A rare case of spontaneous splenic infarction with polysplenia and situs inversus is presented. The diagnosis was made by ultrasound and confirmed by CT which showed multiple aberrant right-sided spleens with situs inversus and one of the spleen showing low attenuation areas representing infarct. Polysplenia syndrome is a rare entity associated with heterotaxy syndromes, congenital cyanotic heart diseases, billiary atresia, intestinal malrotation, immotile cilia syndrome, annular or short pancreas, preduodenal portal vein and vena caval anomalies.
In the family presented here the first child had asplenia syndrome with cor biloculare' transposition of the great vessels, pulmonary stenosis, and anomalous pulmonary venous drainage. Another sib had situs inversus with polysplenia syndrome, including very similar cardiovascular defects and biliary atresia. The possibility that these two syndromes, namely asplenia and polysplenia, are different manifestations of a similar defect in the normal asymmetrical development of internal organs is discussed.
We report a rare association of duodenal atresia with situs inversus abdominus in a newborn. The infantogram revealed “reverse double-bubble sign” without dextrocardia. The sonography and echocardiography confirmed the diagnosis of situs inversus abdominus with multiple cardiac anomalies. Laparotomy and a duodenoduodenostomy were carried out.
Duodenal atresia; reverse double- bubble; situs inversus abdominus
Biliary atresia (BA) is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. Up to 10% of cases have other congenital anomalies, such as polysplenia, asplenia, situs inversus, absence of inferior vena cava and pre-duodenal portal vein, for which we have coined the term Biliary Atresia Splenic Malformation (BASM) syndrome. For these infants the aetiology lies within the first trimester of gestation. For others affected with BA, aetiology is more obscure and perinatal destruction of fully-formed ducts perhaps by the action of hepatotropic viruses has been suggested. Whatever the cause, the lumen of the extrahepatic duct is obliterated at a variable level and this forms the basis for the commonest classification (Types I, II, III). All patients with BA present with varying degree of conjugated jaundice, pale non-pigmented stools and dark urine. Key diagnostic tests include ultrasonography, biochemical liver function tests, viral serology, and (in our centre) a percutaneous liver biopsy. In some centres, duodenal intubation and measurement of intralumenal bile is the norm. Currently BA is being managed in two stages. The first stage involves the Kasai operation, which essentially excises all extrahepatic biliary remnants leaving a transected portal plate, followed by biliary reconstruction using a Roux loop onto that plate as a portoenterostomy. If bile flow is not restored by Kasai procedure or life-threatening complications of cirrhosis ensue then consideration should be given to liver transplantation as a second stage. The outcome following the Kasai operation can be assessed in two ways: clearance of jaundice to normal values and the proportion who survive with their native liver. Clearance of jaundice (<2 mg/dL or <34 µmol/L) after Kasai has been reported to be around 60%, whereas five years survival with native liver ranges from 40% to 65%.
Biliary atresia; surgical jaundice
One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure. In conclusion: (a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.
Situs inversus abdominus with rotational anomaly of the intestines is an extremely rare condition. Although intestinal malrotation has been recognized as a cause of obstruction in infants and children and may be complicated by intestinal ischaemia, it is very rare in adults. When it occurs in the adult patient, it may present acutely as bowel obstruction or intestinal ischaemia or chronically as vague intermittent abdominal pain. Herein, we present an acute presentation of a case of situs inversus abdominus and intestinal malrotation with Ladd’s band leading to infarction of the intestine in a 32 year old woman.
