Splenic Malformation Syndrome Research Articles

Rare spontaneous monochorionic dizygotic twins: a case report and a systematic review

BackgroundMonochorionic dizygotic twins are a rare condition, mostly related to assisted reproductive technology. This type of twinning is burdened by the same risk of pregnancy complications found in monochorionic monozygotic pregnancies.Case presentationWe report a case of spontaneous monochorionic dizygotic twins sharing situs inversus abdominalis and isolated levocardia, with only one twin affected by biliary atresia with splenic malformation syndrome. We also conducted a literature review of the 14 available documented monochorionic dizygotic twin gestations spontaneously conceived.ConclusionsIt is still unclear how this unusual type of twinning can occur in spontaneous conception. The evidence so far suggest the importance to timely diagnose the chorionicity, in order to adequately manage the typical complications associated with monochorionicity.

Outcomes of biliary atresia splenic malformation (BASM) syndrome following Kasai operation: a systematic review and meta-analysis.

Outcomes of biliary atresia splenic malformation (BASM) syndrome following Kasai operation: a systematic review and meta-analysis.

Biliary Atresia Splenic Malformation Syndrome: A Single Center Experience

Abstract:
 Introduction: The cause of biliary atresia (BA) is not understood exactly as well as biliary atresia splenic malformation (BASM) syndrome. BA is destructive biliary fibrosis; the etiology may be multifactorial. Association of cytomegalovirus (CMV) and BA have been shown in many reports but CMV and BASM have not been mentioned in the literature. So we aimed to report BASM experiences, an association of CMV infection and need of duodenoduodenostomy if preduodenal portal vein exists.
 Materials and Methods: The data were collected retrospectively from Cukurova University which is one of the largest tertiary hospitals in Turkey between 2005-2017. The patients of sex, age, blood chemistry counts, TORCH infections blood parameters, BA types, operational findings and mortality were noted.
 Results: In total, 59 BA patients were diagnosed between 2005- 2017. Seven of them were classified as BASM. The median age of them was 60 days (45-90 days). Three of them were girl and 4 of them were male in gender. The main complaint of whole patients was jaundice. The jaundice of 6 patients began since birth. One of them began at 20 days-age. Median total / direct blood bilirubin levels were 9.6 / 5.4 mg/dl. Median values of liver function tests; ALT, AST and GGT were 77 IU/L, 201 IU/L and 607 IU/L respectively.
 Five of the patients showed positive results for anti-CMV immunoglobulin M. All had positive anti-CMV Ig G and anti-toxoplasmosis Ig G.
 Evaluation of the types of BA revealed that one patient had type 2, while all others had type 3. Four of BASM patients had polysplenia and one had asplenia. Five of them had a preduodenal portal vein. All of them had midgut malrotation. One had inferior vena cava interruption. One had hepatic artery anomaly which was originated from SMA.
 The median time of follow-up was 4 years (1-5 years). All of them are alive and 1 required liver transplantation.
 Conclusion: BASM should be kept in mind by the surgeon for the requirement of additional surgical procedures such as Ladd procedure, duodenoduodenostomy with Kasai Porto-enterostomy.
 Duodenoduodenostomy may be performed when the existence of preduodenal portal vein.
 Further research is recommended for CMV infection and BASM.

Ductal Plate Malformation-Like Arrays in Early Explants after a Kasai Procedure Are Independent of Splenic Malformation Complex (Heterotaxy)

Biliary atresia has at least 2 proposed forms, the common perinatal and the less common embryonic subtype with earlier onset and/or extrahepatic developmental anomalies. Histologic evidence of ductal plate malformation (DPM)-like change in liver has been proposed both as a marker for the embryonic type and as a predictor of poor outcome after Kasai portoenterostomy. We investigated the prevalence of DPM-like change in liver explants in usual biliary atresia (BA) and in BA with splenic malformation syndrome (BASM). Liver sections from 8 patients with BA and 6 with BASM, all of whom had a Kasai procedure followed by explant before age 2 years, were analyzed using hematoxylin and eosin, trichrome, CK7, and AE1/AE3 stains. Each block was scored for inflammation and fibrosis. We estimated the number of portal areas per block and counted the number of definite and possible examples of DPM-like change, defined as a circumferential duct complex arranged around a fibrovascular core. We assessed whether the frequency per portal area was related to low and high scores for either inflammation or fibrosis. Definite and possible examples of DPM-like arrays were present in about 10% of portal areas in both patient groups, but these were unevenly distributed. There was no statistical difference between BA and BASM in terms of the number of examples per portal area. No correlation existed between degree of fibrosis and the intensity of portal inflammation and the number of DPM-like arrays. Ductal plate malformation-like arrays do not distinguish perinatal BA from BA associated with heterotaxy in liver explants after a failed Kasai procedure.