Intracranial bleeding (ICB) as a catastrophic complication of Streptococcus gordonii infective endocarditis (IE) in an immunocompetent patient

We present an interesting case of late-onset intracranial bleeding (ICB) as a complication of Streptococcus gordonii causing infective endocarditis. A previously healthy young woman was diagnosed with infective endocarditis. While she was already on treatment for 2 weeks, she had developed seizures with a localising neurological sign. An urgent non-contrasted CT brain showed massive left frontoparietal intraparenchymal bleeding. Although CT angiogram showed no evidence of active bleeding or contrast blush, massive ICB secondary to vascular complication of infective endocarditis was very likely. An urgent decompressive craniectomy with clot evacuation was done immediately to release the mass effect. She completed total 6 weeks of antibiotics and had postoperative uneventful hospital stay despite having a permanent global aphasia as a sequel of the ICB.

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Rare cause of gastric outlet obstruction: xanthogranulomatous cholecystitis

Description 

A 62-year-old Indian female patient presented with right upper quadrant pain and multiple episodes of vomiting for 10 days. She denied history of fever, jaundice, past anorexia/weight loss and previous surgeries. She was dehydrated, tachycardia (120/min) was present, and there was tenderness in the right upper quadrant. Routine investigations revealed presence of anaemia (haemoglobin 9.1 g/dL), raised total leucocyte counts (17 x10^9/L) and hypokalaemia (2.8 mEq/L). All other blood tests (liver function/kidney function/blood sugar and coagulation profile) were normal. Patient was stabilised with intravenous fluids, antibiotics and other supportive management. Imaging with ultrasound (USG) was suboptimal due to the massive intestinal gas and uncooperative nature of the patient. A collapsed gallbladder (GB) and a mass with a smooth curvilinear surface and posterior acoustic shadowing in the distal part of the duodenum were the only distinguishable findings. Due to repeated episodes of vomiting, the patient underwent an upper gastrointestinal endoscopy, which was...

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Tubercular septal abscess in a postoperative patient: a novel entity

Extrapulmonary tuberculosis is a rather uncommon and potentially challenging phenomenon. Such manifestation, particularly in the scenario of a late postoperative period, is extremely rare and requires a high index of suspicion, prompt diagnosis and appropriate treatment.

We present the case scenario of a patient with history of successfully treated pulmonary tuberculosis many years earlier, without any signs of recurrence, who developed primary nasal septal tuberculosis after undergoing septoplasty to correct his septal deviation. His postoperative course remained uneventful for 4 months. He then presented with a spontaneous nasal septal abscess, which proved to be tubercular in nature on investigations. Appropriate diagnosis was established, and the patient was treated successfully with antituberculous therapy.

Recurrent tuberculosis may present a diagnostic challenge for healthcare professionals. Only a high index of suspicion, modern diagnostic tools and institution of appropriate treatment including surgical intervention as required will ensure a promising outcome.

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Penile cutaneous horn: still an enigma

Description

A 60-year-old man with history of Johanson's staged urethroplasty for pan anterior urethral stricture due to lichen sclerosus et atrophicus 3 months previously presented to us with a cutaneous horn over his glans penis. This horn was previously excised superficially, and now had recurred over the same site in the last 1 month. It was initially small in size and then gradually increased to a size of around 2.5 cm (figure 1). The patient had no documents mentioning the histopathology of previously excised horn. The patient had been circumcised in childhood. He had no history of genital malignancy or other factors that could have been implicated in cutaneous horn formation. He was managed with excision of the horn along with deep biopsy from base of the lesion to rule out any malignant pathology. The excision site healed well, and biopsy revealed benign pathology. At present, patient has...

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Unusual right internal carotid artery supraclinoid segment fenestration associated with multiple aneurysms treated with flow diversion and coiling

Description 

The supraclinoid (carotid-ophthalmic) segment of the internal carotid artery (ICA) under the Bouthillier classification is defined as the segment between the ophthalmic and posterior-communicating arteries.1 We describe an unusual congenital anomaly of the supraclinoid internal carotid artery characterised by a large fenestration of its carotid-ophthalmic segment, associated with multiple aneurysms.

