Biliary Atresia With Situs Inversus Abdominalis Managed by Kasai Portoenterostomy: A Case Report From Muhimbili National Hospital, Tanzania

ABSTRACT

Biliary atresia associated with situs inversus abdominalis is a rare congenital combination that complicates diagnosis and surgical orientation. Early recognition, meticulous preoperative planning, and timely Kasai portoenterostomy can achieve favorable short‐term outcomes. Surgeons should consider anatomical variations to optimize operative safety and improve biliary drainage and prognosis.

1. Introduction

Biliary atresia (BA) is an uncommon and fatal illness affecting roughly 1 in 10,000 to 15,000 live newborns [1, 2]. The Kasai portoenterostomy is the recommended course of treatment, especially if it is performed when the child is 60 days old or less [3, 4, 5]. Situs inversus abdominalis is a rare congenital anomaly whereby the abdominal organs are positioned in a mirror image of the normal anatomy, affecting about 1 in 10,000 people [6, 7]. Co‐occurrence of situs inversus abdominalis with BA is rare, and the atypical anatomy may make surgery more challenging. Since there aren't many cases reported in the literature, this case highlights the importance of careful preoperative evaluation and surgical modification.

2. Case History and Examination

An 11‐week‐old female baby was brought to our facility after she presented with jaundice since Day 4 of life that was associated with pale stool and dark urine. Her perinatal history was uneventful, and she was born full‐term via cesarean section due to poor progress of labor, and weighed 3.0 kg. On examination she was jaundiced, had hepatomegaly (liver palpable on the left side, 5 cm below the costal margin) with no clinically detected ascites. Left side heart sounds were audible when the chest was auscultated.

3. Differential Diagnosis, Investigations, and Treatment

Liver function tests showed elevated transaminases and direct hyperbilirubinemia, the TORCH screen was negative except for CMV IgG that was positive (previous exposure) (Table 1). Abdominal ultrasonography showed the spleen on the right and the liver on the left with atretic gallbladder suggesting situs inversus abdominalis with biliary atresia. The screening echocardiography showed levocardia with small ASD and PDA ruling out situs inversus totalis. MRCP also showed situs inversus of the abdominal organs including liver, spleen, and pancreas with atretic gallbladder and biliary ducts (Figure 1). Hepatobiliary HIDA scan was not done in our setting as it is not available.

TABLE 1.

Laboratory investigations on admission, 2 weeks, and 6 weeks after Kasai portoenterostomy.

Investigation	Reference range	Admission	2 weeks post‐Kasai	6 weeks post‐Kasai	Interpretation/trend
Coagulation profile
Prothrombin time (PT), s	9.5–13.5	9.8	16.9	12.4	Normalized
Activated partial thromboplastin time (aPTT), s	25–35	23.7	114.0	31.2	Normalized
International normalized ratio (INR)	0.8–1.2	0.92	1.67	1.08	Normalized
Liver function tests
Total bilirubin (μmol/L)	3–20	174.0	168.0	24.0	Marked improvement
Direct bilirubin (μmol/L)	< 5	135.7	136.0	8.0	Near normal
Aspartate aminotransferase (AST), U/L	10–50	284.49	225.44	42.0	Normalized
Alanine aminotransferase (ALT), U/L	7–56	225.40	137.42	35.0	Normalized
Alkaline phosphatase (ALP), U/L	150–420	465.02	187.81	170.0	Normalized
Gamma‐glutamyl transferase (GGT), U/L	< 150	2466.25	2015.06	145.0	Normalized
Albumin (g/L)	35–50	39	31	40	Normalized
Infectious/TORCH screening (admission only)
Hepatitis A IgM	Negative	Negative	—	—	No acute infection
Hepatitis B surface antigen (HBsAg)	Negative	Negative	—	—	No evidence of hepatitis B
Hepatitis C antibody	Negative	Negative	—	—	No evidence of hepatitis C
Cytomegalovirus (CMV) IgG	Negative	Positive	—	—	Previous exposure/passive maternal antibody
Cytomegalovirus (CMV) IgM	Negative	Negative	—	—	No acute CMV infection
Toxoplasma IgM	Negative	Negative	—	—	No acute toxoplasmosis
Rubella IgM	Negative	Negative	—	—	No acute rubella infection

FIGURE 1.

In (A–C), the MRI/MRCP shows no bile flow in the bile ducts with the right liver lobe on the left side; in (D), the extrahepatic bile ducts are not visible and atretic gallbladder is seen.

