatment-resistant symptomatic keloids. METHODS: Six patients with a total of 13 inoperable large keloid lesions and no response to previous treatments were admitted to our department between 2017 and 2019. All patients were examined for detailed wound localization, size, contour, and color assessment, and for objective and subjective symptoms. Response to treatment was graded as "complete remission" in case of full symptomatic relief and >75% decrease in lesion size, as "partial remission" in case of partial symptomatic relief and 25-75% decrease in lesion size, and as "stable disease" in case of no symptomatic relief or <25% decrease in lesion size. Patients were followed up monthly for the first 3 months and every 3 months thereafter by physical examination. RESULTS: A total dose of 37.5 Gy external RT in five fractions was prescribed by 6­MeV electrons in 4 patients and 6­MV photons in 2 patients. Complete response was obtained in all patients at the 6­month control. All patients were satisfied with cosmetic results at their last control. Grade 2 dermatitis developed in all patients during the second week of RT but resolved completely in all after 6 months following the end of RT. CONCLUSION: In keloids that are unresponsive to standard treatment, hypofractionated RT using a total dose of 37.5 Gy in five fractions is feasible.

A New Experimental Lymphedema Model: Reevaluating the Efficacy of Rat Models and Their Clinical Translation for Chronic Lymphedema Studies.

BACKGROUND: Our aim was to create a new rodent hind limb lymphedema model lacking the fibrosis effect induced by radiotherapy and subjected to the inhibition of lymphangiogenesis via sirolimus (rapamycin) to maintain a chronic lymphedema model and investigate its reliability for human treatment modalities. METHODS: Forty-two Sprague-Dawley rats were randomly assigned to 7 groups: (1) surgery control, (2) vehicle-surgery control, (3) vehicle control, (4) rapamycin control, (5) surgery with 1 mg/kg per day rapamycin, (6) surgery with 1.5 mg/kg per day rapamycin, and (7) surgery with 2 mg/kg per day rapamycin. All surgeries were performed on the right hind limbs, with the left hind limbs also considered as a control. The drug and its solvent were administered daily into the relevant groups intraperiteonally. The presence of lymphedema was investigated by weekly limb circumference measurements, microcomputed tomography, fluorescence lymphography using indocyanine green dye, and microscopic evaluation at the end of the sixth week to determine any histological changes in the hind limbs. RESULTS: In group 1, lymphedema was observed for 2 weeks (P = 0.032), whereas in groups 5, 6, and 7, lymphedema lasted for 3 weeks (P < 0.05.) Fluorescence using indocyanine green revealed that the edema was totally resolved after 6 weeks of surgery by a well-developed superficial lymphatic organization instead of the normal distinct vessel structure. Histologically, groups 1, 5, 5, and 7 demonstrated a significant increase in both the number of macrophages (P < 0.001) and newly formed lymphatic vessels in the right side surgically treated hind limb (P < 0.05). CONCLUSIONS: Despite the extreme surgical destruction and lymphangiogenesis inhibition in the rat model, the sustained lymphedema did not last >3 weeks. Because of the rapid neolymphangiogenesis in murines and a different wound healing mechanism, they should not be considered as an appropriate model for research on human lymphedema in first place.

Serum 25-hydroxyvitamin D is associated with insulin resistance independently of obesity in children ages 5-17.

