CASE REPORT

Extra-hepatic bile duct hamartoma in a 10-month-oldwith a morgagni hernia and multiple anatomical anomalies:a rare and incidental finding

Adil A. Shah • Michael Karass • Andrew J. Page •

Bahig M. Shehata • Megan M. Durham

Accepted: 16 January 2013

� Springer-Verlag Berlin Heidelberg 2013

Abstract Von Meyenburg complexes (VMCs), also

known as bile duct hamartomas, are a part of a group of

ductal plate malformations. They are typically present in-

trahepatically. In this case, we present to our knowledge

the first report of an extra-hepatic VMC in the pediatric

population. The patient presented as a 10-month-old infant

with a weeklong history of progressive breathing difficulty.

A chest radiograph was obtained, showing intestinal loops

in the thoracic cavity consistent with a Morgagni’s hernia,

unrelated to his breathing difficulty. The patient then

underwent an elective repair of his congenital diaphrag-

matic defect. During the operation, the bile duct hamar-

toma was found adherent to the accessory lobe of the liver,

present to the left of the ligamentum teres.

Introduction

Bile duct hamartomas, also known as VMCs, are uncom-

mon, often noted as incidental findings during abdominal

procedures [1]. They are part of a group of defects and

conditions caused by ductal plate malformations including

choledochal cyst, adult autosomal dominant polycystic

liver disease, Caroli’s disease, and Caroli’s syndrome [1].

They are infrequent anomalies in adults (5.6 %) and even

rarer in children (0.9 %) [2]. However, the incidence from

radiological studies is different from the above reported

figures [1, 5, 8]. Believed to sporadically occur as a con-

sequence of ductal plate dysgenesis, these lesions are

considered anatomical anomalies rather than neoplastic

transformations. Histopathologically, they consist of dis-

ordered aggregates of dilated bile ductules embedded in a

fibrocollagenous stroma [3]. Radiological diagnosis may be

difficult for small nodules. Multiple lesions are often

mistaken for metastasis [4, 5].

We report a case of a 10-month-old boy, initially diag-

nosed with a Morgagni hernia at the age of 5 months.

Intraoperatively, in addition to the diaphragmatic hernia, he

had an intra-abdominal mass, that was histopathologically

an extra-hepatic bile duct hamartoma.

Case presentation

A 5-month-old male infant presented to our institution with

a weeklong history of progressive difficulty in breathing

and nasal congestion, for which he had received nebulized

inhalation therapy at an outside facility. He was brought to

our ED when symptoms failed to improve. His oral intake

had decreased significantly with increased work of

breathing. Clinically, the child appeared dehydrated, tach-

ycardic and tachypneic with noticeable wheezing, grunting

and chest retractions. Pulse oximetry, however, revealed

his saturations to be in the 90 % range. Suctioning of the

airway and intermittent nebulizations with albuterol and

ipratropium bromide failed to produce significant

improvement.

A chest radiograph (Fig. 1) showed intestinal loops in

the thoracic cavity consistent with a Morgagni’s hernia.

A. A. Shah

Medical College, Aga Khan University, Karachi, Pakistan

M. Karass � B. M. Shehata (&)

Department of Pediatric Pathology, Children’s Health Care

of Atlanta, Emory University School of Medicine, Atlanta,

Georgia, USA

e-mail: bshehat@emory.edu

A. J. Page � M. M. Durham

Department of Pediatric Surgery, Children’s Health Care

of Atlanta and Emory University, Atlanta, Georgia, USA

123

Pediatr Surg Int

DOI 10.1007/s00383-013-3271-2

Surgery was consulted and it was determined that his

respiratory condition was unrelated to the congenital dia-

phragmatic hernia. The patient was admitted to the pedi-

atrics service for management of his respiratory symptoms.

He was found to be respiratory syncytial virus (RSV)

positive which was expectantly managed with supple-

mental oxygen and nebulizers with the diagnosis of acute

bronchiolitis.

Elective repair of the congenital diaphragmatic defect

(CDH) was scheduled once his respiratory symptoms

resolved. He had no significant past medical or surgical

history. He was born at term with no complications and

was developmentally appropriate. The child’s CDH had

been completely asymptomatic and had no history of dif-

ficulties in feeding or stooling.

The pediatric surgery team laparoscopically reduced the

contents of the hernia. A large defect in the ligamentum

teres was visualized through which part of his bowel was

traversing from the left side of the abdominal cavity into

the chest. The contents of the hernia sac consisted of the

right and transverse colon and an accessory hepatic lobe

(Fig. 2). Further exploration of the defect revealed an

absent anterior pericardium. However, the Morgagni sac

was in continuum with the posterior aspect of the peri-

cardium. The large size of the defect warranted conversion

to an open procedure and the defect was repaired primarily

with pledgeted monofilament permanent sutures in a

‘mattressed’ fashion where feasible. In the remaining

defect, a 2-mm thick piece of Gore-TexTM Soft Tissue

Patch (W. L. Gore & Associates, Inc. Medical Products

Division, Flagstaff, Arizona, USA) with pledgets on either

side of the muscular diaphragm was used.

