True congenital multicystic disease of the pancreas in the infant: a very rare case

Abstract

The most frequent pancreatic cysts in patients of developmental age are pseudocysts, neoplastic cysts, posttraumatic cysts, and parasitic cysts. Retention cysts, duplication cysts, and congenital cysts are called true cysts. Among the true cysts, congenital cysts are very rare and include 1% of all pancreatic cysts. The finding of true congenital pancreatic cysts in children under 3 years represents a very rare event; less than 30 have been described in the literature. We describe the case of a 3-year-old male who came to our observation for abdominal pain and underwent an abdomen ultrasound examination. The ultrasound examination showed unilocular sub-centimeter cystic lesions, distributed throughout the pancreatic parenchyma. The patient was, therefore, not subjected to any treatment, and after a few days, the symptoms regressed spontaneously. This description probably represents the first case in the literature of “congenital multicystic disease” of the pancreas.

Electronic supplementary material

The online version of this article (10.1007/s40477-020-00472-9) contains supplementary material, which is available to authorized users.

Introduction

Cystic lesions of the pancreas in children are not uncommon and can be highlighted both during the prenatal period, in routine screening [1], and during the postnatal period. The most frequent pancreatic cysts in patients of developmental age are pseudocysts, neoplastic cysts, posttraumatic cysts, and parasitic cysts; retention cysts, duplication cysts, and congenital cysts are less frequent. The last three are called true cysts, because they have a true epithelial lining, unlike pseudocysts and posttraumatic cysts. Among the true cysts, congenital cysts [2–4] include 1% of all pancreatic cysts [5]. The origin of the pathology is unknown, but it is thought to be due to an embryological defect and, in particular, to the seizure and isolation of the primitive pancreatic ducts that fill with liquid.

The ultrasound finding of true congenital pancreatic cysts represents a very rare event; few cases have been described at present (< 30), almost all unilocular and mainly in children under the age of 2 years [6].

In the few multilocular cases, the cysts were predominantly located in the caudal portion of the pancreas. The first instance exam is generally an ultrasound examination; only in cases associated with laboratory alterations or with important clinical alterations or before surgery, MR or CT examinations are used.

The therapy of pancreatic cysts during developmental age is generally conservative and is linked to the benign nature of the pathology, even if, in selected cases, a surgical approach aimed at total exeresis of the cysts and the conservation of the pancreatic parenchyma or at bilious-digestive derivation [7] is preferred. We describe a very rare case of congenital multicystic disease of the pancreas, not associated with laboratory alterations or other pathologies.

Case report

A 3-year-old male, born from spontaneous childbirth, came to our observation for abdominal pain widespread in all abdominal quadrants, not linked to meals, arising from 5 days. Upon physical examination, the child was in good general condition, had no growth retardation or fever, and was channeled to feces and gas. The abdomen was palpable, and the laboratory tests and the glucose intolerance test were negative. An abdomen ultrasound examination was performed using an Aplio XG (Toshiba) ultrasound device with a 3.5 MHz convex and a 7.5 MHz linear probe. The ultrasonographic scans were performed in supine, lateral right, and left decubitus. Longitudinal and transverse scans were performed. The examination was performed by an operator with 10 years of experience in pediatric ultrasound. All abdominal organs, bowels, and wormlike appendages have been studied.

Sub-xiphoid scans performed in supine decubitus showed the following: multiple roundish, anechoic, sub-centimetric lesions, diffused in all portions of the pancreas, that did not show continuity with the intra-pancreatic bile ducts (Fig. 1a–c); the absence of biliary tract dilation; a pancreas with regular morphology and dimensions; and the absence of peri-pancreatic and/or abdominal effusion. No changes in the liver, spleen, kidneys, gallbladder, intestines (moderate hyper-peristalsis), and/or appendix were observed.

Fig. 1.

Ultrasound examination of the pancreas: sub-xiphoid scan with patient in supine decubitus. a Cystic lesions (short arrows) involve the cephalic portion of the pancreas (heads of arrows). Spleno-mesenteric confluence (long arrow). b Cystic lesions (short arrows) involve the body of the pancreas. Abdominal aorta (long arrow). c Cystic lesions (short arrows) involve the caudal portion of the pancreas. Spleno-mesenteric confluence (long arrow). Splenic vein (heads of arrows)

The patient was subsequently subjected to an abdomen CT examination that excluded neoplastic pathologies of the bone structures and confirmed the presence of pancreatic cysts (Fig. 2a, b). The patient was then kept under observation for a few days until the spontaneous regression of the painful symptoms; after discharge, he underwent periodic checks with an ultrasound examination and laboratory tests, which showed no alterations up to the present state.

Fig. 2.

CT examination of the abdomen with contrast medium. a In the reconstruction on an axial plane, the cystic lesions in the pancreas (short arrows) are highlighted. Spleno-mesenteric confluence (long arrow). S spleen, L liver. b In the reconstruction on a coronal plane, cysts (short arrows) are recognized in the cephalic portion of the pancreas and in the body. Portal vein (heads of arrows). L liver, S spleen

Discussion

The diagnosis of congenital cysts of the pancreas during developmental age is mainly ultrasonographic; prenatal diagnosis is very rare and accounts for about 30% of the total diagnoses [8]. When the cysts reach a considerable dimension, an MRI or CT is used to establish their nature and any communication with the pancreatic ducts. Differential diagnosis essentially involves pancreatic pseudocysts [9], pancreatic duplication cysts [10, 11], and retention cysts [12], which are always symptomatic and cause alterations in pancreatic function indices (amylase and lipase) and/or glucose intolerance. In the cases reported in the literature, the congenital cysts, which were predominantly unilocular, had large dimensions (> 2 cm) and were all associated with different symptoms: vomiting and abdominal pain, palpable abdominal mass, abdominal over-distension, compression of the contiguous structures, etc.

The association between polycystic kidney disease and von Hippel–Lindau syndrome has been described in the literature [13] but has been excluded in our case. Surgically treated patients had severe symptoms, and total cyst exeresis was not always practicable; it was limited by the size and especially by the distribution of the cysts in the pancreatic parenchyma. In our case, the cystic morphological characteristics, the absence of communication with the pancreatic ducts, the absence of dilation of the bile ducts, and the absence of laboratory alterations have allowed us to exclude the retention cysts, the duplication cysts, and the pseudocysts.

Currently, many authors consider true congenital cysts to be benign [14] and prefer a conservative approach. For this reason, we decided to keep the child under observation until spontaneous regression of the symptoms, which were probably of an intestinal nature: enteritis, meteorism, indigestion, etc.

In conclusion, true congenital cysts in children, although rare, in our opinion, should be considered in the differential diagnosis, especially if patients are asymptomatic and/or do not show laboratory changes. Thanks to new technological developments [15] and new, more efficient linear probes [16], ultrasound seems to be the ideal imaging method for studying the pancreas in patients of developmental age.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

The consent was obtained from the patient for the publication of this case report and accompanying images.