Situs inversus; Malrotation; Ischaemia; Intestine
The atrial morphology and venous connections were assessed "blind" in 51 necropsy specimens from patients with visceral heterotaxy. This was compared with bronchial morphology as established by dissection. Six specimens were found to have both atria and bronchi in situs solitus or inversus, and were rejected. In the remainder, atrial isomerism was diagnosed, though this required minor revision of the atrial assessment in two patients. Thirty-four patients had isomeric right atria and bronchi, while 11 had isomeric left atria and bronchi. In seven cases, splenic status was unknown, but in seven of the remaining 38 (18.4%) atrial isomerism was not associated with either asplenia or polysplenia. Nevertheless, right isomerism was strongly associated with total anomalous pulmonary venous drainage (as is asplenia) and left isomerism was likewise associated with interruption of the inferior vena cava (as is polysplenia). Bilateral superior venae cavae and hepatic veins, and absence of the coronary sinus, were frequent in both forms of isomerism (as they are in asplenia and polysplenia). These findings suggest that atrial situs can be defined as solitus inversus, right isomerism, and left isomerism. This determination of atrial situs is quite independent of any other abnormalities of visceral situs. The high incidence of anomalies of both venous return and common atrium resulted in presumed complete mixing of blood at atrial level in all but one patient (97.8%), making the haemodynamic connection between atria and ventricles almost always ambiguous. To describe this anatomical connection as ambiguous when there are two ventricles present is therefore no more than recognition of anatomical and haemodynamic reality.
Situs inversus is a rare congenital anomaly characterized by the complete inversion of thoracic and abdominal organs. Liver transplantation in such patients or from donors in situs inversus is technically challenging because of the reversed anatomic structures. A small number of successful liver transplantation cases concerning situs inverus in either recipients or donors have been recently reported with different graft position and orientation. Here we reported an extremely rare case of liver retransplantation from an ABO incompatible situs inversus donor to an adult situs inversus recipient.
A 53-year-old complete situs inversus man developed graft failure due to severe biliary complication after his first liver transplantation from a situs solitus donor. Re-transplantation was performed using a graft liver from a likewise situs inversus donor. Although the blood type between donor and recipient was incompatible, the post-operative outcome was excellent under proper prophylaxis to the antibody-mediated rejection.
To the best of our knowledge, this is the first report of liver transplantation from situs inversus to situs inversus in adult recipient. Liver transplantation using situs matching donor makes the procedure much easier at the surgical point of view, which has a benefit of less potential surgical complications. Furthermore, ABO-incompatibility is acceptable for donor allocation in cases that both donor and recipient are situs inversus.
Liver transplantation; Retransplantation; Situs inversus; Abo incompatible
Laterality defects are rare in cattle and usually manifest as asplenia or
polysplenia syndrome. These syndromes may be associated with situs ambiguus,
which is a dislocation of some but not all internal organs. The objective of
this report was to describe the clinical and post-mortem findings including
the macroscopic and microscopic anatomy of selected organs in a cow with
polysplenia and situs ambiguus.
A 3.5-year-old Brown Swiss cow was referred to the Department of Farm
Animals, Vetsuisse Faculty, University of Zurich, because of poor appetite
and recurrent indigestion. A diagnosis of situs ambiguus was based on the
results of physical examination, ultrasonography, exploratory laparotomy and
post-mortem examination. The latter revealed that the rumen was on the right
side and lacked compartmentalisation. There were two spleens, one on the
left (26.5 x 12.0 cm) and one on the right (20.5 x 5.5 cm), and the omasum
was located craniolateral to the ruminoreticulum on the left. The abomasum
was located on the right, although it had initially been displaced to the
left. The three-lobed liver occupied the left and central cranioventral
aspect of the abdominal cavity (cavum abdominis). Only the right and left
hepatic veins (vena hepatica dextra and sinistra) drained into the thoracic
segment of the caudal vena cava (vena cava caudalis), and histological
changes in the liver were indicative of impaired haemodynamics. The
mesojejunum was not fused with the mesentery of the spiral loop (ansa
spiralis) of the ascending colon (colon ascendens). The latter was folded
and the transverse colon (colon transversum) ran caudal to the cranial
mesenteric artery (arteria mesenteria cranialis). Fibrotic constrictions
were seen in the lumen of the caecum and proximal loop (ansa proximalis) of
the ascending colon. Both kidneys were positioned retroperitoneally in a
lumbar position. The lumbar segment of the caudal vena cava did not descend
to the liver and instead drained into the right azygous vein (vena azygos
Recurrent digestive problems and poor production in this patient may have
been caused by a lack of rumen compartmentalisation, abnormal abomasal
motility, constrictions in the large intestine (intestinum crassum) and
fibrosis of the liver. The abomasum had abnormal motility most likely
because it was anchored inadequately and only at its cranial aspect to the
liver by the lesser omentum (omentum minus) and to the dorsal abdominal wall
and rumen by a short greater omentum (omentum majus).
