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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 1  |  Page : 57-59

An unusual case of steatorrhea


1 Department of Gastroenterology, Pondicherry Institute of Medical Sciences, Ganapathichettikulam, Puducherry, India
2 Department of Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India
3 Department of Gastroenterology, Christian Medical College, Vellore, Tamil Nadu, India

Date of Submission12-Mar-2020
Date of Decision16-Apr-2020
Date of Acceptance19-Apr-2020
Date of Web Publication20-Jul-2020

Correspondence Address:
C Bharath Kumar
Department of Gastroenterology, Pondicherry Institute of Medical Sciences, Ganapathichettikulam, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrsm.jcrsm_4_20

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  Abstract 


Partial agenesis of the pancreas is a rare developmental anomaly of the pancreas which can lead to exocrine and endocrine insufficiency. We report a 61-year-old male patient with steatorrhea who was found to have complete agenesis of the dorsal pancreas. He presented with a history of loose stools for 5 years, and the stools were at times oily in nature without mucus or blood. His clinical examination was unremarkable. Seventy-two hour stool fat was increased (21.4 g, normal<18 g) and stool elastase was low (120 μg, normal >200), and computed tomography of the abdomen revealed a normal head and uncinate process of the pancreas, abscence of body and tail of pancreas associated with a nondilated duct system. He was put on pancreatic enzyme supplements and he responded to the treatment, and on follow-up after 6 months, his stool frequency had declined and his fecal elastase had improved.

Keywords: Agenesis of pancreas, partial agenesis of pancreas, steatorrhea


How to cite this article:
Kumar C B, Simon B, Simon EG. An unusual case of steatorrhea. J Curr Res Sci Med 2020;6:57-9

How to cite this URL:
Kumar C B, Simon B, Simon EG. An unusual case of steatorrhea. J Curr Res Sci Med [serial online] 2020 [cited 2020 Oct 27];6:57-9. Available from: https://www.jcrsmed.org/text.asp?2020/6/1/57/290250




  Introduction Top


Partial agenesis of the pancreas is a rare developmental anomaly of the pancreas which can lead to exocrine and endocrine insufficiency. We report a 61-year-old male patient with steatorrhea who was found to have complete agenesis of the dorsal pancreas.


  Case Report Top


A 61-year-old gentleman presented with a history of loose stools for 5 years, passing 3–4 semisolid stools/day. The stools were at times oily in nature without mucus or blood. He also had associated dyspeptic symptoms, but there were no episodes of frank abdominal pain. There was no significant loss of weight or anorexia. He was a known case of diabetes mellitus for 15 years and also had chronic pulmonary obstructive disease for the past 5 years. He was a reformed smoker and a social drinker. There was no prior history of abdominal trauma or surgery. His clinical examination was unremarkable. There was no evidence of water or fat-soluble vitamin deficiency. On evaluation, liver function tests, creatinine, and lipid profile were normal. His blood sugars were elevated, 72 h stool fat was increased (21.4 g, normal<18 g), and stool elastase was low (120 μg, normal >200). Ultrasound (US) of the abdomen was reported as being normal. Computed tomography (CT) of the abdomen was done in view of elevated blood sugars and steatorrhea, and it revealed a normal head and uncinate process of the pancreas, abscence of body and tail of pancreas associated with a nondilated duct system [Figure 1] and [Figure 2]. He was put on pancreatic enzyme supplements, and the insulin doses were increased. He was advised a low-fat diet and to abstain from alcohol consumption. On his review to us after 6 months, his stool frequency had declined and his fecal elastase had improved, while his diabetes was under control.
Figure 1: Axial computed tomography section of the abdomen at the level of splenic hilum shows the splenic vein (white arrow) and the absence of the body and tail of the pancreas anterior to it

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Figure 2: Axial computed tomography section of the abdomen at the level of the superior mesenteric vein (white arrow) shows a normal head and uncinate process of the pancreas (black arrow) and absence of the body of the pancreas anterior to the superior mesenteric vein