A 60-year-old female patient underwent investigation for persistent headaches and was found to have what appeared to be irregular fusiform dilatation of the intracranial right internal carotid artery on 1.5 T magnetic resonance angiography (MRA) time-of-flight imaging. Cerebral digital subtraction angiography (DSA) performed to further characterise this abnormality demonstrated two discrete aneurysms, one measuring 5 mm and the other 2 mm, arising from the posterior limb of a supraclinoid ICA fenestration (figure 1A).

Figure 1

(A) Three-dimensional reconstruction of a rotational digital subtraction angiogram (DSA) demonstrating the supraclinoid right internal  carotid  artery  (ICA) fenestration....

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Drug-induced autoimmune hepatitis associated with turmeric dietary supplement use

Turmeric dietary supplement sales, which accounted for US$69 million in spending in 2016, have been increasing exponentially in the USA, making this one of the most popular botanical supplements sold in the USA. Herbal supplement use, which is generally regarded as safe by consumers, is not usually reported to healthcare providers. We reported here on a case of autoimmune hepatitis, occurring in a 71-year-old woman taking turmeric dietary supplements for the maintenance of cardiovascular health, which resolved rapidly following discontinuation of the turmeric supplements. Of particular note, turmeric use was not documented in the patient's medical records and the potential causative role of the turmeric supplementation was ultimately identified by the patient rather than the healthcare providers. To our knowledge, this is the first documented report of turmeric supplement-induced autoimmune hepatitis.

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Ivermectin-induced fixed drug eruption in an elderly Cameroonian: a case report

Cutaneous adverse reactions to medications are extremely common and display characteristic clinical morphology. A fixed drug eruption is a cutaneous adverse drug reaction due to type IV or delayed cell-mediate...

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Spontaneous reduction of acute cecal herniation through the foramen of Winslow

Abstract

This is the case of a 70-year-old woman who presented to the emergency department complaining of 2 hours of acute-onset epigastric pain. She had experienced this pain once before which had spontaneously resolved. Axial imaging demonstrated the cecum in an abnormal position within the lesser sac, as well as compression of the inferior vena cava and portal vein. She was taken emergently to the operating room for laparotomy, where a free-floating cecum and ascending colon was identified without ischemia, and a right hemicolectomy was performed. Foramen of Winslow hernias are rare internal hernias with a high rate of strangulation and bowel ischemia, requiring urgent surgical intervention. Operative treatment depends on the type of herniated viscera. Spontaneous reduction is not well documented.

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Takayasu's aorto-arteritis: Not your regular lesion for angioplasty

Navdeep Singh, Vivek Athwani, Vikas Bansal, Shaveta Kundra

Annals of Pediatric Cardiology 2018 11(3):312-314

We report a case of a 6-year-old female child with Takayasu's aorto-arteritis (TA) with severe coarctation of the aorta which resulted in an aortic dissection post-ballooning. This happened despite ensuring that markers for disease activity were negative, with appropriate corticosteroid therapy started before the procedure, and using a low-profile, low-pressure, and slightly undersized balloon for dilating the stenotic segment. It required immediate endovascular stenting to tide over the crisis. Following the procedure, she became normotensive with well-palpable lower limb pulses.

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Repaired congenital heart disease and our social milieu

Krishna S Iyer

Annals of Pediatric Cardiology 2018 11(3):233-234



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Is transposition a defect of laterality?

