ABSTRACT
Fibrous hamartoma of infancy in the middle ear is extremely rare. We report the case of a 26-month-old male patient who presented with a mass in the left middle ear. A temporal bone CT scan showed complete opacification of the left middle ear and mastoid air cells without ossicular erosion. On MRI, the mass revealed heterogeneous signal intensities indicative of fat and fibrous components. A definitive diagnosis was made postoperatively based on the histological results. Although rare, fibrous hamartoma of infancy should be considered as a differential diagnosis of a middle ear mass during childhood.
ABSTRACT
The coronary sinus (CS) is the venous drainage system of the heart. CS ostium atresia is a rarely seen cardiac malformation. Congenital atresia of the CS is usually found together with persistent left superior vena cava (LSVC) and other cardiac malformations. However, isolated congenital atresia of the CS is very rare. We present a rare case of isolated congenital atresia of the CS connecting the left atrium and coronary veins without persistent LSVC in a 58-year-old female.
ABSTRACT
The coronary sinus (CS) is the venous drainage system of the heart. CS ostium atresia is a rarely seen cardiac malformation. Congenital atresia of the CS is usually found together with persistent left superior vena cava (LSVC) and other cardiac malformations. However, isolated congenital atresia of the CS is very rare. We present a rare case of isolated congenital atresia of the CS connecting the left atrium and coronary veins without persistent LSVC in a 58-year-old female.
ABSTRACT
PURPOSE: In Korea, neonatal clinical features and feeding pattern are not reported systematically. So we report the neonatal presentation of Prader-Willi syndrome. METHODS: This is a retrospective study of 14 neonate with Prader-Willi syndrome whose diagnosed in neonatal intensive care unit, Samsung Medical Center between January 1st 1997 and December 30th 2011. RESULTS: The birth characteristics of patients, maternal age was 31.5+/-4.5 years and 5 (35.7%) were primiparous. Oligohydramnios were reported in 2 cases (14.2%), polyhydramnios were reported in 4 cases (28.5%). 5 pregnancies (35.7%) had complained that decreased fetal movements. Mean gestational age were 38.1+/-2.2 weeks and 12 patients (85%) were term infants, one patient (7.1%) was post term infant, the other patient was preterm infants (7.1%). The average weight at birth were 2509.0+/-442.5 grams. All 14 patients (100%) had hypotonia and a weak cry at birth. 9 patients (64.5%) had the characteristic facial dysplasia. In all, nine male patients (100%) had cryptorchidism. All patients had weak sucking power, so they started to eat via nasogastric tube at birth. The mean duration of use nasogastric tube were 3.2+/-2.0 months. The median age of diagnosis were 1.5 months. 7 cases (50%) were diagnosed within one month of life. Median time of starting GH treatment was 2.0 years. The mean interval of from diagnosis to treatment of growth hormone were 2.2+/-2.0 years. CONCLUSION: Early diagnosis of Prader-Willi syndrome is a very important to improve long-term prognosis.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Male , Pregnancy , Cryptorchidism , Early Diagnosis , Feeding Behavior , Fetal Movement , Gestational Age , Growth Hormone , Infant, Premature , Intensive Care, Neonatal , Korea , Maternal Age , Muscle Hypotonia , Oligohydramnios , Parturition , Phenotype , Polyhydramnios , Prader-Willi Syndrome , Prognosis , Retrospective StudiesABSTRACT
Pleural effusion and diaphragmatic palsy secondary to fluid extravasation after central line insertion in the neonate are rare complications. Here we report a case of right pleural effusion and diaphragmatic palsy caused by fluid extravasation associated with peripherally inserted central venous catheter in the preterm infant.
Subject(s)
Humans , Infant, Newborn , Central Venous Catheters , Infant, Premature , Paralysis , Pleural EffusionABSTRACT
The congenital complete atrioventricular block(CCAVB) is a rare disease, which is the most serious complication of neonatal lupus erythematosus. Newborn with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. Transplacental steroid and beta-adrenergic agonist therapies are useful for the first and second degree atrioventricular block. But those therapies are usually not effective for complete atrioventricular block. If the fetus has a complete atrioventricular block, delivery should be considered unless other delivery contraindications. In this situation, early pacemaker implantation surgery can improve the survival rate of patients. We report one case of premature newborn with congenital complete atrioventricular block who is successfully recovered by pacemaker implantation.
Subject(s)
Humans , Infant, Newborn , Adrenergic beta-Agonists , Atrioventricular Block , Cardiac Output , Fetus , Heart Failure , Lupus Erythematosus, Systemic , Rare Diseases , Survival RateABSTRACT
PURPOSE: To investigate the efficacy of early infliximab use and to follow the progress of pediatric cases of Crohn's disease for 3 years. METHODS: We reviewed the medical records of 28 pediatric patients who had been treated with infliximab for Crohn's disease. Eighteen patients (the 'top-down' group) received infliximab and azathioprine for induction and maintenance therapy for the first year, and then were treated with azathioprine for 2 additional years. Ten patients who were refractory to conventional therapy were categorized in the 'step-up' group. All patients were followed for at least 36 months. Treatment efficacy was assessed by the relapse rate using the pediatric Crohn's disease activity index (PCDAI) score in each group at 12, 24, and 36 months. Blood samples were available from 10 patients, and were used to assess antibody to infliximab (ATI). RESULTS: The relapse rate in 'top-down' group was lower than that in 'step-up' group at 1, 2, and 3 years. But, just the relapse rate at the 2 years was significantly different. At 3 years, the relapse rate according to different characteristic variables (sex, age at diagnosis, involvement, PCDAI at diagnosis) was not significantly different. Only one patient treated with infliximab had an adverse event, consisting of dyspnea and tachycardia. ATI was not detected in the blood samples from 10 patients. CONCLUSION: Early induction with infliximab at diagnosis ('top-down' therapy) is effective for reducing the relapse rate compared to conventional therapies in pediatric Crohn's disease possibly for up to 3 years.
Subject(s)
Humans , Antibodies, Monoclonal , Azathioprine , Crohn Disease , Dyspnea , Follow-Up Studies , Medical Records , Recurrence , Tachycardia , Treatment Outcome , InfliximabABSTRACT
Obstetrical procedures such as prolonged obstructed labour are responsible for the majority of vesicovaginal fistula(VVF) in developing countries, while gynecological procedures are more common causes in developed countries with hysterectomy accounting for 75% of fistulae. Other less common causes of VVF include uterine cancer, pelvic irradiation, gastrointestinal pelvic surgery, tuberculosis, intra-uterine device or Bechet's disease. Although radiation techniques are being advanced, several recent studies have shown the incidence of VVF after pelvic radiation therapy for cervical cancer to range from 0.6~2%. We report, a case of 47-year-old female with VVF after pelvic radiation therapy, which was treated by neurovascular pudendal-thigh flap.
Subject(s)
Female , Humans , Middle Aged , Developed Countries , Developing Countries , Fistula , Hysterectomy , Incidence , Tuberculosis , Uterine Cervical Neoplasms , Uterine Neoplasms , Vesicovaginal FistulaABSTRACT
Wilms' tumor is the most common renal malignancy in childhood. However Wilms' tumor originating in horseshoe kidney is rare. We present a case of Wilms' tumor arising from the isthmus of horseshoe kidney, and aim to highlight the problems faced in the diagnosis and management.