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Direct hyperbilirubinemia as a sole presentation of annular pancreas

 

Bilal Mirza, Afzal Sheikh
Department of Pediatric Surgery, The Children’s Hospital & The Institute of Child Health,
Lahore, Pakistan

 

Correspondence  

Bilal Mirza
Department of Pediatric Surgery, The Children’s Hospital & The Institute of Child Health, Lahore, Pakistan
Tel: +923454772583
Email: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Annular pancreas is a congenital malformation of pancreas often associated with congenital duodenal stenosis. Its main presentation is mostly confined to very initial days of life with clinical features of duodenal obstruction. Rarely the presentation is solely obstructive jaundice. A 20-day old female was admitted in our hospital with complaints of pathological jaundice starting from second day of life. Ultrsonographic and radiographic evidence of dilated duodenum led us to the suspicion of partial duodenal obstruction. At operation annular pancreas was encountered and managed with an annulus-bypass duodeno-duodenostomy. Symptoms relieved after the surgical intervention. Annular pancreas may be enlisted in the causes of obstructive jaundice.

key words: annular pancreas, hyperbilirubinemia, jaundice

 

Introduction

Congenital duodenal obstruction is one of frequent causes of surgical interventions in neonates. In annular pancreas the duodenum is encircled by the pancreatic tissue all around and frequently associated with congenital duodenal stenosis. The encircling by pancreas and associated intrinsic duodenal stenosis led to the early presentation of the patient with clinical features of duodenal obstruction [1].

The usual presentation is with bilious vomiting and intolerance to feed, however, the patient may have associated prolonged jaundice. Direct hyperbilirubinemia as a sole presentation of annular pancreas is scarcely reported [2]. A case of annular pancreas presented with direct hyperbilirubinemia as a sole presentation, is being reported.

Case presentation

A 20-day old female neonate having a weight of 3kg was referred to our hospital from a private hospital. The baby was born to a 20-year old primigravida, in that hospital, through a Caesarian section due to cervical dystocia. The patient developed jaundice on the second day of life. The baby was tolerating mother feed, thus, the jaundice was considered physiological and patient discharged after counseling regarding the jaundice. The patient was re-admitted in the same hospital due to failure of resolution of jaundice on 10 day of life. The baby also had two episodes of bulky bilious vomiting, few days th back, but the baby was tolerating the feeds and passing stool. The color of stool was some times greenish and other times pale. Her laboratory investigations revealed a total bilirubin of 10mg/dl and the direct bilirubin was 4.8mg/dl. Ultrasonography was unremarkable regarding any hepatobiliary pathology.

Patient was labeled as a case of pathological jaundice and referred to our institution at 20 day of life. During index admission in our institution, the weight of th baby was 2.8kg and the total bilirubin was 11mg/dl with conjugated bilirubin of 4.5mg/dl. The ultrasound abdomen revealed dilated stomach and duodenum and no other abnormality. We requested erect abdominal radiograph that also delineated the dilated stomach and duodenal shadow. A contrast upper GIT study was performed that showed dilated duodenum but the contrast was also passing into the small intestine. A diagnosis of partial congenital duodenal obstruction was made and the parents counseled about the necessary operation.

At operation the stomach and duodenum were much dilated and pancreatic tissue found encircling the duodenum all around ( fig. 1). A diamond shaped duodeno-duodenostomy was performed. The post operative recovery was uneventful. The patient was started orally on 6 post operative day. The patient showed a substantial improvement in jaundice and it took two weeks to become clinically anicteric. The baby is on follow up and symptoms free for last 6 months.

9 JPSS 8 1 2010-9

Figure 1: Operative view of the patient showing annular pancreatic  (AP) encircling the duodenum all around and the dilated duodenum (D) can also be appreciated.

Discussion

 Annular pancreas develops before 8 week of gestation due to failure of ventral bud to rotate and fuse with dorsal bud, thus encircling the duodenum. Very often, it is associated with intrinsic duodenal stenosis resulting in partial or complete duodenal obstruction; hence, increases the likelihood of an early presentation. But, few cases have been reported in their adulthood or even in senile age. These findings might be attributed to the absence of intrinsic duodenal stenosis. The annular pancreas may also be associated with duodenal atresia, malrotation, and sometimes cardiac anomalies [1-3].

The clinical features of annular pancreas are bilious emesis (obstruction distal to ampulla of Vater), failure to thrive (in prolonged history) and intolerance to feed. In case of persistent and frequent emesis, the patient may present with electrolyte disturbances and even in a state of shock. Jaundice neonatorum is usually prolonged in case of any congenital duodenal obstruction. In our case the patient was tolerating the feeds and there were only few episodes of emesis thus precluding a significant weight loss, electrolytes and acid base imbalances. The prolonged jaundice worried the parents for that they had to seek the medical advice. A number of case reports have been published indicating association of obstructive jaundice with annular pancreas; but most of cases belonged to the adult patients having obstructive jaundice secondary to pancreatic head malignancies [4, 5]. In contrast, our case is unique as there was no cause of direct hyperbilirubinemia identified and patient improved after surgical management of annular pancreas. In our opinion, annular pancreas with duodenal stenosis has some obstructive consequences on the biliary drainage and its substantial improvement after duodeno-duodenostomy is a point towards its proof. However, patient may need an evaluation by magnetic resonance cholangio-pancreatography (MRCP) to rule out any pancreato-biliary ductal abnormality missed on ultrasonography.

It is inferred that patients with direct hyperbilirubinemia may have duodenal obstruction as a causative factor. Dilated stomach and duodenum, on abdominal radiograph and ultrasound, can aid in the diagnosis of congenital duodenal pathology.

 

 

 

References 

  1. Applebaum H, Lee SL, Puapong DP. Duodenal atresia and stenosis. In: O’Neill JA Jr, Rowe MI, Grosfeld JL, et al, editors. Pediatric surgery, 6th ed. Chicago: Year Book; 2006. p. 1260-9
  2. Kandpal H, Bhatia V, Garg P, Sharma R. Annular pancreas in an adult patient: diagnosis with endoscopic ultrasonography and magnetic resonance cholangio-pancreatography. Singapore Med J 2009; 50:e29
  3. Mirza B, Ijaz L, Saleem M, Sheikh A. Multiple associated anomalies in a single patient of duodenal atresia: a case report. Cases J 2008; 1:215
  4. Benger JR, Thompson MH. Annular pancreas and obstructive jaundice. Am J Gastroenterol 1997; 92:713-4
  5. Sharma AK, Mishra PK, Chibber S. Annular pancreas: MR and MR Pancreaticography a useful tool. Indian J Radiol Imaging 2006;16:433-7