Robert H Anderson, John Stickley

Annals of Pediatric Cardiology 2018 11(3):235-236



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Anomalous origin of the right coronary artery from the pulmonary artery associated with an aortopulmonary window

Padebettu Subramanya Seetharama Bhat, Chandana Nirmala Chandrashekar, Divya Mallikarjun, SL Girish Gowda

Annals of Pediatric Cardiology 2018 11(3):325-327

Anomalous origin of the right coronary artery (RCA) from the pulmonary artery is a rare entity that causes chronic left-to-right shunting of blood from the RCA, through the coronary collaterals into the pulmonary artery. This results in persistent myocardial ischemia and ventricular dysfunction. Association of this anomaly with an aortopulmonary window worsens the condition further due to an additional shunt. We encountered a combination of these two anomalies along with an ostium secundum atrial septal defect in a 10-month-old baby who presented with excessive crying and failure to thrive. Evaluation by transthoracic echocardiography and cardiac computed tomography scan confirmed the diagnosis. Intrapulmonary baffle was done using a pericardial patch to connect the RCA origin to ascending aorta through the aortopulmonary window, similar to Takeuchi procedure. Concomitantly, ostium secundum atrial septal defect was also closed.

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Transposition of the great arteries: A laterality defect in the group of heterotaxy syndromes or an outflow tract malformation?

Rana S Al-Zahrani, Samaher H Alharbi, Rawan M A Tuwaijri, Bayan T Alzomaili, Alaa Althubaiti, Talat Mesud Yelbuz

Annals of Pediatric Cardiology 2018 11(3):237-249

Background/Aim: Transposition of the great arteries (TGA) is traditionally classified as a "conotruncal heart defect", implying that TGA evolves from abnormal development of the outflow tract (OFT) of the embryonic heart. However, recently published genetic data suggest that TGA may be linked to laterality gene defects rather than OFT gene defects. The aim of our study was to clarify whether there is any statistically significant link between TGA and clinically diagnosed laterality defects (heterotaxy). Methods: Retrospective cross-sectional analysis of 533 patients diagnosed with TGA at our cardiac center over a period of 13 years (2002-2015). Hospital informatics and digital data recording systems were used for collecting patients' data and all patients were reviewed to check the echocardiograms for verification of the diagnosis, type (TGA, congenitally corrected TGA (ccTGA), and levo-position of the great arteries (LGA)), complexity of TGA, and all other variables (e.g., abdominal organ arrangement, cardiac position, presence or absence of other cardiac defects). Results: Of 533 TGA patients, 495 (92.9%) had the usual arrangement of the internal organs, 21 (3.9%) had mirror-imagery, 7 (1.3%) had left and 10 (1.8%) had right isomerism. 444 (83.3%) patients had TGA. The number of patients who had usual visceral arrangement in each TGA type was: 418 (94.1%) in TGA, 49 (92.4%) in ccTGA, and 28 (77.7%) in LGA. 6 (1.4%) TGA patients, 4 (11.1%) patients with LGA were found to have right isomerism, while no ccTGA patient presented with this asymmetry. 4 (0.9%) TGA patients, 1 (1.9%) ccTGA patient and 2 (5.6%) patients with LGA had left isomerism. Heterotaxy (mirror-imagery, left and right isomerism) was more associated with LGA than TGA or ccTGA with a statistically significant difference (P value of 0.001). Conclusion: In contrast to recently published genetic data, our morphological data do not disclose a significant link between TGA and heterotaxy.

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Endovascular management of vein of Galen aneurysmal malformation in a neonate

Siddharthan Deepti, Rajnish Juneja, Leve Joseph Devarajan Sebastian

Annals of Pediatric Cardiology 2018 11(3):304-307

A term baby presented on the 7th day of life in cardiogenic shock due to vein of Galen aneurysmal malformation. A successful embolization of the malformation was performed through transarterial route on day 12 of life after a period of initial stabilization.

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Validation of the accuracy of handheld echocardiography for diagnosis of congenital heart disease

