Crossed fused renal ectopia with segmental fusion of bilateral ureters and abdominal aortic anomalies in a patient with caudal regression syndrome.

Caudal regression syndrome consists of multiple congenital anomalies, mainly caudal segment defects. We describe a preterm baby born to a healthy mother with typical caudal regression picture, including imperforated anus with rectovesical fistula, sacral agenesis, multiple rib and vertebral anomalies, and club feet. Crossed fused renal ectopia with fused ureters resulting in urinary obstruction was managed with transureteroureterostomy and cutaneous vesicostomy. We also found a single large umbilical artery with high abdominal aortic insertion which usually presents in sirenomelia. Because of the anatomical diversity of the urinary and cardiovascular systems associated with multiple congenital anomalies, careful evaluation is mandatory.

Role of thermography in the diagnosis of undescended testes.

OBJECTIVES: The location of an undescended testis is important for the choice of therapy. Ultrasonography cannot serve as a stand-alone screening method in the management of the undescended testis because of its limited sensitivity and accuracy. The aim of this study was to clarify the diagnostic value of thermography in the patients with undescended testes. METHODS: We evaluated prospectively 28 patients with 36 undescended testes from January 1995 to December 1996. The patients' ages ranged from 16 to 39 months with a mean age of 26.3 +/- 8.2 months. In addition to physical palpation by a pediatric surgeon, ultrasonography and thermography were performed for screening the locations of retained testes. RESULTS: The diagnostic rates were 63.9% (23/36) by palpation, 65.7% (23/35) by ultrasonography and 54.5% (18/33) by thermography. The results of the three diagnostic methods showed no significant difference by Fisher's exact test. Of the 17 higher located testes (inguinal canal and above external ring) 7 were palpable, 8 were identified by ultrasonography, 10 were detected by thermography. Of the 7 nonpalpable testes and testes not detected by ultrasonography, 5, including 2 intra-abdominal testes, were identified by thermography. CONCLUSION: It is suggested that thermography can play a role in locating high undescended testes which are nonpalpable and not detected by ultrasonography.

Successful early separation of a premature xipho-omphalopagus conjoined twins: a case report.

Premature xipho-omphalopagus conjoined twins were successfully separated at 7 days of age. The total body weight of the twins before separation was 3502 g. One twin had persistent patent ductus arteriosus and signs of cardiac failure at 4 days of age. This was managed with indomethacin. Thirty-six hours later the second twin became anuric, necessitating early emergency separation. The twins were joined from the lower sternum to the infraumbilical area. The liver was fused, but there was no major vascular connection. There were no other major anomalies. The babies stood the procedure well and were healthy at follow-up 8 months after separation.

Manifestations and surgical treatment of choledochal cyst in different age group patients.

Whether early surgery is necessary for asymptomatic choledochal cyst is still controversial. In order to evaluate the manifestations of choledochal cyst on different age group patients, we investigated 57 patients, 36 females and 21 males, who underwent surgery for choledochal cyst at National Taiwan University Hospital from July 1988 to June 1995. Of these, 93% were classified as type I according to the Alonzo-Lej or Todani classification method. Patients were divided into three groups according to their age at surgery: group I, 14 infants (< 1-yr); group II, 28 children (1-16 yr); and group III, 15 adults (> 16 yr). The most common symptom in infants was jaundice, while abdominal pain was the most frequent in children and adults. Preoperative evaluation of liver function showed that alanine aminotransferase was higher in group II (160 +/- 77 IU/L) and group III (164 +/- 75 IU/L) than group I (74 +/- 28 IU/L). A higher tendency of biliary sludge and stone formation was noticed in group III (6/15, 40%) when compared with group I (2/14, 14.3%) and group II (5/28, 17.9%). The incidence of high bile amylase concentration in the choledochal cyst was higher in groups II (16/21,76.2%) and III (9/11, 81.8%) than in group I (2/9, 22.2%). There was more inflammatory changes detected in the choledochal cyst wall and gall bladder by histologic examination in older patients (groups II and III). Therefore, an early surgery for choledochal cyst is suggested for prevention of liver injury, biliary sludge, stone formation and pancreatitis.

