Rare Case of Dorsal Pancreatic Agenesis presenting as Pain Abdomen

Dr Divendu Bhushan at Department of Gastroenterology, Paras HMRI, Patna, Bihar and colleagues have reported Rare Case of Dorsal Pancreatic Agenesis presenting as Pain Abdomen. The case has appeared in the Journal of Association of Physicians of India.

Agenesis of the dorsal pancreas is an extremely rare congenital pancreatic anomaly.

While complete agenesis of the dorsal pancreas is extremely rare, partial agenesis of the dorsal pancreas is thought to be more common than ventral pancreatic agenesis. While many patients with this anomaly are asymptomatic, some patients may present with abdominal pain.

It results from an embryological failure of the dorsal pancreatic bud to form the body and tail of the pancreas.

Partial dorsal pancreatic agenesis may be appreciated as a short, rounded pancreatic head adjacent to the duodenum with the absence of the pancreatic neck, body, and tail. With complete agenesis of the dorsal pancreas, the neck, body, and tail of the pancreas, the duct of Santorini, and the minor duodenal papilla are all absent. With partial agenesis of the dorsal pancreas, the size of the body of the pancreas varies, there is a remnant of the duct of Santorini, and the minor duodenal papilla is present.

A 24 yr old male with no addiction presented to Medicine OPD Paras HMRI, Patna with pain abdomen around the umbilicus, burning type radiating to back. There was no tenderness in the abdomen. Investigations including blood glucose, LFT and S. Amylase and lipase, Ultrasound abdomen UGI endoscopy were normal. CT abdomen which reveals agenesis of pancreatic body and tail, Pancreatic duct was not dilated (Figure 1).

 

It is critical to rule out pancreatic carcinoma with proximal atrophy, pancreas divisum, and pancreatic masses as these may resemble agenesis of the dorsal pancreas in imaging.It may be associated with

• hyperglycemia: may be present in a significant proportion of cases (up to 50% )


• solid pseudopapillary tumor of the pancreas: possible association ²


• polysplenia


• pancreatitis

Treatment is conservative including low-fat diet and enzyme supplements.