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  Discussion Top


The pancreas originates from the endoderm between the 6th and 8th week of embryonic life. It develops from two evaginations, dorsal and ventral, of the primitive duodenum. These two evaginations fuse together to form a single organ and the fusion is more or less complete. The upper part of the head, body, and tail of the pancreas arises from the dorsal pancreatic bud and they drain through the duct of Santorini. The major part of the head and uncinate process of pancreas arises from the ventral pancreatic bud and they drain through the duct of Wirsung. The fusion between ventral and dorsal pancreas occurs at 6–7 weeks of gestation. Congenital abnormalities can occur during the development of the pancreas.[1]

Agenesis of the dorsal pancreas was first described in 1911.[2] It is a rare congenital malformation, in which there is only one pancreatic duct system with no duct of Santorini or body and tail of the pancreas.[3],[4],[5],[6],[7] Partial agenesis of the pancreas or pancreatic hypoplasia occurs in approximately 1–2/10,000 patients and is often associated with other congenital malformations such as polysplenia syndrome, wandering spleen, interruption of the inferior vena cava, hemiazygos and azygos continuation, symmetrical liver, anomalous hepatic fissure or lobe, left-sided inferior vena cava, median gallbladder, inverted gallbladder and stomach, and intestinal malrotation.[8]

There are case reports showing the familial occurrence of agenesis of the dorsal pancreas in the mother and her sons; the mode of transmission is most likely autosomal dominant or X linked dominant.[9],[10]

Agenesis of the dorsal pancreas may be associated with diabetes mellitus and abdominal pain. The absence of the body and tail contributes to the development of diabetes mellitus.[11] Abdominal pain can develop due to sphincter of Oddi dysfunction and hypertrophy of the remnant ventral gland with higher intrapancreatic duct pressure.[12] Steatorrhea is a rare presentation of agenesis of the dorsal pancreas and it is due to exocrine pancreatic insufficiency; usually, it is associated with diabetes mellitus and more common in children.[13],[14] Only one case of exocrine pancreatic insufficiency has been reported in the absence of diabetes mellitus.[15]

Agenesis of the dorsal pancreas can be diagnosed on abdominal imaging such as US, CT, or magnetic resonance imaging when the body and tail of the pancreas are not visualized ventral to the splenic vein.[10],[16],[17],[18],[19],[20],[21] US of the abdomen may not be very helpful when the bowel gas obscures the body and tail of the pancreas, and the diagnosis can be missed when the examination is done in a cursory manner. Endoscopic or magnetic retrograde cholangiopancreaticography can define the anatomy of the pancreatic ducts more precisely.[21] Rarely, pancreatic adenocarcinoma, solid-pseudopapillary tumor of the pancreas, and periampullary carcinoma have been detected in association with this condition.[22],[23],[24] Treatment is usually symptomatic and supportive.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Moore KL, Persuad TV. The Developing Human, Clinical Oriented Embryology. 8th ed. Philadelphia, PA: Saunders Elsevier; 2008. p. 221-2.  Back to cited text no. 1
    
2.
Schnedl WJ, Piswanger C, Soelkner C, Wallner SJ, Krause R, Lipp RW. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci 2009;54:481-7.  Back to cited text no. 2
    
3.
Uygor-Bayramicli O, Reddy DN, Kilicoglu G, Dolapcioglu C, Ostas D. Dorsal pancreatic agenesis. JOP 2007;8:450-2.  Back to cited text no. 3
    
4.
Rakesh K, Choung OW, Reddy DN. Agenesis of the dorsal pancreas (ADP) and pancreatitis – Is there an association? Indian J Gastroenterol 2006;25:35-6.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Pasaoglu L, Vural M, Hatipoglu HG, Terekhoglu G, Koparal S. Agenesis of the dorsal pancreas. World J Gastroenterol 2008;14:2915-6.  Back to cited text no. 5
    
6.
Joo YE, Kang HC, Kim HS, Choi SK, Rew JS, Chung MY, et al. Agenesis of the dorsal pancreas: A case report and review of the literature. Korean J Intern Med 2006;21:236-9.  Back to cited text no. 6
    
7.
Fadell II MF, Elsamaloty HM, Barak R. Agenesis of the dorsal pancreas. Applied Radiology 2010;39:62.  Back to cited text no. 7
    
