Pancreas divisum pancreatitis: a case report

Abdominal Imaging

ª Springer Science+Business Media, LLC 2010 Published online: 14 May 2010

Abdom Imaging (2011) 36:215–217 DOI: 10.1007/s00261-010-9619-3

Pancreas divisum pancreatitis: a case report Elena Gologan,1 Dorin Achitei,2 Roxana Bocan,2 Gheorghe Balan1 1 2

University of Medicine and Pharmacy, Gastroenterology, Iasi, Romania Institute of Gastroenterology and Hepatology, Iasi, Romania

Pancreas divisum is considered to be the most frequent congenital pancreatic abnormality but, depending upon the diagnostic method employed, wide ranges of incidence are reported [1]. At necropsy, the incidence is less than 14% but when using endoscopic retrograde cholangiopancreatography (ERCP), the incidence reaches 25%, obviously due to the selection bias linked to studying a symptomatic population [2]. Imaging is essential for the diagnosis which is then confirmed by cholangiopancreatography, either using magnetic resonance or ERCP. This approach is preferrable due to its non-invasive character [3]. Contrast computed tomography and ultrasound examinations are not useful, except for the acute pancreatitis resulting from pancreas divisum; they reveal better the secondary parenchymal changes rather than allow the identification of the two separate, non-communicating ducts. The secretin test is not recommended because of its acute pancreatitis risk [3]. We present here an unusual case of pancreatitis in a patient with pancreas divisum in whom only one of the pancreatic areas was affected. In such situations imaging should consider all the pancreatic areas because even if one may appear normal, this does not exclude the existence of pancreatitis lesions in the other.

Case report A 19-year-old student presented to the Institute of Gastroenterology and Hepatology from Iasi, Romania with left hypochondral pain, irradiating to the upper abdomen, predominantly but not exclusively after meals. The patient had no significant family history. Notable in the patient’s history is an episode two weeks earlier of

Electronic supplementary material: The online version of this article (doi:10.1007/s00261-010-9619-3) contains supplementary material, which is available to authorized users. Correspondence to: Elena Gologan; email: elenagologan2007@yahoo. com

acute pancreatitis and bulbar duodenal ulcer, recently diagnosed in a district hospital where she was treated, No clear alimentary trigger could be identified at that time. The patient was a non-smoker and did not drink alcohol. She was not under any medication at the time the pancreatitis symptoms appeared. Physical examination revealed mild left hypochondral and mesogastric pain at deep abdominal palpation. Laboratory tests revealed normal hematological values; biochemistry showed blood amylase 131 IU/L, urine amylase 811 IU/L, and blood lipase 237 IU/L. Calcium levels and inflammation biomarkers were normal (possibly because the patient was admitted two weeks after the beginning of the pain, that was initially much more severe). Given the suspected clinical diagnosis of acute pancreatitis, an ultrasound examination was performed which revealed an overall enlarged pancreas. The pancreas head (Fig. 1) had a normal structure (uniform echogenicity slightly higher than the liver). The pancreas body and tail displayed a cephalic extension in the posterior plane and had a distinctly hypoechoic structure, rounded edges and was overall enlarged out of proportion in relation to the pancreas head (Fig. 1B). There were no dilatations of the pancreatic ducts and there were no necrotic areas or pancreatic and peripancreatic fluid collections. The biliary ducts, liver and spleen were normal. There was no fluid in the peritoneal cavity. Subsequently, the patient was explored endoscopically in order to identify a gastric ulcer lesion, as described during a recent endoscopy. However, there were only inflammatory aspects of the gastro-duodenal mucosa, but no ulcer could be identified. Given the suspicion of acute pancreatitis due to pancreas divisum suggested by the ultrasound examination and the exclusion of other more frequent causes of acute pancreatitis (alcohol consumption, biliary causes) as well as rare causes (dyslipidemias, hyperparathyroidism, trauma, drugs, allergy, autoimmunity, infections), a magnetic resonance cholangiopancreatograpy (MRCP) was carried out in order to identify ductal and parenchymatous