The patient was noted to have malrotation of the intes-

tines. Dense fibrous Ladd’s bands were extended from the

cecum and proximal ascending colon to the right retro-

peritoneum. A Ladd’s procedure was performed along with

an inversion appendectomy. The entirety of the hernia sac

was not resected, as it was contiguous with the posterior

pericardial tissue. A hamartomatous lesion adherent to the

accessory lobe of the liver was found to be located to the

left of the ligamentum teres (Fig. 3). The lesion was

biopsied and sent for frozen section. The lesion was care-

fully excised and on final pathologic evaluation was

revealed to be a bile duct hamartoma (liver tissue with

cirrhosis; proliferating bile duct, consistent with bile duct

hamartoma 4.0 9 2.8 9 1.5 cm).

The child tolerated the procedure well and his sub-

sequent hospital course was uneventful.

Fig. 1 Chest radiograph upon initial presentation

Right Hemidiaphragmc

Colon traversing defect

Ligamentum Teres

Morgagni Hernia Defect

Fig. 2 Laparoscopic view showing central diaphragmatic defect

with view from abdomen into thoracic space

Fig. 3 Extra-hepatic bile duct hamartoma arising from ligamentum

teres

Pediatr Surg Int

123

Discussion

First described in 1918 by von Meyenberg, VMCs can only

be diagnosed histopathologically. Biliary hamartomas are

usually benign malformations of the hepato-biliary system,

are largely asymptomatic, and usually found during lapa-

rotomies or in 0.5 to 5.6 % of autopsies [2, 5]. To the best

of our knowledge, this is the first report of an extra-hepatic

VMC in the pediatric literature.

The most common variant of hepatobiliary fibrocystic

disease occurring as a result of ductal plate malformation is

the von Meyenberg complex. Persistence of the double-

layered ductal plate, that is a normal constituent of fetal

livers, post-natally is called ductal plate malformation. It is

postulated that interruption of hepatic ductal plate recon-

stitution and remodeling during the late phase of embryo-

logical biliary differentiation may lead to its occurrence [2,

6–8].

VMCs are diagnosed using imaging as well as through

biopsies. Typical imaging findings include multiple comet-

tail echoes on US, small hypodense lesions on CT dis-

persed throughout the liver, and a cystic appearance on

MRI [5, 8]. On ultrasound, hypoechoic and hyperechoic

foci are seen distributed uniformly among the liver, while

on the MRI, they appear as hypointense lesions on T1 and

hyperintense lesions on T2. Needle biopsies are also used

to diagnose VMCs and are especially useful due to the

possibility of mixing biliary hamartomas with liver

metastases, microabscesses, lymphoma, and simple liver

cysts. Biliary hamartomas are usually uniform in size and

distribution, while liver metastases are larger and have a

variable size and distribution [5, 8].

Bile duct hamartomas are gray to white lesions that are

normally small, measuring anywhere from 1 mm to 1.5 cm

and are multifocal and irregular in appearance. On micro-

scopic evaluation they consist of multiple, small to mod-

erate sized ductules. These ductules are cystically dilated

and are typically separated by dense collagenous bands of

stroma. Bile duct hamartomas are commonly situated

peripherally around the portal tracts and consist of irregu-

larly shaped cystic spaces containing eosinophilic frag-

ments and inspissated bile that help differentiate these

lesions from normal ducts. The cells lining the ductules are

flattened, cuboidal cells that are distinguished by their

well-circumscribed, round to oval nuclei [8–10]. They are

typically composed of multiple lesions that are less than

1.5 cm each and are generally dispersed throughout the

liver, especially in the subcapsular region [8]. However,

solitary lesions have been discovered [5].

It is pertinent to note here that the VMC in our patient

had an extra-hepatic location. The lesion was found on top

of the accessory lobe of the liver, located to the left of the

ligamentum teres. This may have been brought about as a

result of ectopic hepatic tissue adherent to the ligamentum

teres.

Literature on the nature and natural history of this

seemingly benign lesion remains scarce. Some cases were

associated with polycystic liver and kidney disease, pan-

creatic cysts, and simple liver cysts [8]. Ductal plate mal-

formations (DPM), which include VMCs, are associated

with an increased incidence of hepatic malignancy

including cholangiocarcinoma, hepatocellular carcinoma,

adenosquamous carcinoma, squamous cell carcinoma, and

papilloma [6]. There is also a positive correlation between

bile stasis and the development of malignancy in DPMs

[6]. Thus far, only a few cases have been reported in the

literature of neoplastic transformation of VMCs [6]. Fur-

thermore, VMCs that progress to a neoplasm have a low

metastatic potential [6].

Some studies suggest that patients with VMCs have a

greater chance of developing hepatic carcinoma. However,

the rate of occurrence of hepatic carcinoma is thought to be

proportional to the size of the lesion. Bile duct hamartomas

or VMCs can also rarely undergo malignant transformation

to cholangiocarcinoma [6, 11, 12]. However, some of the

literature suggests that the association of cholangiocarci-

noma with VMCs is likely an under recognized phenom-

enon due to the obtainment of small sample sizes from

patients and misdiagnosis of cholangiocarcinoma as HCC

[6].

The nature of his disease and the consequent interven-

tion brought this interesting lesion to medical attention,

which may have gone unnoticed otherwise.

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