AIM: To determine the association of vitamin D with insulin resistance and obesity in children. METHODS: A total of 92 obese and 58 non-obese children aged 5-17 years were evaluated. Data were collected related to anthropometric (weight, height), and biochemical parameters (fasting plasma glucose, serum insulin, serum 25-hydroxyvitamin D, lipid profile, vitamin B12, parathormone) and physical examination (blood pressure, acanthosis nigricans, stria, lipomastia). Insulin resistance (IR) was calculated using the homeostasis model assessment (HOMA). HOMA-IR = fasting insulin level (µU/ml) × fasting glucose (mg/dL)/405. A HOMA-IR value >2.5 was defined as insulin resistance. RESULTS: According to the US Endocrine Society classification, vitamin D deficiency (0-20 ng/ml) was determined at significantly higher rates in the obese group than in the control group (p < 0.001). The rate of subjects with a vitamin D level of 20-30 ng/ml was significantly lower in the obese group than in the control group (p < 0.001) Within the obese group a statistically significant difference was determined between the insulin resistant and non-insulin resistant groups in respect of serum 25-hydroxyvitamin D levels (p = 0.001) and vitamin B12 levels (p = 0.001). A significant negative correlation was determined between serum 25-hydroxyvitamin D and HOMA-IR (r=-0.256, p = 0.016) and insulin (r = -0.258, p = 0.015). The systolic blood pressure (p = 0.001) and diastolic blood pressure (p = 0.003) values were significantly different in the control and obese groups. A statistically significant difference was determined between the control and obese groups in terms of the levels of insulin, HOMA-IR, HbA1c, cortisol, LDL, total cholesterol, HDL, triglyceride, hemoglobin, MCV, MPV, and calcium. CONCLUSION: The prevalence of vitamin D deficiency was higher in obese children compared to normal-weight and overweight children. Serum 25(OH)D levels showed a negative correlation with insulin and HOMA-IR. Serum 25(OH)D is associated with insulin resistance independently of obesity.

Monozygotic twins with familial hypercholesterolemia and high lipoprotein(a) levels leading to identical cardiovascular outcomes: Case report and review of the literature.

Homozygous familial hypercholesterolemia (HoFH) is a rare, autosomal dominant disease that leads to premature cardiovascular disease (CVD). Since monozygotic twins share the intrauterine environment and have the same age and gene profile, they could represent a very special resource for the investigation of the causes and the natural course of FH. This report is a description of 36-year-old monozygotic twin brothers with almost identical early coronary artery involvement due to FH concomitant with high lipoprotein(a) (Lpa) levels and a review of the literature. Sequence analysis revealed that the twins were homozygous for the LDLR c.1060+10G>A (rs12710260) mutation and heterozygous for the LDLR c.542C>T (rs557344672) mutations. Both were also homozygous for the c.1060+7T>C (rs2738442) and c.1586+53A>G (rs1569372) mutations in the LDLR gene as well as c.4265A>T (rs568413) mutations in the APOB gene. In the literature, there are 7 twin cases with reported FH, but none with high Lpa levels. The HoFH twins in this case report had lower low-density lipoprotein (LDL) cholesterol levels than expected (before treatment 204 and 223 mg/dL), with almost identical coronary involvement. Both had an extremely high Lpa level (308 and 272 nmol/L) with a very low coronary calcium score (16 AU) and a good response to statins (>60%). There was a history of the first CVD event occurring at nearly the same age (32-34 years) in the family. This could be an important aspect of FH families as a result of the similar timing of cumulative LDL exposure exceeding the threshold of CVD events. In conclusion, this first report of monozygotic HoFH twins with elevated Lpa levels and almost identical early coronary artery involvement at the same age provides evidence to substantiate the hypothesis of lifetime cholesterol burden/exposure.

Oberg-Manske-Tonkin Classification of Congenital Upper Extremity Anomalies: The First Report From Turkey.

PURPOSE: Although the Oberg-Manske-Tonkin (OMT) classification has been recommended by the International Federation of Societies for Surgery of the Hand owing to some insufficiencies of the Swanson classification system, it has not achieved a universal adoption by hand surgeons. In this study, we hypothesize that the OMT classification can be used easily to classify congenital upper extremity anomalies. We also aim to make epidemiological analysis of congenital upper extremity anomalies with the OMT classification and to compare the applicability of the OMT and the Swanson classifications. METHODS: We retrospectively analyzed 711 patients and 833 extremities operated on between 2012 and 2017. Photographs, plain x-rays, and brief medical histories of the patients were evaluated by 4 plastic surgeons. Two independent evaluations were made by each surgeon in 1-month interval using these classification systems. RESULTS: Total number of upper extremity anomalies recorded was 1050. Of the 711 patients operated on, 122 had bilateral anomalies. The anomalies were identified in 833 extremities because many extremities had more than a single diagnosis. We were able to classify all of the anomalies within the OMT classification. The OMT classification gives better reliability results compared with the Swanson classification according to intrarater and interrater reliabilities. CONCLUSIONS: Compared with the Swanson classification system based on phenotypic evaluation of the extremity, the OMT classification system is easier to apply and the association of the anomaly with the embryologic origin during evaluation is possible. We believe that multiple studies from different centers will boost the international acceptance of the OMT classification.

