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PubMed1991

[Vesico-intestinal fissure: a multidisciplinary problem].

Vesico-intestinal fissure or exstrophy of the cloaca is a rare but serious birth defect of the urogenital tract and distal part of the digestive tract. The most important hallmarks are: bladder exstrophy, fusion between bladder and the exstrophied iliocoecal region, short blind-ending colon and imperforate anus. In addition the lower part of the body shows defects of the abdominal wall, symphysis, upper urinary tract, the internal and external genitals, lumbosacral spine, spinal cord and the lower extremities. It is just after 1960 that corrective surgery started to become successful. Because of the complexity of the anomaly it seems that only management by a team of specialists in a centre for pediatric surgery guarantees optimal treatment. The quality of live of these children can still be improved by application of the newest surgical techniques and excellent cooperation between the different involved specialists. The defects, surgical treatment and results in eight patients with vesico-intestinal fissure, seen at the Universitair Centrum voor Chirurgie bij Kinderen Nijmegen (UCCKN) from 1974 till 1989 inclusive, are presented.

Abnormalities, Multiple↗
PubMed1976

Duplication of the alimentary tract.

Duplications of the intestinal tract as can be seen from the embryologic and anatomic descriptions are varied and frequently complex. The great variety of presentation and pathology encountered requires almost individual treatment for each case. Certainly total surgical excision, or at the very least, good complete internal drainage should be carried out in these cases. Correct therapy should afford relief of the patients's symptoms and prevent future complications.

Abnormalities, Multiple↗
PubMed1985

Hypospadias in British Columbia.

The British Columbia Health Surveillance Registry (BCHSR) records the frequency, incidence, and distribution of congenital malformations and other disabilities among individuals within the province using multiple sources of ascertainment. The most important sources of ascertainment for this study were Physician's Notice of Birth forms and discharge diagnosis from all hospitals in the province on children 7 years old or younger. These data were used to determine the minimal incidence of hypospadias with and without other congenital anomalies in order to provide information useful in management and to establish baseline prevalence data on a common genital malformation. In addition, incidence over time was evaluated. The study found the minimal incidence of hypospadias in British Columbia to be 4.44 per 1,000 male live births (1,314 cases out of 295,656 male live births) during 1966-1981. This is in the previously reported range of the incidence of hypospadias (2-8.2 per 1,000 male live births). Hypospadias was the only malformation in almost 80% of all individuals identified. The most frequent additional anomalies involved the genital and inguinal regions (7.2% of all cases or 36% of cases with additional anomalies). Cardiac lesions were the next most common anomalies, representing 14% of those cases with additional anomalies. Limb malformations and gastrointestinal anomalies were also quite common, representing 12.1% and 9.1% of cases of hypospadias with additional anomalies, respectively. Thus, 1 in 225 males born in British Columbia has some degree of hypospadias, and 20% of these infants also have at least one other anomaly.

Abnormalities, Multiple↗
PubMed1989

Bendectin and human congenital malformations.

The relationship between Bendectin exposure during the first trimester of pregnancy and the occurrence of congenital malformations was prospectively studied in 31,564 newborns registered in the Northern California Kaiser Permanente Birth Defects Study. The odds ratio for any major malformation and Bendectin use was 1.0 (95% confidence interval 0.8-1.4). There were 58 categories of congenital malformations; three of them were statistically associated with Bendectin exposure (microcephaly--odds ratio = 5.3, 95% confidence interval = 1.8-15.6; congenital cataract--odds ratio = 5.3, 95% confidence interval = 1.2-24.3; lung malformations (ICD-8 codes 484.4-484.8)--odds ratio = 4.6, 95% confidence interval = 1.9-10.9). This is exactly the number of associations that would be expected by chance. An independent study (the Collaborative Perinatal Project) was used to determine whether vomiting during pregnancy in the absence of Bendectin use was associated with these three malformations. Two of the three (microcephaly and cataract) had strong positive associations with vomiting in the absence of Bendectin use. We conclude that there is no increase in the overall rate of major malformations after exposure to Bendectin and that the three associations found between Bendectin and individual malformations are unlikely to be causal.

Abnormalities, Drug-Induced↗
PubMed2004

An extremely rare inversion of the preduodenal portal vein and common bile duct associated with multiple malformations. Report of an adult cadaver case with a brief review of the literature.

A preduodenal position of the portal vein (PDPV) is a very rare congenital anomaly; even rarer is its association with a preduodenal position of the common bile duct (PDCBD). To the seven cases of PDCBD mentioned in the literature, we add this particularly rare case which is associated with multiple abnormalities such as situs inversus totalis, intestinal malrotation, short pancreas, bilobed spleen, accessory spleen, and abnormal ramification of the celiac axis, superior mesenteric artery and renal arteries. Besides describing and illustrating this case, we also discuss the anatomy and embryology of these structures and briefly review the patterns of previously reported cases that we found. We performed an immunohistochemical examination of the pancreas to demonstrate the ventro-dorsal pancreas in our case. For the explanation of the embryology of the PDCBD, the ventro-dorsal pancreas and PDPV malformation, we emphasized the reverse rotation of the ventral pancreas and duodenum.

Abnormalities, Multiple↗