A 16-year-old woman (without a history of interest) was referred to our hospital for generalized pruritus, jaundice, choluria and colic stools.
Physical examination revealed good general condition, jaundice cutaneomucosa, and hepatomegaly of bladders.
The rest of the anamnesis by equipment was normal.
Biochemistry: direct bilirubin 22.13 mg/dl, AST 66 IU/l, ALT 91 IU/l, alkaline phosphatase 1838 IU/l, GGT 37 IU/l.
CA 19-9 86 IU/l, rest of biochemical parameters and coagulation study were normal.
Abdominal ultrasound showed dilation of the intra-extrahepatic bile duct without the presence of lithiasis.
CT and Cholangio MRI showed hepatomegaly, dilation of the intra-extrahepatic bile duct, dilation of the Wirsung duct, destructuring and increased size of the uncinate process and head of pancreas, suggestive.
It was decided to place a percutaneous external biliary drainage internal to solve obstructive jaundice.
Under suspicion of pancreatic neoplasia, the patient was operated on; the intraoperative examination showed induration of the head of the pancreas and hiliar adenopathies; of both, intraoperative and negative interstitial biopsies were taken for neoplasia, respectively.
Pancreatoduodenectomy was performed with cephalea preservation.
The pathological study reported chronic obstructive fibrosing pancreatitis and inflammatory ductal hyperplasia of the pancreatic duct.
The patient was discharged on the twelfth postoperative day without complications.
A genetic study of the N34S mutation in the SPINK1 gene was performed in the 6th postoperative month, which was negative.
Two years later she is asymptomatic and disease free.
1.
PIP is a rare cause of chronic pancreatitis in children.
The age range is 4 months to 20 years with a mean age of 10 years (1).
To date, only 44 cases have been published in the literature.
The etiology is unknown, although it has been associated with certain genetic alterations (CFTR, PRSS1), highlighting by its frequency the N34S mutation of the SPINK1 gene, which has also been related to tropical juvenile pancreatitis (1,3,4).
Unlike autosomal dominant hereditary pancreatitis, patients with PIP have no family history.
The most common form of presentation of PIP is obstructive jaundice and recurrent abdominal pain crises (1,2,5,6,).
Analytically, patients with PIP have a cholestatic, cytolytical pattern and occasionally a slight elevation of serum and urinary amylase.
With respect to diagnosis, imaging tests are useful to objectify the pancreatic origin of bile duct obstruction.
CT and CholangioMRI allow, as in our case, a clearer visualization of the pancreas, whose head will usually be enlarged and destructured.
Wirsung may present prestenotic dilatation.
The definitive diagnosis is achieved by excluding other more frequent pathologies and by pathological study (biopsy or surgery).
Histologically, the pancreas shows bands of collagenized tissue surrounded by a typical appearance.
The inflammatory infiltrate accompanying fibrosing proliferation is not usually prominent (1,6,).
Treatment should begin, if any, with resolution of obstructive jaundice pending the surgical procedure.
Percutaneous biliary drainage or stent placement by ERCP are the techniques of choice (1.6,).
Surgical treatment is controversial and can be classified into derivative and resective.
Abdominal pain persists in disuse due to failure to resolve the obstruction of the Wirsungs anomaly (1). Endoduodenostomy and placement of a condom.
Among the resective procedures stands out the DPC with pyloric preservation.
Some authors advise against the high frequency of diabetes and poor postoperative digestion, as well as poor long-term pain relief.
It was the technique used in our case and 24 months later, the patient remains asymptomatic and free of disease, so we cannot support this statement.
Pancreatectomy with ceftazidime preservation of the duodenum (DPPHR) eliminates complications of chronic pancreatitis and provides long-term pain relief in more than 80% of patients (1).
G. Suárez Go arthritis, J. Rodríguez Muñoz, A. Gómez Bravo, F. Docobo Durántez, R. García Lozanochel S. Pga Alcon
General Surgery and Digestive Appliance Service.
Hospital Universitario Virgen del Rocío.
Hold it.
