The extranasal glioma - a cause of neonatal respiratory distress

Normal brain tissue in an abnonnal configuration, occurring at a site away from the cranial cavity, is temied an Extranasal Glioma. Since its first description in 1852 by Reid2, only 140 patients with this entity have been reported. Heterotopic brain tissue when present in a confined area may cause obstmction, pressure and pain. Its occurrence in the nasopharyngeal region is relatively rare and only 19 cases have been reported so far2, the majority presenting with respiratory distress in the early neonatal period. The objective of this report is to create an awareness amongst the physicians of the presentation and management of this condition

Malignant pheochromocytoma with widespread bony and pulmonary metastases

Pheochromocytoma is a rare benign tumor of the adrenal gland. A select few cases may be malignant, and metastatic cases are exceedingly rare. It often presents with symptoms of catecholamine excess, such as sweating, palpitations, headaches, and characteristic paroxysmal hypertension. Due to its diffuse symptoms, it is difficult to diagnose and is often diagnosed late. We describe the unique case of a 44-year-old female patient who presented with uncontrolled hypertension and vomiting, accompanied by lower back pain. She was diagnosed with malignant pheochromocytoma with multiple metastases to the lungs, vertebrae, scapulae, and skull. Because of the advanced state of her disease, the patient was started on treatment with the chemotherapeutic combination of cyclophosphamide, vincristine, and dacarbazine. However, she had a complicated hospital course and died because of aspiration pneumonia and sepsis

Surgical outcomes of pediatric patients with ventricular septal defects in a tertiary referral center in Pakistan: a retrospective cohort study

Background: The nature of complications and outcomes for Ventricular septal defect (VSD) repair differ between developed and developing countries. Understanding these differences can help optimize patient management according to geographical location.Objective: The purpose of this study was to investigate the risk factors associated with in-hospital peri- and post-surgical complications of VSD repair in a developing country.Design: A retrospective cohort study. Setting: A tertiary referral center. Participants: 117 patients under 18 years of age admitted for surgical closure of ventricular septal defect between July 1998 and June 2008.Main Outcomes: Only patients with isolated VSD or VSD associated with acyanotic congenital heart disease were included. Outcomes were defined as in-hospital minor and major complications and mortality.Results: Adverse complications occurred in 35.9% (42/117) and death in 3.4% (4/117) of cases. Age \u3e12 months (OR 0.17 13 months-5 years; 0.10 5-18 years), weight \u3e10 kg (OR 0.24 11-20 kg; 0.13\u3e21 kg), and absence of pulmonary artery hypertension (OR 0.43) were all significantly associated with a reduced rate of adverse events. A longer stay in intensive care unit/semi-intensive care unit (OR 11.6 5-7 days; OR 6.1 \u3e8 days) and larger size of ventricular septal defect (OR 5.4 medium size; 3.9 large size) were associated with an increased risk of adverse events. Infection (20%) and pneumonia (10%) were the commonest complications. Conclusions and relevance: Age under one year, weight less than ten kilograms, pulmonary artery hypertension, and moderate to large size ventricular septal defect are more likely to be associated with adverse outcomes after surgical repair of ventricular septal defect. Infection and pneumonia comprise almost one third of the total complications, compared to higher arrhythmic events in developed countries. Developing countries have a distinct spectrum of complications that may be avoidable and treatable

Single lung ventilation in a young child for repair of coarctation of aorta

Single lung ventilation in small children is extremely challenging. In this case, a paediatric bronchial blocker was successfully inserted in a 19-month-old child to provide single lung ventilation using a modified insertion technique. It provided excellent working conditions during thoracotomy and our method of insertion may help in reducing the cost

Sinus of valsalva aneurysm rupturing into main pulmonary artery: A rare paediatric cardiac emergency

Congenital Sinus of Valsalva aneurysm is a rare congenital cardiac disease. Most common site of origin is the right sinus. It ruptures into right ventricle or right atrium most of the times. Only in less than 2% of the cases it ruptures into the pulmonary artery. We report a rare case of right sinus of valsalva aneurysm rupturing into pulmonary artery

Kawashima operation: functional modification of bidirectional Glen shunt with left superior vena cava in single ventricular morphology

Single ventricle physiology offers limited options with significant morbidity and mortality. The Glenn shunt is a mid-stage procedure for the Fontan circulation. With persistent left Superior Vena Cava (SVC) and Azygos continuation of the Inferior Vena Cava (IVC) to the SVC, needs the development of bilateral bidirectional Glenn shunts. Fifteen patients underwent Bidirectional Glen shunt procedure over a period of ten years. Of these two were found to have left SVC along with interrupted IVC and its Azygos continuation to the SVC. These two patients underwent the Kawashima operation. A seven years old girl was admitted via emergency with severe cyanosis. She had tricuspid atresia, azygos continuation of IVC with single ventricle physiology. She had a complicated postoperative course with prolonged hospital stay after bilateral bidirectional Glen shunt (Kawashima operation). She successfully has completed Fontan after 5 years. The second case ofa 7 year old girl had elective surgery for DORV (double outlet right ventricle), pulmonary atresia and azygos continuation of IVC and persistent left SVC. She had an uneventful postoperative course. The Kawashima operation is an established surgical procedure to deal with bilateral SVCs. The procedure is safe; outcome is favourable and prepares the patient for total cavo-pulmonary shunt