Sulafa Ali, Tajudeen Bushari

Annals of Pediatric Cardiology 2018 11(3):250-254

Background: Handheld echocardiography (HHE) has been increasingly used for rheumatic heart disease screening and in adult emergency room settings. Aims: This study aimed to validate the accuracy of HHE in the diagnosis of congenital heart disease (CHD). Settings and Design Methodology: This is a prospective study carried out at three pediatric cardiology outpatient clinics in Khartoum, Sudan. All patients with suspected CHD were evaluated by clinical examination followed by HHE, performed using a modified segmental approach. Then, a complete study was performed using a standard echocardiography machine. The results were then compared using appropriate statistical tools. Results: A total of eighty cases were included with the following diagnoses either in isolation or combination: ventricular septal defect (n = 23), atrial septal defect (n = 10), pulmonary stenosis (n = 7), tetralogy of Fallot (n = 7), patent ductus arteriosus (n = 6), atrioventricular septal defect (n = 6), transposition of the great arteries (n = 6), and other diagnoses (n = 15). Agreement between HHE and SE was excellent both for visualizing heart segments (κ =77%–100% with a mean of 92.9%) and for diagnosis of CHD (κ =66%–100% with a mean of 91.7%). The sensitivity of HHE was 69.2%–100% (mean = 90.2%) and specificity was 98.5%–100% (mean = 99.3%). Conclusion: This study supports extending the utility of HHE in children for screening of CHD in addition to its current role in rheumatic heart disease screening.

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Neonatal supraventricular extrasystole as early clinical debut of cardiac rhabdomyoma

Roger Esmel-Vilomara, Amparo Castellote, Luz Santana, Dimpna Calila Albert

Annals of Pediatric Cardiology 2018 11(3):318-321

We are reporting the case of a newborn with a diagnosis of frequent supraventricular extrasystoles, up to 25% of beats at Holter monitoring, and partial response to beta-blockers. Initial echocardiographic studies were normal until the identification of a right atrial mass at 4 months of life. Given the progressive growth of the mass and the suspicion of myxoma or thrombus in the magnetic resonance study, surgical resection of the tumor was performed. The surgical specimen was histologically diagnostic of rhabdomyoma. Currently, the patient remains asymptomatic after a 6-year follow-up period. A single rhabdomyoma is described, located in an atypical situation, near the crista terminalis, and diagnosed from frequent extrasystoles which appeared before the echocardiographic resolution was able to identify it. Magnetic resonance showed nondiagnostic tissue enhancement characteristics.

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Predictors of abnormal electrocardiograms in the pediatric emergency department

Shiv Gandhi, Miranda Lin, Sharon R Smith, Jesse J Sturm

Annals of Pediatric Cardiology 2018 11(3):255-260

Background: Electrocardiograms (ECGs) are ordered in the pediatric emergency room for a wide variety of chief complaints. Objectives: Criteria are lacking as to when physicians should obtain ECGs. This study uses a large retrospective cohort of 880 pediatric emergency department (ED) patients to highlight objective criteria including significant medical history and specific vital sign abnormalities to guide clinicians as to which patients might have an abnormal ECG. Methods: Retrospective review of Pediatric ED charts in all patients aged < 18 years who had ECG performed during ED stay. Pediatric ED physician interpretation of the ECG, clinical data on vital signs and past medical history was collected from the medical record for analysis. Results: Of 880 ECGs performed in the ED, 17.4% were abnormal. When controlled for medical history and demographic differences, abnormal ECGs were associated with age-adjusted abnormal ED vital signs including increased heart rate (odds ratio [OR] 1.85, 95% confidence interval [CI] 1.1–3.09) and increased respiratory rate (OR 1.74, CI 1.42–2.62). In a logistic regression analysis, certain chief complaints and history components were less likely to have abnormal ECGs including complaints of chest pain (OR 0.38, CI 0.18–0.80) and known history of gastrointestinal or respiratory condition (i.e., asthma) (OR 0.48, CI 0.29–0.79). Conclusions: In this cohort of patients, those with a chief complaint of chest pain or known respiratory conditions and normal age-adjusted vital signs in the ED have low likelihood of an abnormal ECG.