8.
Rastogi R, Kumar R, Bhargava S, Rastogi V. Isolated pancreatic hypoplasia: A rare but significant radiological finding. Saudi J Gastroenterol 2009;15:289-90.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Wildling R, Schnedl WJ, Reisinger EC, Schreiber F, Lipp RW, Lederer A, et al. Agenesis of the dorsal pancreas in a woman with diabetes mellitus and in both of her sons. Gastroenterology 1993;104:1182-6.  Back to cited text no. 9
    
10.
Schnedl WJ, Reisinger EC, Schreiber F, Pieber TR, Lipp RW, Krejs GJ. Complete and partial agenesis of the dorsal pancreas within one family. Gastrointest Endosc 1995;42:485-7.  Back to cited text no. 10
    
11.
Wittingen J, Frey CF. Islet concentration in the head, body, tail and uncinate process of the pancreas. Ann Surg 1974;179:412-4.  Back to cited text no. 11
    
12.
Güçlü M, Serin E, Ulucan S, Kul K, Ozer B, Gümürdülü Y, et al. Agenesis of the dorsal pancreas in a patient with recurrent acute pancreatitis: Case report and review. Gastrointest Endosc 2004;60:472-5.  Back to cited text no. 12
    
13.
Haldorsen IS, Vesterhus M, Raeder H, Jensen DH, Søvik O, Molven A, et al. Lack of pancreatic body and tail in HNF1B mutation carriers. Diabet Med 2008;25:782-7.  Back to cited text no. 13
    
14.
Chen R, Hussain K, Al-Ali M, Dattani MT, Hindmarsh P, Jones PM, et al. Neonatal and late-onset diabetes mellitus caused by failure of pancreatic development: Report of 4 more cases and a review of the literature. Pediatrics 2008;121:e1541-7.  Back to cited text no. 14
    
15.
Doxey BW, Jackson WD, Adler DG. A unique presentation: Dorsal agenesis of the pancreas manifesting as pancreatic exocrine insufficiency in the absence of diabetes mellitus in an 8-year-old boy. Dig Dis Sci 2008;53:2005-6.  Back to cited text no. 15
    
16.
Deignan RW, Nizzero A, Malone DE. Case report: Agenesis of the dorsal pancreas: A cause of diagnostic error on abdominal sonography. Clin Radiol 1996;51:145-7.  Back to cited text no. 16
    
17.
Nonent M, Linard J, Lévèque E, Larroche P, Bobeuf J, Sénécail B. Dorsal pancreas agenesis: Computed tomography appearance with three-dimensional volume rendering reconstruction. Surg Radiol Anat 2003;25:161-3.  Back to cited text no. 17
    
18.
Macari M, Giovanniello G, Blair L, Krinsky G. Diagnosis of agenesis of the dorsal pancreas with MR pancreatography. AJR Am J Roentgenol 1998;170:144-6.  Back to cited text no. 18
    
19.
Shah KK, DeRidder PH, Schwab RE, Alexander TJ. CT diagnosis of dorsal pancreas agenesis. J Comput Assist Tomogr 1987;11:170-1.  Back to cited text no. 19
    
20.
Gold RP. Agenesis and pseudo-agenesis of the dorsal pancreas. Abdom Imaging 1993;18:141-4.  Back to cited text no. 20
    
21.
Itoh H, Saito M, Ishimori M, Ohshiro K, Guo YY, Sakai T. A case report of dorsal pancreas agenesis diagnosed by MRI and ERCP. Radiat Med 1991;9:108-9.  Back to cited text no. 21
    
22.
Ulusan S, Yakar T, Koc Z, Kayaselcuk F, Torer N. Adenocarcinoma of the pancreas associated with dorsal agenesis. Pancreas 2006;33:437-9.  Back to cited text no. 22
    
23.
Kapoor A, Singh R. Periampullary carcinoma in a patient with agenesis of dorsal pancreas. J Surg Case Rep 2011;2011:4.  Back to cited text no. 23
    
24.
Ulusan S, Bal N, Kizilkilic O, Bolat F, Yildirim S, Yildirim T, et al. Case report: Solid-pseudopapillary tumour of the pancreas associated with dorsal agenesis. Br J Radiol 2005;78:441-3.  Back to cited text no. 24
    


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  [Figure 1], [Figure 2]



 

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