Pulmonary atresia and ventricular septal defect with collaterals to right lung associated with anomalous left pulmonary artery from the ascending aorta.

We present a 10-month-old boy with cyanosis. This is a rare case of pulmonary atresia, ventricular septal defect (VSD), major aorto-pulmonary collateral arteries (MAPCAs) to the right lung with absent native right pulmonary artery (RPA) in association with anomalous left pulmonary artery (LPA) from the ascending aorta (AAo). Echocardiography was unable to identify all of the cardiovascular abnormalities. Multidetector CT demonstrated all of these abnormalities and is the investigation of choice instead of cardiac catheterization.

Acute suppurative thyroiditis due to pyriform sinus fistula: a case report.

Congenital pyriform sinus fistula is an unusual developmental abnormality, but it is the most common cause of acute suppurative thyroiditis or recurrent lateral neck infection in pediatric group. It is often overlooked because of poor diagnostic roles. Further investigations are important for diagnosis and evaluation. The authors herein present a case of pyriform sinus fistula and diagnostic roles to confirm the diagnosis.

Presacral fibrosarcoma in childhood: a case report.

Presacral fibrosarcoma is a rare malignant tumor in childhood. Because of its rarity in the presacral region, it is often missed from the differential diagnosis of presacral mass in childhood. The authors present a case of large presacral fibrosarcoma involving spinal canal, with an initial presentation of back pain and abnormal gaiting.

Inflammatory bowel disease in Thai children: presentations and outcomes of treatment.

Inflammatory bowel disease (IBD) is characterized by idiopathic chronic intestinal inflammation, due to abnormalities in gastrointestinal immunoregulation. Pediatric IBD has been rarely reported in Thailand. We describe eight children, five girls and three boys, who were diagnosed with IBD at Ramathibodi Hospital during 1999-2005 and had a follow-up of more than one year. Four cases had Crohn's disease (CD) and four cases had ulcerative colitis (UC). The ages at diagnosis ranged from 3.5 to 15.5 years. Diagnosis of IBD was delayed for more than 12 months in five patients. Five out of eight patients had early onset of disease, before 6 years of age. The manifestations included chronic diarrhea, abdominal pain, rectal bleeding and perianal lesions. The common extraintestinal manifestations were oral ulcer, anemia, weight loss and failure to thrive. Most patients had moderate to severe diseases and ileocolic fistula developed in one patient with CD. The disease was controlled with 5-aminosalicylic acid and corticosteroid in most patients. Four patients required additional therapy with azathioprine. Infliximab was used in two patients who were chronically steroid-dependent CD, one also had persistent ileocolic fistula and both patients responded well. During the follow-up period ranging from 1.1 to 5.8 years, three patients remained growth retardation; all had early onset of disease before 6 years of age, long duration of symptoms of more than 3 years before diagnosis and had multiple relapses. It is concluded that there is an increasing number of IBD in Thai children during the recent years. Most patients had moderate to severe diseases. Early onset of disease, delay in diagnosis and treatment are responsible for more complications, particularly persistent growth impairment. Early recognition of IBD and treatment are essential for a satisfactory long-term outcome.

Cardiovascular examination with 3.0 Tesla magnetic resonance imaging: first 100 cases at Ramathibodi Hospital.

BACKGROUND: Cardiovascular magnetic resonance imaging (CMR) has been utilized for diagnosis in various cardiovascular diseases and most of those were performed on a 1.5 Tesla CMR system. Recently, a 3.0 Tesla magnetic resonance imaging system has been introduced into clinical practice, however the clinical experience on cardiovascular examination using this system is limited. Therefore, the authors' institution has integrated a team for developing a CMR program on this 3.0 Tesla system. OBJECTIVE: To describe the authors' experience on the 3.0 Tesla CMR system. MATERIAL AND METHOD: The data on patients referred to the authors' CMR unit between August 2004 and October 2005 were reviewed. RESULTS: One hundred patients were referred for CMR examination. The mean age was 56 years (2 month - 85 years) and 65 patients were male. The most common indication was to assess coronary artery disease (64 patients). The performed examination was divided into cardiac structure and function assessment (39%), stress testing (23%), coronary magnetic resonance angiography (13%), myocardial viability assessment (12%), magnetic resonance angiography (9%), and flow assessment (4%). CONCLUSION: The present study highlights that comprehensive assessment of various cardiovascular diseases can be performed on the 3.0 Tesla CMR system.

Cardiac CT angiography in children with congenital heart disease.

Cardiac imaging plays an important role in both congenital and acquired heart diseases. Cardiac computed tomography (angiography) cCT(A) is a non-invasive, increasingly popular, complementary modality to echocardiography in evaluation of congenital heart diseases (CHD) in children. Despite radiation exposure, cCT(A) is now commonly used for evaluation of the complex CHD, giving information of both intra-cardiac and extra-cardiac anatomy, coronary arteries, and vascular structures. This review article will focus on the fundamentals and essentials for performing cCT(A) in children, including radiation dose awareness, basic techniques, and strengths and weaknesses of cCT(A) compared with cardiac magnetic resonance imaging (cMRI), and applications. The limitations of this modality will also be discussed, including the CHD for which cMRI may be substituted.

Transcoronary bone marrow-derived progenitor cells in a child with myocardial infarction: first pediatric experience.

BACKGROUND: Recent advances in stem cell therapy to restore cardiac function have great promise for patients with congestive heart failure after myocardial infarction in an adult population. OBJECTIVE: We examined the benefits of bone marrow-derived progenitor cells treatment modality for the pediatric patient. METHODS AND RESULTS: We present our first case of transcoronary autologous stem cell transplantation in a 9-year-old girl with refractory congestive heart failure secondary to myocardial infarction 1 year after transcatheter revascularization. The child received daily injections of granulocyte colony-stimulating factor for 3 days prior to the bone marrow aspiration. The bone marrow cells were isolated to constitute CD133+/CD34+ more than 90% of the total number. Subsequently, the progenitor cell suspension was injected via a transcoronary catheter without any complication. Three months after stem cell therapy, her cardiac function, assessed by both cardiac magnetic resonance and echocardiogram, has been improved with the left ventricular ejection fraction at 47% compared to the baseline of 30%. CONCLUSION: This is the first reported pediatric case of successful transcoronary injection of bone marrow-derived progenitor cells for end-stage heart disease. The procedure is considered safe and feasible for the pediatric population.

Secondary Burkitt lymphoma in a retinoblastoma patient with 13q deletion syndrome.

We report a boy with constitutional deletion 13q chromosome associated with dysmorphic features and bilateral retinoblastoma. The patient developed secondary Burkitt lymphoma 5 years after the diagnosis of retinoblastoma at the age of 8 months. He has completed treatment for both malignancies. At present, he is 7 years old and still in remission.