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Professor Ivatury Mritunjay Rao

Krishna Subramony Iyer

Annals of Pediatric Cardiology 2018 11(3):330-331



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Native aortic coarctation in neonates and infants: Immediate and midterm outcomes with balloon angioplasty and surgery

Supratim Sen, Sandeep Garg, Suresh G Rao, Snehal Kulkarni

Annals of Pediatric Cardiology 2018 11(3):261-266

Background: Native aortic coarctation in young infants can be treated surgically or with balloon angioplasty, although the latter approach remains controversial. We sought to compare midterm outcomes of balloon and surgical coarctoplasty at our center and to review the current practices in literature. Methods: The study design was a retrospective record review. Patients with coarctation aged <1 year who underwent balloon or surgical coarctoplasty at our center (January 2010–January 2016) with >6-month post-procedure follow-up were studied. Patients with significant arch hypoplasia or interruption, long-segment coarctation, and associated intracardiac lesions were excluded from the study. Clinical, echocardiographic, and procedural details were recorded at initial presentation and follow-up. Need for reintervention was noted. Results: Out of a total of 75 patients, 28 underwent balloon (Group A) and 47 underwent surgical coarctoplasty (Group B). There were two deaths in Group B (mortality 4.2%) and none in Group A. In neonates, 63.6% of Group A and 17.4% of Group B patients required reintervention (P = 0.007). Beyond the neonatal age, 29.4% of Group A and 41.6% of Group B patients required reintervention (not statistically significant). On univariate analysis, residual coarctation on predischarge echocardiogram was the only significant predictor of reintervention (P = 0.04). On Kaplan–Meier analysis, with neonatal presentation, freedom from reintervention in Group B was significantly higher than Group A (P = 0.028). Conclusion: In neonates with native aortic coarctation, surgery unequivocally yields better immediate and midterm results and is the preferred treatment modality. In older infants, balloon coarctoplasty has good early and midterm outcomes with acceptable reintervention rates and should be considered a viable alternative to surgery, especially in critical patients with high surgical risk.

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Successful Angiojet® aortic thrombectomy of extracorporeal membrane oxygenation-related thrombus in a newborn

Maria Elena Gutierrez, Jeffrey A Alten, Mark A Law

Annals of Pediatric Cardiology 2018 11(3):300-303

Thrombosis and systemic embolization are important complications of extracorporeal membrane oxygenation (ECMO). We present a 2.5 kg neonate born at 37.4 weeks with hypoplastic left heart supported on ECMO that developed an acute, occlusive distal aortic thrombus that was emergently managed by transcatheter Angiojet® (Boston Scientific, Boston, MA) thrombectomy. The procedure successfully restored perfusion to the lower extremities with sustained result upon 1-week follow-up. This case highlights the Angiojet® thrombectomy as a treatment option for limb- or organ-threatening acute thrombus in even the most complex ECMO patients.

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Early outcomes of modification of end to side repair of coarctation of aorta with arch hypoplasia in neonates and infants

Anil Kumar Dharmapuram, Nagarajan Ramadoss, Sudeep Verma, Goutami Vejendla, Rao Mrutyunjaya Ivatury

Annals of Pediatric Cardiology 2018 11(3):267-274

Background: In coarctation of aorta associated with proximal arch hypoplasia, extended end-to-end anastomosis through a thoracotomy would result in a residual gradient between the origins of the innominate and the left common carotid arteries. To eliminate this, we modified the surgical technique. Patients and Methods: Between March 2012 and May 2017, 50 patients (14 neonates) underwent repair of coarctation of aorta through a thoracotomy. The age ranged from 6 days to 2 years (median 2 months) and the weight from 1.8 to 8.0 kg (median 4.3 kg). A total of 15 patients (Group A) underwent repair by the extended end-to-end anastomosis. Among them, two patients developed early restenosis at the proximal arch requiring surgical reintervention. Hence, in the second half of the study, 35 patients (Group B) who were identified to have significant hypoplasia of the proximal arch underwent a modified end-to-side anastomosis of the descending aorta to the proximal arch incorporating the distal ascending aorta in the anastomosis and leaving the left subclavian artery end of the isthmus as an end-on vessel. Results: One neonate in Group B died due to a cause not related to the repair. All the other patients in Group B are doing well without a residual gradient during a median follow-up of 23 months. There were no airway issues related to extensive mobilization of the aorta. Conclusion: End-to-side anastomosis of the descending aorta to the proximal arch and side of the ascending aorta is possible through a thoracotomy and can be achieved with good outcome in neonates and infants.