Patients with genetic disorders associated with multiple congenital anomalies present unique challenges to the anesthesiologist. We report the successful perioperative management of a child with biliary atresia, situs inversus totalis, and Kartegener syndrome scheduled for corrective biliary surgery. We recommend that patients with multiple congenital anomalies need to be thoroughly and cautiously evaluated. The perioperative management should be individualized based on associated anomalies along with appropriate monitoring.
Anesthesia; biliary atresia; hepatobiliary surgery; Kartegener syndrome; situs inversus totalis
Situs inversus totalis is is a congenital anomaly associated with various visceral abnormalities, but there is no data about the relationship between secondary biliary cirrhosis and that condition. We here present a case of a 58 year-old female with situs inversus totalis who was admitted to our clinic with extrahepatic cholestasis. After excluding all potential causes of biliary cirrhosis, secondary biliary cirrhosis was diagnosed based on the patient's history, imaging techniques, clinical and laboratory findings, besides histolopathological findings. After treatment with tauroursodeoxycholic acid, all biochemical parameters, including total/direct bilirubin, alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase and gama glutamyl transferase, returned to normal ranges at the second month of the treatment. We think that this is the first case in literature that may indicate the development of secondary biliary cirrhosis in a patient with situs inversus totalis. In conclusion, situs inversus should be considered as a rare cause of biliary cirrhosis in patients with situs inversus totalis which is presented with extrahepatic cholestasis.
Situs inversus totalis; secondary biliary cirrhosis; tauroursodeoxycholic acid
A proposed mechanism in the pathogenesis of biliary atresia involves an initial virus-induced, progressive T cell–mediated inflammatory obliteration of bile ducts. The aim of this study was to characterize the inflammatory environment present within the liver of infants with biliary atresia to gain insight into the role of a primary immune-mediated process versus a nonspecific secondary response to biliary obstruction. Frozen liver tissue obtained from patients with biliary atresia, neonatal giant cell hepatitis, total parenteral nutrition (TPN)–related cholestasis, choledochal cysts, and normal control subjects was used for fluorescent immunohistochemistry studies of cellular infiltrates, cytokine mRNA expression, and in situ hybridization for localization of cytokine-producing cells. Immunohistochemistry revealed increases in CD8+ and CD4+ T cells and Kupffer cells (CD68+) in the portal tracts of biliary atresia. Reverse transcription–PCR analysis of biliary atresia tissue showed a Th1-type cytokine profile with expression of IL-2, interferon-γ, tumor necrosis factor-α, and IL-12. This profile was not seen in normal, neonatal hepatitis or choledochal cyst livers but was present in TPN-related cholestasis. In situ hybridization revealed that the Th1 cytokine–producing cells were located in the portal tracts in biliary atresia and in the parenchyma of TPN-related cholestasis. A distinctive portal tract inflammatory environment is present in biliary atresia, involving CD4+ Th1 cell–mediated immunity. The absence of similar inflammation in other pediatric cholestatic conditions suggests that the portal tract inflammation in biliary atresia is not a secondary response to cholestasis but rather indicates a specific immune response involved in the pathogenesis of biliary atresia.
Heterotaxy syndrome is a rare, complex, and confusing type of the situs anomalies. It is not possible to estimate the degree of lateralization, isomerism, and rotational variation in these types of cases. Heart and abdominal organ anatomy is specific to the individual, and it should be defined specifically on the basis of each case due to possible cardiac and extracardiac surgical interventions in patients with heterotaxy syndrome. Here, we present our findings obtained from a 58-year-old female patient with heterotaxy syndrome. The main components of this rare variation consist of right-hand-sided aorta, aortic arc, cardiac apex, gall bladder and left-hand-sided inferior vena cava, stomach, and spleen (polysplenia, 3 foci) according to the midline. Besides, the components include left-dominant liver, right-hand-sided large intestines, and left-hand-sided small intestines.
