Congenital coronary arteriovenous fistulas (CCAFs) are rare coronary artery abnormalities in which blood is shunted into a cardiac chamber or great vessel. If the fistula itself is large and tortuous, it is generally recommended to occlude the fistula to prevent several complications. In approaches of transcatheter occlusion, the transvenous approach is preferred over the transarterial approach. The transvenous approach would enable the cannulation of a relatively larger catheter or sheath without potential damage to the femoral vessels or normal coronary arteries, which can occur in the transarterial approach. The transvenous approach may also minimize the blind pouch after releasing the devices. Herein, we report the success of transvenous proximal closure of a CCAF using an Amplatzer vascular plug (AVP) in a 3-year-old patient with cardiomegaly. Complete occlusion was achieved by a single AVP and thrombus formation of the distal aneurysmal portion of the fistula. We suggest that this strategy of closing the proximal end with a dilated fistula using a single AVP by the transvenous approach may be a good option in treating CCAFs in a young child.
Aneurysm ; Arteriovenous Fistula ; Cardiomegaly ; Catheterization ; Catheters ; Child ; Coronary Angiography ; Coronary Vessel Anomalies ; Coronary Vessels ; Fistula ; Glycosaminoglycans ; Humans ; Septal Occluder Device ; Thrombosis

Sideroblastic anemia is a rare, heterogeneous group of disorders characterized by hyperferremia, microcytic hypochromic anemia, and bone marrow erythroid hyperplasia with the presence of numerous ringed sideroblasts. We describe herewith the case of a rare coincidence of sideroblastic anemia and mitral valve prolapse with resultant regurgitation in a 2-year-old boy. In addition to the inherent propensity for the development of cardiac dysfunction in sideroblastic anemia due to transfusion-associated myocardial iron overload and chronic anemia, a coincidence of MVP will further increase the likelihood of the morbidity or mortality of th patient. in this patient. After response to pyridoxine, the patient remains in good condition with stable hemoglobin levels.
Anemia ; Anemia, Hypochromic ; Anemia, Sideroblastic* ; Bone Marrow ; Child, Preschool ; Humans ; Hyperplasia ; Iron Overload ; Male* ; Mitral Valve Prolapse* ; Mitral Valve* ; Mortality ; Pyridoxine*

Testicular feminization syndrome (TFS) is a genetic disorder due to androgen insensitivity of the target organs. The most common clinical presentation of complete TFS is inguinal hernia in the infant or primary amenorrhea in the adolescence. A 7-year old phenotypically female patient was seen with a complaint of a right inguinal mass. Under the diagnosis of right inguinal hernia, high ligation was performed. Six months later, the patient showed a left inguinal mass. On operation, the mass looked like a testis. The external genitalia were normal female, but a uterus and ovary were not identified. Chromosome study showed a 46, XY karyotype and the levels of serum testosterone and dihydrotestosterone were increased after HCG stimulation. The patient was diagnosed as complete TFS and underwent bilateral gonadectomy 6 months later.
Adolescent ; Amenorrhea ; Androgen-Insensitivity Syndrome* ; Child ; Diagnosis ; Dihydrotestosterone ; Female ; Genitalia ; Hernia, Inguinal ; Humans ; Infant ; Karyotype ; Ligation ; Male ; Ovary ; Testis ; Testosterone ; Uterus

Testicular feminization syndrome (TFS) is a genetic disorder due to androgen insensitivity of the target organs. The most common clinical presentation of complete TFS is inguinal hernia in the infant or primary amenorrhea in the adolescence. A 7-year old phenotypically female patient was seen with a complaint of a right inguinal mass. Under the diagnosis of right inguinal hernia, high ligation was performed. Six months later, the patient showed a left inguinal mass. On operation, the mass looked like a testis. The external genitalia were normal female, but a uterus and ovary were not identified. Chromosome study showed a 46, XY karyotype and the levels of serum testosterone and dihydrotestosterone were increased after HCG stimulation. The patient was diagnosed as complete TFS and underwent bilateral gonadectomy 6 months later.
Adolescent ; Amenorrhea ; Androgen-Insensitivity Syndrome* ; Child ; Diagnosis ; Dihydrotestosterone ; Female ; Genitalia ; Hernia, Inguinal ; Humans ; Infant ; Karyotype ; Ligation ; Male ; Ovary ; Testis ; Testosterone ; Uterus