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Morphology of intramural ventricular septal defects: Clinical imaging and autopsy correlation

Neil D Patel, Richard W Kim, Suwanna Pornrattanarungsi, Pierre C Wong

Annals of Pediatric Cardiology 2018 11(3):308-311

An intramural ventricular septal defect (IVSD) is a type of interventricular communication that can occur following biventricular repair of a conotruncal malformation. There have been no previous reports depicting the actual pathologic anatomy of this defect. We describe two cases of IVSDs with their clinical imaging and postmortem pathology.

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Modified technique for uncommon coarctation of the aorta with arch hypoplasia

Brijesh Parayaru Kottayil, Praveen R Bayya, Luis Baquero, Amitabh C Sen, Raman K Kumar

Annals of Pediatric Cardiology 2018 11(3):275-277

A true presubclavian coarctation of the aorta with arch hypoplasia without major intracardiac anomaly can result in a surgical dilemma. Theoretically, one can avoid repair through median sternotomy using selective cerebral perfusion and its sequelae since no major intracardiac repair is needed. Repair through thoracotomy is technically challenging if arch reconstruction is required and precise surgical planning is required to avoid spinal hypoperfusion and to maintain cerebral perfusion. Moreover, inadequate repair often has resulted in hypoplastic aortic arch or residual coarctation on follow-up requiring future intervention. We have employed a modified surgical repair through thoracotomy avoiding cardiopulmonary bypass to address presubclavian coarctation of the aorta with diffuse arch hypoplasia with theoretically less chance for future stenosis.

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Balloon angioplasty for supravalvular aortic stenosis as an early complication following arterial switch operation

Kaitlin Carr, Osamah Aldoss, Bijoy Thattaliyath, Manish Bansal

Annals of Pediatric Cardiology 2018 11(3):315-317

Supravalvular aortic stenosis as an early complication of transposition of the great artery repair is rare with few cases reported. Furthermore, transcatheter intervention is uncommon as surgical re-intervention has been traditionally done. We describe two cases of supravalvular aortic stenosis at the anastomotic site as an early complication of the arterial switch operation. Both patients underwent balloon angioplasty of the supravalvular aorta with improvement in postangioplasty gradients and angiographic appearance. Both patients at short-term follow-up had persistent improved gradient without need for further intervention.

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Descending aortic translocation procedure for relief of bronchial compression in left aortic arch and right descending aorta

Roy Varghese, Ronak Sheth, Sivakumar Kothandam

Annals of Pediatric Cardiology 2018 11(3):278-281

Compression of the left bronchus by a right-sided descending aorta arising from a left aortic arch constitutes an exceedingly rare form of vascular ring. This manuscript describes the surgical technique that involves translocation of the descending aorta directly to the proximal ascending aorta, thereby relieving the bronchial compression.

https://ift.tt/2MdpI08

Total anomalous pulmonary venous connection masking an aortopulmonary window: A rare combination of defects

Shyamajit Samaddar, Dheeraj Bhatt, Munish Guleria, Dinesh Kumar Yadav, Anubhav Gupta

Annals of Pediatric Cardiology 2018 11(3):322-324

The association of aortopulmonary (AP) window with total anomalous pulmonary venous connection (TAPVC) has so far not been reported. We report a unique case of an 8-month-old child who presented with congestive cardiac failure and severe pulmonary arterial hypertension. Initial echocardiography revealed supracardiac TAPVC. Cardiac computed tomography showed the presence of Type I AP window along with the TAPVC. In the presence of severe pulmonary hypertension and dilated right ventricle, AP window may easily be missed if not actively looked for.

