Annular pancreas in two consecutive siblings: an extremely rare case.

Annular pancreas is the rare congenital anomaly where the pancreas forms a full or incomplete ring around the second segment of the duodenum, causing various degrees of stenosis or atresia. It is estimated that it appears in 1 out of 12 000-15 000 births of living neonates and until now, in the literature, only 6 cases have been reported among individuals of the same family. We present the case of two siblings, a boy and a girl, with annular pancreas from consecutive pregnancies of the same couple. Both neonates had a prenatal diagnosis of duodenal obstruction and they underwent duodenoduodenal, proximal transverse to distal longitudinal anastomosis. Furthermore, the girl had a mobile ascending colon. Their postoperative condition was perfect. The case we are reporting is an addition to the other 6 cases of familial presentation of annular pancreas and is similar to one of them. In these families, a total of 16 persons present this congenital anomaly while 14 are seemingly healthy. Twelve of the affected persons are female and 4 male. In conclusion, it can be stated that female individuals seem to have a greater propensity to transmit the disease to their descendants, compared to males, suggesting the possible action of an autosomal recessive sex-influenced gene. The recording of such rare family cases should be encouraged, in order to fully recognize a possible type of inherited transmission.

Colostomy type in anorectal malformations: 10-years experience.

AIM: The aim of this study was to evaluate the influence of colostomy type on morbidity during the treatment of anorectal malformations. METHODS: Sixty-eight infants (male: female ratio 1.3:1) with anorectal malformations that required colostomy were treated in our clinics during the period 1991-2001. Of these patients, 26 had received a loop colostomy: 14 of these underwent posterior sagittal anorectoplasty (PSARP) at the age of 9-12 months (Group A), and 12 underwent PSARP at the age of 2-4 months (Group B). Forty-two infants received a separated-stomas colostomy and underwent PSARP at the age of 9-12 months (Group C). The incidence of complications among groups was compared using the 2 sided Fisher's exact test. RESULTS: Eight cases from group A were complicated with prolapse of the stomas, perianal wound infection, pull-through dehiscence, and anal fibrotic stricture. The only complication observed in groups B and C was perianal wound infection, which occurred in 1 case from each group. A statistically significant difference was observed in the incidence of complication between groups A and C (p<0.001) and between groups A and B (p=0.014). The results from groups B and C did not differ significantly (p=0.398). When the cases complicated with colostomy prolapse were removed from the statistical analysis, groups A and C still differed significantly (p=0.001) but groups A and B did not (p=0.069). CONCLUSIONS: As the incidence of complications increases with time after a loop colostomy, we encourage either an early corrective procedure or the modification into separated-stomas colostomy (SSC) before PSARP is performed for those cases that would involve definitive surgery in late infancy.

Splenic artery ligation: a ten-year experience in the treatment of selected cases of splenic injuries in children.

Splenic artery ligation (SAL) combined with either splenorrhaphy or partial splenectomy has been used as a spleen saving procedure in the management of massively bleeding splenic injuries. During the last 10 years, 37 children have been submitted to SAL following a selective management schedule. This study was jointly undertaken by two separate Pediatric Surgical Units in two different countries, in order to evaluate some preliminary observations published previously, with regard to; 1) the percentage of splenic injuries requiring ligation of the splenic artery; 2) the effect of this procedure on the arrest of bleeding; 3) the postoperative complications related to dearterialization of the spleen; 4) the immunological status after the operation and; 5) the postoperative imaging of the spleen using radioscintigrams and ultrasonograms. The mean age of the patients was 6.9 years and the follow up period ranged from 1 to 10 years. Thus, SAL was concluded to be an effective mode of treatment for rare cases of splenic injury unable to be treated nonoperatively or by splenorrhaphy alone. No postoperative complications were recorded in this series, while the immunological status remained undisturbed postoperatively and imaging of the spleen revealed intact and functional tissue with adequate healing.

The collateral arterial network of the spleen following ligation of the splenic artery in traumatic rupture of the spleen; an arteriographic study.

Ligation of the splenic artery, combined with splenorrhaphy, was used successfully on eight children with splenic trauma involving major segmental vessels. Evaluation of the collateral arterial network and of its adequacy was done by means of arteriography on four out of the eight patients. Aortographies were carried out on the twentieth day, the first month, the third month, and the fourth month following the ligation. A collateral arterial network, deriving mainly from the short gastric arteries was detected, dyestream disruption and retrograde filling of the artery. No complications after aortography were noted. Findings demonstrate that collateral blood supply develops rapidly and its pattern is rather uniform.