PURPOSE: Pylorus-preserving pancreatoduodenectomy (PPPD) is an alternative surgical procedure for periampullary lesions. Early delayed gastric emptying is the most common and frustrating complication in the immediate postoperative period after PPPD and late delayed gastric emptying has been reported in some long-term follow-up studies. We evaluated the incidence of early delayed gastric emptying and analyzed temporal changes in gastrointestinal function after PPPD. METHODS: The incidence of early delayed gastric emptying was retrospectively evaluated from the medical records of 15 patients who underwent PPPD. Gastric emptying tests (GETs) using 99mTc-DTPA scan were performed on 11 of the patients every three months until 1 year, where possible. RESULTS: The incidence of early delayed gastric emptying was 6.7%. Five of the eight patients (62.5%) and six of the eight (75%) who underwent scintigraphy at 3 months and 6 months respectively, showed delayed gastric emptying. But at 12 months, all of the four patients who underwent GETs showed normal gastric emptyings. CONCLUSION: The incidence of early delayed gastric emptying after PPPD was 6.7%. Though there were few symptoms in long-term follow-up study using 99mTc-DTPA scan, delayed gastric emptying was frequently observed 3 to 9 months after PPPD. However, gastric emptying might be normalized in almost all patients around 1 year after PPPD.
Follow-Up Studies ; Gastric Emptying* ; Humans ; Incidence ; Medical Records ; Pancreaticoduodenectomy* ; Postoperative Period ; Radionuclide Imaging ; Retrospective Studies

PURPOSE: Mouse liver nonparenchymal cells play an important role in the development of active apoptosis in graft- infiltrating cytotoxic T lymphocytes, and this apoptosis can be an explanation for liver graft acceptance. We intended to clarify whether immature mouse liver dendritic cells can induce apoptosis in allogeneic activated T cells and determine which mechanism is involved in this phenomenon. METHODS: A radiometric DNA fragmentation test ("JAM" assay) was used to determine whether mouse liver dendritic cells were able to induce activated T-cell apoptosis in vitro. In addition, immunohistochemical staining for Bax and Bcl-2 was examined to clarify whether Bax or Bcl-2 was involved in this apoptosis. RESULTS: Immature mouse liver dendritic cells were quite strong inducers of activated T cell apoptotic death in allogeneic mice in vitro (39.2+/-13.2% at E/T ratio=12.5/1) compared with spleen cells as effectors (4.7+/-13.4% at E/T ratio=12.5/1) (P<0.0001). By using immunohistochemical staining, we also showed that Bax might play some role in this phenonenon, but that Bcl-2 might not. CONCLUSION: Our data indicate that immature mouse liver dendritic cells might have a strong apoptotic activity toward activated T cells in allogeneic mice in vitro through a Bax-involved mechanism.
Animals ; Apoptosis* ; Dendritic Cells* ; DNA Fragmentation ; Liver* ; Mice* ; Spleen ; T-Lymphocytes* ; T-Lymphocytes, Cytotoxic ; Transplants

PURPOSE: Anomalies in the thyroglossal duct are the most common midline, or paramedian cervical lesions of congenital origin. Presenting as a thyroglossal duct cyst (TGDC) or a thyroglossal duct sinus (TGDS), they are found in all age groups. This clinical study was intended to elucidate the clinical characteristics, and the outcomes, of surgical treatment of these anomalies. METHODS: Between January 1992 and May 2002, 91 patients underwent surgery for a TGDC or a TGDS at 4 hospitals affiliated to Hallym University. The demographics of the patients, the clinical characteristics of the lesions, and the outcomes of the treatments were retrospectively evaluated. These characteristics were evaluated according to age groups; younger than 15 (38 patients) and older than 15 (53 patients) years. RESULTS: The male to female ratio was 1.67: 1 (It is better to give the actual figures.), with the first decade showing the highest incidence in the age distribution (33%). Painless masses (83.6%) were the most common presenting symptom, with most symptoms having been manifest for less than 1 year (67%). TGDCs were 90% and TGDSs were 10%. There were 62 cases of infrahyoid and 18 of suprahyoid lesions, with 80 cases on the midline and 11 on the paramedian within 2 cm from the midline. Of the paramedian lesions, 2 cases were in the younger group and 9 were in the older group. This tendency of laterality in the location by age group showed no statistical significance (p=0.172). All the patients underwent a Sistrunk operation, and one experienced a recurrence. CONCLUSION: This study justified the Sistrunk operation as the treatment of choice for anomalies of the thyroglossal duct. The lateral lesions occurred in the old age groups more frequently. A careful approach is needed with paramedian cervial lesions in adults to avoid the loss of the proper treatment of possible hidden congenital lesions.
Adult ; Age Distribution ; Demography ; Female ; Humans ; Incidence ; Male ; Recurrence ; Retrospective Studies ; Thyroglossal Cyst*