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Rationale and design of long-term outcomes and vascular evaluation after successful coarctation of the aorta treatment study

Jose D Martins, Justin Zachariah, Elif Seda Selamet Tierney, Uyen Truong, Shaine A Morris, Shelby Kutty, Sarah D de Ferranti, Jonathan Rhodes, Marta Antonio, Maria Guarino, Boban Thomas, Diana Oliveira, Kimberlee Gauvreau, Nuno Jalles, Tal Geva, Miguel Carmo, Ashwin Prakash

Annals of Pediatric Cardiology 2018 11(3):282-296

Background: Coarctation of the aorta (CoA) can be treated using surgery, balloon angioplasty, or stent implantation. Although short-term results are excellent with all three treatment modalities, long-term cardiovascular (CV) morbidity and mortality remain high, likely due to persistently abnormal vascular function. The effects of treatment modality on long-term vascular function remain uncharacterized. The goal of this study is to assess vascular function in this patient population for comparison among the treatment modalities. Methods: We will prospectively assess vascular Afunction in large and small arteries fusing multiple noninvasive modalities and compare the results among the three groups of CoA patients previously treated using surgery, balloon angioplasty, or stent implantation after frequency matching for confounding variables. A comprehensive vascular function assessment protocol has been created to be used in 7 centers. Our primary outcome is arterial stiffness measured by arterial tonometry. Inclusion and exclusion criteria have been carefully established after consideration of several potential confounders. Sample size has been calculated for the primary outcome variable. Conclusion: Treatment modalities for CoA may have distinct impact on large and small arterial vascular function. The results of this study will help identify the treatment modality that is associated with the most optimal level of vascular function, which, in the long term, may reduce CV risk.

https://ift.tt/2Mgkfpe

Coronary-to-pulmonary artery collaterals in pulmonary atresia

Deepa Sasikumar, Bijulal Sasidharan, Anoop Ayyappan, Arun Gopalakrishnan, Kavasseri M Krishnamoorthy

Annals of Pediatric Cardiology 2018 11(3):328-329

A 2-month-old baby with ventricular septal defect and pulmonary atresia was found to have coronary-to-pulmonary artery collaterals. Cardiac computed tomography confirmed the coronary collaterals and showed the absence of other systemic to pulmonary artery collaterals. Although these collaterals do not cause coronary ischemia, it is important to delineate them by accurate imaging to plan the appropriate surgical strategy.

https://ift.tt/2NtU96U

Haploidentical Stem Cell Transplantation: A Gateway to Infrequent Availability of HLA-Matched Related Donors

Haploidentical stem cell transplantation provides a plausible alternative for the patients when a fully matched donor is unavailable. Historically, the decision of considering haploidentical transplant has remained elusive; however, with the recent advances, the consideration of haploidentical grafts as a treatment option has become more apparent for both allografting for diseases and engraftment failure. We are reporting here an anecdotal case of a successful haploidentical engraftment in a patient with the prior graft failure of an HLA-matched related donor. Since the patient was severely alloimmunized, desensitization protocol was utilized before the haploidentical transplant, and the patient after 8 months of her second allogeneic transplantation is doing great with successful engraftment, no relapse, and no graft-versus-host disease (GVHD). Numerous reports pertinent to haploidentical graft have shown favorable outcomes in the graft placement, a decline in the rate of GVHD, and an improvement in the morbidity and mortality in affected individuals. Based on the current reports, haploidentical transplantation might be more feasible and has meaningful implications in the situations where matched donors are infrequent.

https://ift.tt/2x2s8K8

Long-Term Safety and Efficacy of Minimally Invasive Lumbar Decompression Procedure for the Treatment of Lumbar Spinal Stenosis With Neurogenic Claudication: 2-Year Results of MiDAS ENCORE