Polysplenia, as part of the heterotaxy syndrome, is a rare embryological disorder which results from failure of development of the usual left–right asymmetry of organs. It is often associated with cardiac and biliary abnormalities, which are the usual causes of death in early neonatal life. A congenitally short pancreas and abnormalities with portal vein formation, gut malrotations and inferior vena cava anomalies are known to be associated with this rare syndrome. We report a case of polysplenia in an adult female presenting with obstructive jaundice owing to choledocholithiasis, possibly formed by biliary stasis as a result of compression of the common bile duct by the preduodenal portal vein, and review the literature. The patient was also found to have complete agenesis of the dorsal pancreas on CT and endoscopic retrograde cholangiopancreatography.
“Heterotaxy is synonymous with `visceral heterotaxy' and `heterotaxy syndrome'. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as `situs solitus', nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as `situs inversus'.” The purpose of this manuscript is to review the data about heterotaxy in the STS Congenital Heart Surgery Database.
We examined all index operations in the STS Congenital Heart Surgery Database over 12 years from January 1, 1998 - December 31, 2009, inclusive. This analysis resulted in a cohort of 77,153 total index operations. Of these 77,153 index operations, 1,505 (1.95%) were performed in patients with heterotaxy. Of the 1,505 index operations performed in patients with heterotaxy, 1,144 were in patients with “asplenia” and 361 were in patients with” polysplenia”.
In every STS–EACTS Congenital Heart Surgery Mortality Category (2009) (STS–EACTS Category), discharge mortality is higher in patients with heterotaxy compared to patients without heterotaxy. Discharge mortality after Systemic to pulmonary artery shunt is 6.6% in a cohort of all Single Ventricle patients except those with heterotaxy, while it is 10.8% in Single Ventricle patients with heterotaxy. Discharge mortality after Fontan is 1.8% in a cohort of all Single Ventricle patients except those with heterotaxy, while it is 4.2% in Single Ventricle patients with heterotaxy.
The STS Database is largest Congenital Heart Surgery Database in North America. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that heterotaxy is a challenging problem with increased discharge mortality in most subgroups.
heterotaxy syndrome; isomerism; asplenia; polysplenia; database; congenital heart disease; outcomes; cardiac surgery; results of treatment
Biliary atresia is a neonatal disorder characterized by aggressive fibroinflammatory obliteration of the biliary tract. Approximately 20 percent of biliary atresia patients demonstrate left-right laterality defects (syndromic biliary atresia). Cilia participate in important physiologic functions in cholangiocytes, and since some ciliopathies have been associated with both laterality defects and hepatic fibrosis, we hypothesized that patients with syndromic biliary atresia exhibit abnormalities of cholangiocyte cilia that disrupt cholangiocyte homeostasis. Nine biliary atresia specimens were studied, including pre-Kasai diagnostic biopsies (n=7) and liver explants (n=2). Five specimens were from patients with laterality defects. These were compared to normal pediatric livers as well as livers affected by primary sclerosing cholangitis, Wilson’s disease, and cardiac cirrhosis. Biopsy sections were stained with antibodies against keratin 19 (a cholangiocyte marker) and acetylated α-tubulin (a cilia marker) and were visualized by confocal microscopy. Computer-assisted relative quantification was used to compare staining of cilia within bile ducts among samples. Surprisingly, cilia in biliary atresia specimens were significantly shorter, abnormal in their orientation, and less abundant compared to normal liver and disease controls regardless of the presence of a laterality defect.
There are significant abnormalities of cholangiocyte cilia in both syndromic and non-syndromic biliary atresia livers compared to normal livers and livers affected by other cholestatic diseases. While this may result from severe cholestasis or inflammation, it may also reflect common mechanistic pathways in different forms of biliary atresia and may have important implications for understanding the progression of the disease.
liver fibrosis; ciliopathy; laterality; situs inversus; cholestasis
The etiology of intestinal atresia remains elusive but has been ascribed to a number of possible events including in utero vascular accidents, failure of recanalization of the intestinal lumen and mechanical compression. Another such event that has been postulated to be a cause in atresia formation is disruption in notochord development. This hypothesis arose from clinical observations of notochord abnormalities in patients with intestinal atresias as well as abnormal notochord development observed in a pharmacological animal model of intestinal atresia. Atresias in this model result from in utero exposure to Adriamycin, wherein notochord defects were noted in up to 80% of embryos that manifested intestinal atresias. Embryos with notochord abnormalities were observed to have ectopic expression of Sonic Hedgehog (Shh) which in turn was postulated to be causative in atresia formation. We were interested in determining whether disruptions in notochord development or Shh expression occurred in an established genetic model of intestinal atresia and utilized the Fibroblast Growth Factor Receptor 2IIIb homozygous mutant (Fgfr2IIIb−/−) mouse model. These embryos develop colonic atresias (100% penetrance) and duodenal atresias (42% penetrance).