All preoperative laboratory results were within normal limits, and the patient was ready for a Kasai portoenterostomy after the diagnosis of biliary atresia was made.

At 12 weeks of age, laparotomy for Kasai portoenterostomy was performed. Intraoperative exploration revealed situs inversus abdominalis with a fibrotic extrahepatic biliary remnant at the porta hepatis, consistent with biliary atresia (Figures 2, 3, 4). Intraoperative cholangiography was not performed because the diagnosis had been established based on clinical presentation, laboratory findings and preoperative imaging. Therefore, Kasai portoenterostomy was performed with Roux‐en‐Y loop designed to fit the inverted anatomy during a Kasai portoenterostomy (Figure 5). Abdominal drain was applied.

FIGURE 2.

During surgery, picture (A) depicts the liver's right lobe on the left (orange arrow), while image (B) shows the spleen on the right (blue arrow).

FIGURE 3.

Intraoperative images (A) and (B), with the stomach on the right (black arrow) and the caecum on the left (blue arrow), respectively.

FIGURE 4.

Intraoperative picture (A) displays the atretic gall bladder (yellow arrow) with white bile, while image (B) shows the portal hepatis (green arrow) following biliary dissection.

FIGURE 5.

Successful Kasai portoenterostomy (green arrow) in intraoperative pictures (A) and (B).

Following surgery, the baby was admitted to a pediatric surgical ward and kept on IV antibiotics and painkillers. Bile flow was established within 7 days following surgery, stool color improved, and serum bilirubin levels gradually dropped. After being discharged on Day 14 after surgery, the child is currently being watched for improving liver function parameters.

4. Conclusion and Results

Biliary atresia co‐occurring with situs inversus abdominalis is an extremely rare and complex condition. Thorough preoperative evaluation and early suspicion based on atypical clinical signs and findings are necessary when planning a surgical intervention. Despite anatomical challenges, the Kasai portoenterostomy can be performed successfully with the appropriate modifications. A multidisciplinary approach involving pediatric surgery, hepatology, radiology, and cardiology is necessary for optimal outcomes.

5. Discussion

An uncommon, progressive fibro‐obliterative disease of the extrahepatic biliary tree, biliary atresia (BA) usually manifests in the neonatal period [8, 9]. If not intervened, it usually causes biliary cirrhosis, cholestasis, and death within the first 2 years of life [10, 11, 12]. In contrast to situs inversus totalis, situs inversus abdominalis is a congenital condition in which the thoracic organs are in their normal placements, but the abdominal organs are displaced from their normal anatomical positions [6, 13, 14]. BA with situs inversus abdominalis is very rare and presents difficulty in diagnosis and operation [6]. This combination is believed to be caused by errors in embryologic development during the early stages of pregnancy and is occasionally associated with other abnormalities such as polysplenia, intestinal malrotation, or congenital cardiac issues [7, 13].

The migration and rotation of the midgut and foregut during early embryonic development determine the proper positioning of abdominal organs. Situs inversus is caused by aberrant expression of laterality‐determining genes, such as LEFTY, NODAL, and ZIC3, which are important in the determination of the left–right axis [15, 16, 17]. Disruption in these pathways may have parallel effects on the development of the biliary system. Some experts speculate that biliary atresia may be a part of certain laterality issues, especially in patients with situs abnormalities. This is supported by the higher frequency of associated cardiac, splenic, and vascular abnormalities in the syndromic type of biliary atresia, termed Biliary Atresia Splenic Malformation (BASM) syndrome [18]. Echocardiography demonstrated no congenital cardiac abnormalities, and the spleen was normal in morphology and position relative to the reversed abdominal anatomy. Consequently, the patient did not fulfill the clinical features of BASM syndrome.

Jaundice in neonates lasting more than 2 weeks, acholic (pale) feces, dark urine, hepatomegaly, and insufficient weight gain are clinical manifestations of BA with situs inversus that are comparable to those of normal BA. Clinical examination results, however, may reveal inverted anatomy, such as a palpable liver on the left side, the spleen on the right side, and heart sounds on the left side that differentiate situs inversus abdominalis from full situs inversus totalis. Associated anomalies (such as intestinal malrotation and vascular anomalies) should always be investigated due to their surgical implications.