Background and Objectives This study evaluated the long-term durability of the minimally invasive lumbar decompression (MILD) procedure in terms of functional improvement and pain reduction for patients with lumbar spinal stenosis and neurogenic claudication due to hypertrophic ligamentum flavum. This is a report of 2-year follow-up for MILD study patients. Methods This prospective, multicenter, randomized controlled clinical study compared outcomes for 143 patients treated with MILD versus 131 treated with epidural steroid injections. Follow-up occurred at 6 months and at 1 year for the randomized phase and at 2 years for MILD subjects only. Oswestry Disability Index, Numeric Pain Rating Scale, and Zurich Claudication Questionnaire were used to evaluate function and pain. Safety was evaluated by assessing incidence of device-/procedure-related adverse events. Results All outcome measures demonstrated clinically meaningful and statistically significant improvement from baseline through 6-month, 1-year, and 2-year follow-ups. At 2 years, Oswestry Disability Index improved by 22.7 points, Numeric Pain Rating Scale improved by 3.6 points, and Zurich Claudication Questionnaire symptom severity and physical function domains improved by 1.0 and 0.8 points, respectively. There were no serious device-/procedure-related adverse events, and 1.3% experienced a device-/procedure-related adverse event. Conclusions MILD showed excellent long-term durability, and there was no evidence of spinal instability through 2-year follow-up. Reoperation and spinal fracture rates are lower, and safety is higher for MILD versus other lumbar spine interventions, including interspinous spacers, surgical decompression, and spinal fusion. Given the minimally invasive nature of this procedure, its robust success rate, and durability of outcomes, MILD is an excellent choice for first-line therapy for select patients with central spinal stenosis suffering from neurogenic claudication symptoms with hypertrophic ligamentum flavum. Clinical Trial Registration This study was registered at ClinicalTrials.gov, identifier NCT02093520. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Accepted for publication June 8, 2018. Address correspondence to: Peter S. Staats, MD, MBA, National Spine and Pain Centers, 170 Avenue at the Common, Ste 6, Shrewsbury, NJ 07702 (e-mail: peterstaats@hotmail.com). P.S.S. and R.M.B. are study principal investigators for MiDAS ENCORE. In this role, they have been responsible for study oversight. Responsibilities include protocol review, assistance with site selection, site investigator support, oversight of patient enrollment and protocol compliance, and adjudication of adverse events. This trial was sponsored by Vertos Medical. The sponsorship includes study-related supplies and expenses, as well as funding for statistical analysis services by an independent provider. The authors declare no conflict of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.rapm.org). Copyright © 2018 by American Society of Regional Anesthesia and Pain Medicine.

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Best perioperative practice in management of ambulatory patients with obstructive sleep apnea

Purpose of review The perioperative outcome in obstructive sleep apnea (OSA) patients undergoing ambulatory surgery can be potentially impacted by the type of anesthetic technique, fluid management and choice of anesthetic agents. This review highlights the best perioperative practices in the management of OSA patients undergoing ambulatory surgical procedures. Recent findings A recent meta-analysis found that STOP-Bang might be used as a perioperative risk stratification tool. Patients with high-risk OSA (STOP-Bang ≥3) were found to be associated with an increased risk of postoperative complications and prolonged length of hospital stay compared with low-risk OSA (STOP-Bang 0-2) patients undergoing noncardiac surgical procedures. A bidirectional relationship exists between OSA and difficult airway. Both suspected or diagnosed OSA may be associated with either difficult intubation or difficult mask ventilation or both. A recent meta-analysis identified OSA as an important risk factor for opioid-induced respiratory depression. A dose–response relationship was shown between the morphine equivalent daily dose and death or near-death events in OSA patients undergoing surgery. Postoperative continuous monitoring is recommended for high-risk OSA patients receiving opioids. Minimising the dose of muscle relaxant, neuromuscular monitoring and ensuring complete reversal of neuromuscular blockade before extubation is essential in OSA patients to avoid postoperative complications. Whenever feasible, regional anesthesia with multimodal analgesia may be considered as a better alternative to general anesthesia in OSA patients. Summary Patients with OSA and associated comorbidities present a challenge to anesthesiologists as they are at a high risk of perioperative complications. It is important to identify patients with OSA, with the goal to raise awareness among providers, mitigate risk and improve outcomes. Correspondence to Frances Chung, Professor, Department of Anesthesia, Toronto Western Hospital, University Health Network, 399 Bathurst Street, MCL 2-405, Toronto, ON M5T 2S8, Canada. E-mail: Frances.chung@uhn.ca Copyright © 2018 YEAR Wolters Kluwer Health, Inc. All rights reserved.

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