Accessory hepatic duct associated with a choledochal cyst.

This case report describes an accessory hepatic duct (AHD) identified by intraoperative cholangiography during excisional surgery of a choledochal cyst (CC). The accessory duct was divided and reconstructed successfully to the Roux-en-Y jejunal loop. The postoperative course was uneventful, and follow-up abdominal sonography revealed neither evidence of biliary tract obstruction nor atrophic changes of the liver. It is advocated that an AHD should be meticulously reconstructed if it is divided during excisional surgery of a CC.

Perforated Meckel's diverticulum in a neonate.

A case of Meckel's diverticulum is reported in an eight-day-old male baby, the youngest patient to have had this condition at National Taiwan University Hospital. The baby had been affected by bilious vomiting of 3 days' duration and abdominal distension before being brought to the hospital. A perforated Meckel's diverticulum was found during emergency laparotomy. The resected Meckel's diverticulum did not contain ectopic tissue. The narrow lumen and narrow base of the diverticulum was thought to be the cause of poor self-emptying and subsequent acute inflammation and perforation.

Pentalogy of Cantrell corrected by one-stage repair.

Pentalogy of Cantrell (POC), is a rare congenital defect associated with five ventral midline abnormalities and high mortality. We report a 2,788 g female infant with partial repair of POC in the neonatal stage. This consisted of repairing the omphalocele, plicating the eventrated diaphragm and placing the heart into the thoracic cavity by fixing the pericardium to the ventral side of the diaphragm. She was well 6 months after surgery.

Alimentary tract duplications.

A total of 17 patients with alimentary tract duplications underwent surgery at National Taiwan University Hospital from 1978 to 1994. Fifteen patients (88%) had gastrointestinal duplication and two (12%) had esophageal duplication. Common presenting symptoms of gastrointestinal duplication were melena and abdominal pain. The ileum was the most common site of duplication. Multiple duplications were seen in three patients. All duplications were cystic, except for one single appendiceal duplication. Ectopic gastric mucosa was detected in nine of the 16 nongastric duplications. One patient with ileal duplication had ectopic pancreatic tissue. Twelve patients received resection of the duplication with a segment of bowel and primary anastomosis, three patients underwent simple excision and two patients had partial resection of the duplication and stripping of the residual mucosa. Two patients had other associated congenital anomalies: one had ventricular septal defect and the other, imperforate anus and malrotation of intestine. There was no operative mortality or morbidity in this series.

Subpopulations of intrathyroidal lymphocytes in Graves' disease.

Subsets of T- and B-lymphocytes were immunophenotyped in frozen thyroid tissues from 20 patients with Graves' disease by using the avidin-biotin-complex peroxidase method. A panel of B- and T-cell monoclonal antibodies were employed to detect the subsets of T cells, activated T cells, natural killer cells and B cells. Serum thyroglobulin and microsomal antibodies were also measured simultaneously from both the thyroid vein and the peripheral vein in 9 patients. The quantitative results found that an almost equal ratio of lymphocytes stained positively for Leu 3a (CD4) and T8 (CD8) in almost all specimens. Activated lymphocytes expressing T9, T10, and Tac (CD25) were sporadically noted in the interstitial areas. The lymphoid aggregates and follicles were B cell clusters. Natural killer cells appear to play an insignificant role in Graves' disease. The thyroid epithelial cells expressed HLA-DR (Ia+) and HLA-ABC, suggesting a possible localized immune reaction between antigen-presenting thyroid epithelial cells and lymphocytes. The titers of serum thyroglobulin and microsomal antibodies from either the thyroid vein or the peripheral vein showed no definite correlation with the lymphoid distribution of the thyroid tissues, implying that the in situ or local immunologic reaction may not reflect the status of systemic antibody production. The immunologic mechanism and pathogenesis of Graves' disease is reviewed and discussed.