The left liver and right spleen may be visible on an ultrasound. The inverted architecture does not make it difficult to interpret the HIDA scan, which indicates that there is no bile moving into the small bowel. Although situs inversus does not preclude interpretation of hepatobiliary scintigraphy, the mirror‐image anatomy requires careful anatomical orientation during image interpretation. HIDA findings should be interpreted in conjunction with ultrasonography and other imaging modalities because HIDA primarily provides functional rather than anatomical information. MRI/MRCP aids in confirming situs inversus and identifying the biliary tract's structure. To confirm the position of the thoracic organs and look for congenital cardiac anomalies, echocardiography is necessary. Histological indicators of biliary atresia include fibrosis, portal tract edema, and bile duct proliferation found in liver biopsies. Intraoperative cholangiography is the gold standard for identifying the kind of biliary atresia and assessing the degree of biliary blockage [13, 19, 20]. Intraoperative cholangiography was not carried out due to the hypoplastic biliary tree and deformed hepatobiliary anatomy from situs inversus abdominalis. Based on intraoperative results, it was necessary to proceed straight to Kasai portoenterostomy due to difficult cannulation and the necessity to avoid needless delay.

The gold standard for treating biliary atresia is Kasai portoenterostomy, which should be performed before 60 days of life for optimal outcomes [3, 4, 5]. Although our patient underwent surgery at 12 weeks (84 days), satisfactory early postoperative recovery with progressive resolution of jaundice was achieved. Nevertheless, the delayed timing may adversely influence long‐term outcomes, necessitating close follow‐up to monitor liver function, growth, and the development of portal hypertension. The delay in surgical intervention was primarily attributable to late referral to our tertiary pediatric surgical center. Although jaundice was noted from the fourth day of life, the infant initially received care at peripheral health facilities where prolonged neonatal jaundice was managed conservatively before biliary atresia was suspected. Referral to our facility occurred only after persistent jaundice, acholic stools, and cholestatic liver dysfunction became evident. This reflects the diagnostic challenges and limited awareness of biliary atresia in resource‐limited settings, where delayed recognition and referral remain common causes of late Kasai portoenterostomy.

In order to prevent internal herniation and ensure proper bile drainage, the Kasai technique requires careful preoperative imaging to understand anatomical differences, modifications to intraoperative ocular orientation for dissection and Roux‐en‐Y loop construction, and attention to mesenteric orientation for people with situs inversus abdominalis [6, 19, 21]. Skilled surgical teams and technical modifications are needed since surgical landmarks are inverted. Roux limb orientation, portal plate isolation, and fibrotic porta hepatis dissection are all impacted by the reversal of organ organization.

The prognosis for BA with situs inversus is similar to that of isolated BA if the Kasai surgery is successful [22, 23, 24]. The prognosis is determined by a number of parameters, including age at surgery, the severity of liver fibrosis, the presence of associated abnormalities, and postoperative complications including cholangitis or portal hypertension [25, 26, 27]. Liver transplantation remains the best option in situations of failed Kasai or increasing liver failure [4, 28, 29]. Patients with situs abnormalities may potentially have problems during liver transplantation due to their aberrant organ orientation and vasculature [30, 31].

Author Contributions

Rajabu Athumani Bakari: conceptualization, data curation, formal analysis, investigation, methodology, project administration, validation, visualization, writing – original draft, writing – review and editing. Alfred Chibwae: conceptualization, data curation, formal analysis, investigation, methodology, validation, visualization, writing – review and editing. Brian Kimanthi: conceptualization, data curation, formal analysis, investigation, methodology, validation, visualization, writing – review and editing. Tariq Aziz: conceptualization, data curation, formal analysis, investigation, validation, visualization, writing – review and editing. Georvin Marco Haule: conceptualization, data curation, formal analysis, investigation, methodology, validation, visualization, writing – review and editing. Novath Ngowi: conceptualization, data curation, formal analysis, investigation, methodology, validation, visualization, writing – review and editing. Ally Hamisi Mwanga: conceptualization, data curation, formal analysis, investigation, methodology, supervision, validation, visualization, writing – review and editing. Zaitun M. Bokhary: conceptualization, data curation, formal analysis, investigation, methodology, supervision, validation, visualization, writing – review and editing.

Funding

The authors have nothing to report.

Ethics Statement

This case report is a single rare case that emerged during our routine surgical practices. Hence, this case report was exempted from the ethical approval at our institution review board.

Consent

Written informed consent was obtained from parents to publish this case report in accordance with the journal's patient consent policy.

Conflicts of Interest

The authors declare no conflicts of interest.

Data Availability Statement

The data that supports the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restriction.