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Fig. 1. A Abdominal ultrasound—normal aspect of the head of the pancreas. B There is a cephalic extension of the parenchymal edema area.

anomalies: one normal and another one with edema (Fig. 2). The MRCP confirmed the presence of two pancreatic ducts with separate openings in the duodenum Fig. 3). For an additional confirmation, the patient was explored with the lateral-view endoscope in order to identify the two distinct papilary openings. Lateral-view duodenoscopy was carried out and the two papilary openings, of similar size were identified. One of them was normally located and the other one was placed above the first at 2 cm distance. These were not cannulated in order to reconfirm the anomaly by endoscopic retrograde pancreatography because of the recent pancreatitis episode and also because duodenoscopy merely confirmed the initial supposition.

Discussion Pancreas divisum develops in the 7th–8th week of intrauterine life. In addition to the complete separation variant, there are other variants in which a minor link is

E. Gologan et al.: Pancreas divisum pancreatitis

Fig. 2. A MRI: edematous pancreatitis in the body and tail of the pancreas; one pancreatic duct can be identified. B MRI: aspect the head of the pancreas which is completely different from the aspect of the pancreas body, showing a clear demarcation limit.

established between the two pancreas channels, so that usually the pancreatic secretion is drained through the minor papilla, although the opposite is also possible. This results from the lack of regression of the Santorini duct and its lack of fusion with the Wirsung duct. Pancreas divisum may not lead to any clinical symptoms, so it is then diagnosed during an unrelated investigation. Alternately, patients can present with acute pancreatitis syndromes, varying in severity, starting with mild forms corresponding to the edematous pathological aspect and reaching severe forms that correspond to the necrotico-hemorrhagic type. Pancreatitis usually results as a consequence of the disproportion between the size of the papillary opening and the secretory debit of the corresponding pancreatic region. Obviously, the clinical picture is dominated by abdominal pain, but in severe forms shock, encephalopathy, intestinal occlusion and paresis, ascites, jaundice or sepsis may occur. These can be triggered by overeating or

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normal place (the major one) and the other usually proximal compared to the first (the minor papilla). The two papillae have comparable sizes due to the drainage needs that the minor papilla has to fulfill [4]. Papillary manometry and echoendoscopy do not produce relevant information, although they are quoted as being of possible use in the literature. For the patients with a clear diagnosis, acute pancreatitis prophylaxis by avoiding foods that trigger intense pancreatic secretions and especially alcohol are indicated. In acute pancreatitis, the treatment is the standard one for the corresponding type, dependent upon the clinical symptoms, evolution, and complications. For chronic pancreatitis, sphincterotomy of the minor papilla, eventually followed by duodenal drainage prosthesis may be carried out [5]. Rarely, in selected cases only, is surgical treatment indicated: surgical sphincterotomy, draining or even partial pancreatectomies: their results are comparable with those achieved by endoscopic procedures.

Conclusion

Fig. 3. MRCP: A Separate pancreatic duct for the anterior part of the head of the pancreas. B Separate pancreatic duct for a part of the head of the pancreas.

alcohol consumption. Alternately, patients may present recurrent episodes of acute pancreatitis that may progressively lead to chronic pancreatitis. Laboratory investigations show abnormal results only during acute pancreatitis episodes—usually an increase in serum and urine levels of the pancreatic enzymes (amylase, lipase), electrolyte and acid–base imbalances, an inflammatory syndrome, hepatocytolysis, hypocalcemia. Lateral-view duodenoscopy is a useful investigation that allows the identification of two papillae, one in the

We investigated in depth an unusual case of pancreatitis in a patient with pancreas divisum in whom only one of the pancreatic areas was affected. Since pancreas divisum is one of the most frequent anatomical abnormalities of the pancreas, it should be kept in mind in the case of patients in whom clinical symptoms suggest pancreatitis and some pancreatic areas appear normal while other areas may not be well visualized. In this respect, thorough abdominal imaging is essential for the diagnosis.

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