Accuracy of echocardiography in diagnosing total anomalous pulmonary venous return

Objective: Total anomalous pulmonary venous return is an uncommon cyanotic congenital heart defect. Echocardiography is the initial diagnostic tool. Complimentary non-invasive modalities like cardiac computerized tomographic angiography and cardiac magnetic resonance imaging have replaced the need for cardiac catheterization in difficult cases. This study aimed to determine the accuracy of echocardiography in diagnosing total anomalous pulmonary venous return, and to determine the factors that may decrease its sensitivity.Methods: This was a cross-sectional study conducted at the Aga Khan University Hospital Karachi, Pakistan from January 2010 to August 2016. All patients who were diagnosed with Total anomalous pulmonary venous return on echocardiography and had subsequent confirmation either on cardiac CT angiography or surgery were included. The diagnostic accuracy of echocardiography was expressed as sensitivity. Previously described taxonomy was used to define diagnostic error. Univariate and multivariate analysis were done by logistic regression OR (95% CI) were reported to identify factors causing the diagnostic error.Results: High diagnostic sensitivity (81%) was found in isolated total anomalous pulmonary venous return and low (27%) in heterotaxy and mixed (20%) varieties. Poor acoustic windows and right isomerism were found to be significant factors responsible for the diagnostic error on multivariate analysis.Conclusion: Echocardiography can diagnose isolated total anomalous pulmonary venous return with high accuracy. Use of additional modalities may be required for a complete diagnosis in cases with mixed variety, heterotaxy and poor acoustic windows

Is early correction of congenital ventricular septal defect a better option in a developing country

OBJECTIVE: To assess and evaluate the effectiveness of early ventricular septal defect (VSD) repair in a developing country by comparing the outcomes in two age groups. METHODS: A total of 39 patients underwent VSD repair at a tertiary care hospital in Karachi over a period of 2.5 years. Patients were divided into Group 1 (years) and Group 2 (2-18 yrs). Perioperative and postoperative variables were reviewed retrospectively for each patient and compared with the development of complications and the effect on postoperative recovery times. The data was analyzed using Mann Whitney test for continuous variables and Fischer\u27s exact test for categorical variables. RESULTS: Mean age at surgery was 10.1 +/- 7.0 months and 108.5 +/- 54.0 months for both groups, respectively. Two patients died. Procedure time was not affected by decreasing age (p = 0.622) but Cardio pulmonary Bypass duration (p = 0.040) and Aortic Cross Clamp time (p = 0.063) were significantly affected. No associated significance was obtained when hospital stay (p = 0.085) was observed. Increased duration of ventilation (p = 0.000) and length of ICU stay (p = 0.003) were highly significant for the younger age group. Presence of residual VSD (p = 0.025) was also significantly affected by lower age. There was no significant difference in the number of patients with complications (p = 1.000) among the 2 groups. CONCLUSION: Age does not increase morbidity rates after VSD repair although postoperative recovery times are increased with decreasing age. Hence, there is no justification in delaying VSD repair

Conversion of cardiac bypass into an extracorporeal membrane oxygenation circuit: a case from Pakistan

A 35 days old neonate with d-loop transposition of great arteries, underwent an arterial switch operation following which he developed hypotension attributed to left ventricular failure. During cardiopulmonary resuscitation decision was made to place him on cardiac bypass again. Due to limited resources and unavailability of a specialized extracorporeal membrane oxygenator machine, the CPB was modified and converted an ECMO. The neonate was successfully decannulated after 72 hours and discharged home after 3 weeks of the operation without any sequel. ECMO is a viable option in developing countries and may help in improving the outcome especially in neonatal congenital heart disease

Tetralogy of Fallot with absent pulmonary valve syndrome; appropriate surgical strategies

Abstract OBJECTIVE: To evaluate patients presenting with Tetralogy of Fallot with absent pulmonary valve syndrome to a tertiary care hospital and their surgical management. METHODS: The retrospective study was conducted at Congenital Cardiac Services, Aga Khan University Hospital, Karachi, Pakistan, and comprised data of Tetralogy of Fallot patients between April 2007 and June 2012. Data was analysed together with follow-up echocardiography. Variables assessed included demographics, imaging, operative technique, complications, post-operative recovery and follow-up echocardiography. SPSS 17 was used for statistical analysis. RESULTS: Of the 204 patients, 6 (3%) had undergone surgical correction for Tetralogy of Fallot with absent pulmonary valve syndrome. All 6(100%) patients underwent complete repair. Median age for surgery was 8.5 years (range: 0.5-29 years). Of the different surgical strategies used, Contegra and Bioprosthetic valve placement had satisfactory outcome with minimal gradient at Right Ventricular Outflow Tract, good ventricular function and mild valvular regurgitation. One (16.6%) patient with Trans Annular Patch developed post-operative Right Ventricle Outflow Tract gradient of 80mmHg with moderate pulmonary regurgitation. One (16.6%) patient with monocusp valve developed free pulmonary regurgitation at 6 months. The other 4(66.6%) patients are currently free from any complications or re-intervention. CONCLUSIONS: Early surgery is preferred in symptomatic patients. The repair depends upon achieving integrity of pulmonary circulation which is best achieved by using right ventricle to pulmonary artery conduit or inserting a pulmonary valve