Wild-type and Fgfr2IIIb−/− mouse embryos were harvested at E10.5, E11.5, E12.5 and E13.5. Whole mount in situ hybridization was performed on E10.5 embryos for Shh. Embryos at each time point were harvested and sectioned for H&E staining. Sections were photographed specifically for the notochord and resulting images reconstructed in 3-D using Amira software. Colons were isolated from wild-type and Fgfr2IIIb−/− embryos at E10.5, then cultured for 48 hours in matrigel with FGF10 in the presence or absence of exogenous SHH protein. Explants were harvested, fixed in formalin and photographed.
Fgfr2IIIb−/− mouse embryos exhibit no disruptions in Shh expression at E10.5, when the first events in atresia formation are known to occur. Three-dimensional reconstructions failed to demonstrate any anatomical disruptions in the notochord by discontinuity or excessive branching. Culture of wild-type intestines in the presence of Shh failed to induce atresia formation in either the duodenum or colon. Cultured Fgfr2IIIb−/− intestines developed atresias of the colon in either the presence, or absence, of Shh protein.
Although disruptions in notochord development can be associated with intestinal atresia formation, in the Fgfr2IIIb−/− genetic animal model neither disruptions in notochord development nor the presence of exogenous Shh protein are causative in the formation of these defects.
Situs ambiguous is rare congenital anomaly in adults. In 2 adult patients who admitted for different cardiac problems, situs ambiguous with polysplenia was detected. A 42-year-old male admitted for radio frequent catheter ablation of atrial fibrillation, and he had left-sided inferior vena cava (IVC), hepatic segment of IVC interruption with hemiazygos continuation, multiple spleens and intestinal malrotation. And in a 52-year-old female case who was hospitalized due to infective endocarditis after implanting pacemaker for sick sinus syndrome, multiple spleens, left-sided stomach, bilateral liver with midline gallbladder, and left-sided IVC were found. Those findings were consistent with situs ambiguous with polysplenia, but their features were distinctive.
Situs ambiguous; Polysplenia syndrome; Adult
The Situs viscerum inversus associated with anomalies of intestinal rotation and fixation is an extremely rare condition. To the authors’ knowledge, this is the first report of colon cancer associated with intestinal malrotation and mesenterium ileocolicum commune.
A 34-year-old man with a 2-month history of diarrhea associated with abdominal pain and weight loss underwent abdominal ultrasonography, colonscopy with biopsies and abdominal computed tomography scan with intravenous contrast. A right colonic neoplasm was diagnosed, observed only at surgery, as neither computed tomography or ultrasonography showed the intestinal malrotation. Particularly, the third and the fourth part of the duodenum descended vertically, without Treitz’s ligament in support to the duodeno-jejunal flexure. The small bowel and the colon were located in the right and left side of the abdominal cavity, respectively.
The anomaly of situs viscerum inversus influenced the surgical strategy in this case because of the vascular and lymphatic anomalies. Lymphatic vessels were therefore marked with subserosal injection of patent blue in the proximity of the tumor. Subsequently, right colectomy was performed. Colectomy extended from the distal ileum to the descending colon, by ligature of the right colic artery and vein at the origin from the superior mesenteric vessels. Patent blue guided lymphadenectomy was also performed with curative intent. Finally, a mechanical ileo-colic anastomosis was carried out. After right colectomy and ileo-descending anastomosis, the Ladd’s procedure for intestinal malrotation was unnecessary. The authors believe that this strategy, despite the anatomical difficulties, represents an effective procedure for the radical surgical treatment of the right colon cancer associated with anomalies of intestinal rotation and fixation.
