Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2017  |  Volume : 22  |  Issue : 1  |  Page : 53--54

Obstructive jaundice caused by hepatic torsion in an infant with congenital diaphragmatic hernia


Iftikhar Ahmad Jan1, Basmah Al Hamoudi1, Abdulla AlJunaibi2, Abdelmatlub Ben Mussa2,  
1 Department of Pediatric Surgery, Zayed Military Hospital, Abu Dhabi, United Arab Emirates
2 Department of Pediatric Medicine, Zayed Military Hospital, Abu Dhabi, United Arab Emirates

Correspondence Address:
Iftikhar Ahmad Jan
Department of Pediatric Surgery, Zayed Military Hospital, Abu Dhabi
United Arab Emirates

Abstract

Right sided congenital diaphragmatic hernia may cause biliary obstruction. We present a 2 months female infant who had respiratory distress and persistent jaundice since birth. Investigations suggested direct hyperbilirubinemia, right-sided diaphragmatic hernia with liver herniation in the thorax, and intra- and extrahepatic biliary dilatation. Laparotomy showed herniation of liver in the chest with hepatic torsion of about 180΀ causing obstruction of bile ducts. Liver torsion was corrected and liver relocated in the abdomen. An operative cholangiogram confirmed free passage of contrast to the intestine after correction of hepatic torsion. Repair of the diaphragmatic hernia was performed. Only skin closure was performed leaving a ventral hernia to avoid abdominal compartment syndrome. Postoperatively, the baby was kept on ventilator for 2 days and then extubated. She showed rapid recovery and was discharged in a stable condition. The ventral hernia was repaired at the age of 6 months. Her total bilirubin levels dropped gradually from 12.50 mg/dl into its normal values within 3 months.



How to cite this article:
Jan IA, Al Hamoudi B, AlJunaibi A, Mussa AB. Obstructive jaundice caused by hepatic torsion in an infant with congenital diaphragmatic hernia.J Indian Assoc Pediatr Surg 2017;22:53-54


How to cite this URL:
Jan IA, Al Hamoudi B, AlJunaibi A, Mussa AB. Obstructive jaundice caused by hepatic torsion in an infant with congenital diaphragmatic hernia. J Indian Assoc Pediatr Surg [serial online] 2017 [cited 2022 Jan 25 ];22:53-54
Available from: https://www.jiaps.com/text.asp?2017/22/1/53/194627


Full Text

 Introduction



Most babies with congenital diaphragmatic hernia (CDH) are now diagnosed on antenatal ultrasound, however, they may present with respiratory distress after birth. Delayed presentation is also seen if the baby has normal respiratory reserves. Right diaphragmatic hernia with persistent jaundice beyond the newborn period is not common. [1],[2] We present a rare case of right diaphragmatic hernia with hepatic torsion causing biliary obstruction and obstructive jaundice. To our knowledge, this anomaly has not been reported in the available literature.

 Case Report



A 2-month-old baby girl was referred with jaundice, acholic stool, and highly colored urine since birth. She was admitted to another hospital with fever, cough and poor intake and treated as a case of pneumonia, right diaphragmatic hernia, and cholestatic jaundice. She was referred to us for further evaluation of jaundice. On admission, the baby was moderately jaundiced but had stable vitals. There were decreased breath sounds on the right basal zone. Abdominal examination revealed palpable liver and normal bowel sounds. Rest of systemic examination was unremarkable. The total bilirubin was 12.50 mg/dl, alanine transaminase 244 U/L, aspartate transaminase 500 U/L, alkaline phosphatase 485 U/L, and albumin 3.2 g/dl. X-ray revealed marked elevation of the right dome of the diaphragm with upward migration of the liver. Ultrasound, computed tomography abdomen and magnetic resonance (MR) cholangiogram showed CDH with a large segment of the liver herniated into the right chest and severe intra and extrahepatic bile duct dilatation.

Surgery was planned with the diagnosis of right diaphragmatic hernia and biliary obstruction. A right upper abdominal transverse laparotomy performed. The child had right diaphragmatic hernia with about 6 cm posterolateral defect, and nearly two-third of the liver was seen in the thoracic cavity. The right lobe of the liver had nearly 180° counter clock rotation causing obstruction to the biliary outflow [Figure 1]. The gallbladder was located in the posteromedial location of the thorax. The right hepatic torsion was corrected, and liver was relocated into the abdomen. After relocation, it was noticed that the hepatobiliary anatomy has been restored [Figure 2]. An operative cholangiogram showed that there were intra and extrahepatic biliary ducts dilatation with dilated common bile duct (CBD) but free flow of the contrast into the intestine. Repair of the diaphragmatic hernia was performed, and a liver biopsy was taken. It was not possible to close the abdominal wall without significant tension, therefore, only skin closure was performed without repair of the abdominal wall muscles. In the postoperative period, the child was kept on assisted ventilation for 2 days, and then gradually weaned off the ventilator. She was discharged in a stable condition after 5 days of surgery. Baby was readmitted at the age of 6 months; repair of ventral hernia was performed and later discharged home in a stable condition. The total bilirubin dropped from 12.50 mg/l into its normal values within 3 months after initial surgery. A repeat ultrasound at 6 months showed normal liver architecture with no evidence of biliary dilatation.{Figure 1}{Figure 2}

 Discussion



The association of jaundice with CDH is well-established and the common causes are herniation of the hepatobiliary apparatus in the chest, [1],[2],[3],[4] malrotation causing occlusion of CBD, [5] neonatal hepatitis, [6] Biliary atresia, and Cytomegalovirus infections [6] to mention a few. Obstructive jaundice has also been reported in other diaphragmatic defects such as hiatal and morgagni hernias. [7],[8] To our knowledge, CHD with liver herniation in the chest and causing hepatic torsion and obstruction to the biliary outflow has not been reported in the available literature. This is a rare case, which presented late with jaundice and respiratory symptoms. X-ray chest picked up the diaphragmatic hernia. Ultrasonography and MR cholangiogram were suggesting dilated hepatobiliary system and dilated CBD with the impression of choledochal cyst. Reviewing the MR cholangiogram retrospectively, the gall bladder was seen on the medial side of the herniated right lobe but due to the distorted anatomy, it was not possible to make a diagnosis of hepatic torsion. It was only after exploration and liver reduction in the abdomen that we found there was liver torsion causing biliary obstruction. Once the torsion was corrected the operative cholangiogram showed the free passage of contrast in the bowel excluding other possible causes such as choledochal cyst and extrahepatic biliary atresia.

Another difficulty faced after liver reduction in the abdomen was small abdominal capacity to close the abdomen without tension. With primary closure, there was high-risk of abdominal compartment syndrome, and therefore, it was decided to leave a ventral hernia for later repair. This approach definitely helped the baby, and she recovered soon, and the ventral hernia was repaired easily at the age of 6 months.

There are several lessons to learn from this case. Diaphragmatic hernia can present late with minimal symptoms. Persistent jaundice in a baby beyond neonatal period needs proper evaluation. The presence of liver in the chest can cause biliary obstruction and in worst-case scenario even torsion of liver. Leaving a ventral hernia for later repair in large CDH with limited abdominal cavity is a feasible option.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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