PURPOSE: Choledochal cyst is a rare disease associated with an anomalous junction of the pancreaticobiliary ductal system (AJPBDS). We intended to demonstrate the characteristics of adult-onset choledochal cyst retrospectively. METHODS: ERCP findings of nineteen adult cases of choledochal cyst were compared with those of nine normal persons, in respect to the length and diameter of the common channel, and the angle of the pancreaticobiliary ductal junction. RESULTS: Patients of adult-onset choledochal cyst displayed a long, ectatic common channel and a larger angle of the pancreaticobiliary junction (p<0.05). All patients except one had AJPBDS. Thirteen patients were classified as Todani type Ia, one as type Ic and five as type IVa. Fourteen patients were classified as choledochopancreatic (C-P) type, two as pancreaticocholedochal (P-C) type, and three were unclassified by Kimura's classification. Sixteen patients were compatible with cystic type and three with cylindrical type by Okada's classification. Among the three cylindrical type patients two of them were classified as P-C type and one as C-P type. Histologically cystic type patients showed mild glandular or fibrotic patterns, however cylindrical type patients tended to show the severe glandular pattern. CONCLUSION: Adult-onset choledochal cyst was closely related with AJPBDS, although the reflux of pancreatic juice due to AJPBDS would not be the only cause to determine the morphology of adult-onset choledochal cyst. The meaning of this tendency toward a severe glandular pattern in cylindrical type patients requires elucidation.
Adult ; Cholangiopancreatography, Endoscopic Retrograde ; Choledochal Cyst* ; Classification ; Humans ; Pancreatic Juice ; Rare Diseases ; Retrospective Studies

Despite of advances in perinatal management and treatment modalities, congenital diaphragmatic hernia (CDH) remains a frustrating problem. Although the sheep has been the most reliable experimental animal of fetal surgery in CDH, the rabbits has some possible advantages over sheep; lower cost, smaller body size, year-round availability, high number of fetuses per pregnancy, and short gestational period. The purpose of this study is to evaluate the feasibility of the animal model of CDH in rabbits. Twenty seven pregnant New Zealand rabbits underwent hysterotomy and fetal operation on gestational day 24 or 25. Two fetuses of each pregnant rabbit were operated. In the fetus of one end of bicornuate uterus, left diaphragmatic hernia was created by excision of fetal diaphragm through open thoracotomy (DH Group). In the fetus of the other end of bicornuate uterus, left diaphragmatic hernia was created and tracheal ligation by small-sized Surgiclip(R) (USSC, Norwalk, Conn., USA) was performed through cervical incision (TL Group). Among twenty seven pregnant rabbits, twelve were born alive with diaphragmatic hernia and eight were born alive with diaphragmatic hernia & tracheal ligation. The most commonly herniated abdominal organ was the left lobe of liver. In DH group, the lungs were hypoplastic with a decrease in lung weight/body weight ratio, a reduction in number of alveoli, an increase of vascular medial wall thickness of pulmonary arteries. The alveoli were immature with thick alveolar septum and increased interstitium. In TL group, the alveoli were more mature than that of DH group and no significant difference from control group was existed. Thus this study demonstrates that (1)Diaphragmatic hernia can be created in rabbits by fetal surgery, (2)Tracheal ligation can be performed in rabbits by fetal surgery, (3)Experimental diaphragmatic hernia results in pulmonary hypoplasia, (4)Concurrent tracheal ligation prevents pulmonary hypoplasia that resulted from diaphragmatic hernia. The above mentioned results show that pregnant New Zealand rabbit is appropriate as the animal model of CDH.
Animals ; Body Size ; Diaphragm ; Fetus ; Hernia ; Hernia, Diaphragmatic* ; Hysterotomy ; Ligation* ; Liver ; Lung ; Models, Animal ; Pregnancy ; Pulmonary Artery ; Rabbits ; Sheep ; Thoracotomy ; Uterus

Kimura's disease is a rare chronic inflammatory and proliferative condition of unknown etiology. It has been thought to be a part of large spectrum of the entity known as angiolymphoid hyperplasia with eosinophilia (ALHE). However, recently the difference is emphasized between the two entities by some authors. It usually presents subcutaneous or dermal mass in the head and neck region. This condition can be mistaken for a malignant tumor. It is defined pathologically as hyperplastic lymphoid follicles, eosinophilic infiltration and vascular proliferation. Authors have experienced three cases of Kimura's disease occurring in the submandibular, axillary and inguinal regions since 1993. There were two males and one female. Two patients presented peripheral eosinophilia. All patients underwent surgical excision. Two patients were managed with oral prednisone postoperatively.
Angiolymphoid Hyperplasia with Eosinophilia ; Eosinophilia ; Eosinophils ; Female ; Head ; Humans ; Male ; Neck ; Prednisone