A family with novel homozygous deletion mutation (c.1255delT; p.Phe419Serfs*12) in the glucokinase gene, which is a rare cause of permanent neonatal diabetes mellitus.

Heterozygous inactivating mutations in the glucokinase gene cause the mildest form of maturity-onset diabetes of the adolescents. However, homozygous or compound heterozygous mutations in the glucokinase gene are a rare cause of permanent neonatal diabetes mellitus. Herein, we present the case of a male child with permanent neonatal diabetes mellitus whose mutational analysis revealed a novel homozygous deletion mutation in the glucokinase gene. The male proband of Turkish ancestry from consanguineous parents was born at 37 weeks gestation with a birth weight of 1870 g (<3rd percentile). Hyperglycemia developed during the first postnatal day and diabetes-related autoantibodies were negative. He was put on insulin on the first day of life. Insulin has never been discontinued since then. The mother was aged 35 years and had gestational diabetes. The father and the two brothers had impaired fasting glucose. Both parents and brothers were heterozygous for this mutation.

Endoscopic retrieval of retracted flexor tendons: An atraumatic technique.

BACKGROUND: The repair of retracted flexor tendons is a challenging problem for hand surgeons. The tendon stump should be handled in an atraumatic manner because any microtrauma to the sheath and tendon can lead to poor functional outcomes. METHODS: Twenty-three patients with flexor zone 2 injuries and intraoperative finding of retracted tendons were randomly divided into two groups: endoscopic retrieval group and proximal incision group. A flexible endoscope and a flexible grasping forceps were used for endoscopic retrieval of the retracted flexor tendons. The groups were compared in terms of infection rate, neurovascular complications, regional pain, total range of active motion (TAM) and functional outcomes. RESULTS: Age, gender, average preoperative pain and general pain perception scores were similar between the two groups. We found a significantly shorter duration of operation and better pain scores at 1-2 weeks in the endoscopic group than in the other group (p = 0.002 and p = 0.020, respectively). A significant difference in TAM was demonstrated between the groups at 3 to 5 weeks (p = 0.003). CONCLUSION: The surgical procedure presented here has the advantages of direct visibility of the proximal tendon end and retrieval in an atraumatic manner, with better results and decreased morbidity. It is a promising approach and can be used as a routine procedure in retracted tendon cases.

Scroll Reconstruction: Fine Tuning of the Interface Between Middle and Lower Thirds in Rhinoplasty.

BACKGROUND: The junction between upper and lower lateral cartilages, known as "the scroll area," is an important determinant of the spatial relationship between the middle and lower nasal thirds. OBJECTIVES: We offer a graduated and reproducible surgical technique of scroll area management that takes into consideration anatomic, functional, and aesthetic relationships. METHODS: This study was a retrospective review of the recorded intraoperative information to investigate the frequency of the use of scroll reconstruction techniques and perioperative parameters that relate to unintended component alterations in the scroll area. A total of 364 consecutive primary open approach rhinoplasty cases performed by the first author (O.B.) between July 2011 and September 2015 were included in the study. RESULTS: Of the 364 cases who underwent primary open approach rhinoplasty, 329 received some form of scroll reconstruction. Scroll reconstruction was performed in all deviated cases, and 88% of straight noses. The most common technique for scroll reconstruction was overlapping repair (88.3%). As expected, both alar rim and alar crease asymmetries were very frequent in the deviated nose group, 89.4% and 75% respectively. Alar rim and alar crease asymmetries were frequent enough in the straight nose group to deserve special attention, 14.2% and 10.4% respectively. CONCLUSIONS: With careful attention to anatomic details of the scroll area, nasal tip refinements can be performed with predictable safety and accuracy. Our approach is a graduated, reproducible, and individualized way of scroll area management that aims to create the ideal cartilage configuration while preventing unintended component alterations.