Situs inversus with dextrocardia occurs in approximately one in 10 000 patients. Successful stent deployment for the treatment of unstable angina and situs inversus is presented. Three technical challenges associated with the procedure are highlighted. Firstly, the successful choice of diagnostic and interventional catheters is based on an understanding of the orientation of the aortic arch. With a right sided aorta Judkins catheters should be successful. Secondly, image reversal is not necessarily required for image interpretation. Thirdly, successful coronary engagement with catheters requires rotation in a direction opposite to that normally used.
Keywords: dextrocardia; situs inversus; stenting; unstable angina
This review presents the cardiac and non-cardiac malformations in 60 cases with asplenia and polysplenia with special reference to distinguishing factors which may be helpful in the clinical recognition of these syndromes. The asplenia cases were predominantly male and presented with cyanosis. They frequently had transposition of the great arteries (72%) with pulmonary stenosis or atresia (88%) and total anomalous pulmonary venous drainage (72%). Deaths were caused by cardiac failure and anoxia in 57 per cent of cases. Most of the patients died in the first year of life (79%), but longer survival is possible in the asplenia syndrome. The polysplenia cases were predominantly female and survived longer. The characteristic clinical findings were the relatively more benign presenting signs and the leftward or superiorly orientated P wave axis on the electrocardiogram. Conotruncal abnormalities were less common and total anomalous pulmonary venous drainage did not occur. On angiography the inferior vena caval drainage via the azygos system was clearly identified and this was present in all cases at surgery. Our study indicated that the cardiac anomalies in polysplenia were less severe than they were in asplenia and therefore the prognosis in the former syndrome is likely to be more favourable. Three families had two affects sibs but no single genetic factor could be identified. The aetiology of these syndromes remains undetermined.
Correlate the anatomic features of atrioventricular septal defect with echocardiographic images.
Materials and methods
Sixty specimen hearts were studied by sequential segmental analysis. Echocardiograms were performed on 34 patients. Specimen hearts with findings equivalent to those of echocardiographic images were selected in order to establish an anatomo-echocardiographic correlation.
Thirty-three specimen hearts were in situs solitus, 19 showed dextroisomerism, 6 were in situs inversus and 2 levoisomerism. Fifty-eight had a common atrioventricular valve and 2 had two atrioventricular valves. Rastelli types were determined in 21 hearts. Nine were type A, 2 intermediate between A and B, 1 mixed between A and B, 4 type B and 5 type C. Associated anomalies included pulmonary stenosis, pulmonary atresia atrial septal defect, patent ductus arteriosus and anomalous connection of pulmonary veins. Echocardiograms revealed dextroisomerism in 12 patients, situs solitus in 11, levoisomerism in 7 and situs inversus in 4. Thirty-one patients had common atrioventricular valves and three two atrioventricular valves. Rastelli types were established in all cases with common atrioventricular valves; 17 had type A canal defects, 10 type B, 3 intermediate between A and B, 1 mixed between A and B and 3 type C. Associated anomalies included regurgitation of the atrioventricular valve, pulmonary stenosis, anomalous connection of pulmonary veins, pulmonary hypertension and pulmonary atresia.
Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography.
This report is to present and discuss an extremely rare association of situs inversus with duodenal atresia in an 11-day-old male neonate born full term and weighing 1.9 kg. The baby presented with recurrent bilious vomiting. Babygram revealed situs inversus and duodenal obstruction. Echocardiography showed dextrocardia with a small ASD. Exploration confirmed a duodenal diaphragm with a central perforation between the third and fourth part of the duodenum and situs inversus. The literature search revealed 20 cases reported so far.
Babygram; congenital duodenal obstructions; reverse double bubble; situs inversus
Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.
Polysplenia syndrome, defined as the presence of multiple spleens of almost equal volume, is a rare condition involving congenital anomalies in multiple organ systems. We report this anomaly in a 41-year-old female who underwent a left lateral sectionectomy due to recurrent cholangitis and impacted left lateral duct stones. Polysplenia syndrome with preduodenal vein was diagnosed preoperatively by computed tomography (CT) and surgery was done safely. Although the polysplenia syndrome with preduodenal portal vein (PDPV) in adult is rarely encountered, surgeons need to understand the course of the portal vein and exercise caution in approaching the biliary tract.
Polysplenia; Polysplenia syndrome; Preduodenal portal vein; Intrahepatic duct stones; Congenital anomaly