CASE REPORT

A case of spontaneous chylous pericardial effusion in Polandsyndrome

Hong Chew • Pratap Shetty • Maqsood Elahi •

Zakir Akhunji

Received: 7 August 2012 / Accepted: 13 April 2013

� The Japanese Association for Thoracic Surgery 2013

Abstract Chylous pericardial effusion is an uncommon

entity that is most commonly associated with post-cardiac

surgery, in particular aortic valve and minimally invasive

cardiac surgery. Post-radiation therapy, infection, medias-

tinal neoplasm, lymphoma and a small group of idiopathic,

spontaneous chylous pericardial effusion have also been

reported as the causes. Here, we report a rare case of

pericardial effusion secondary to chylous fistula in a

63-year-old man with Poland syndrome. The case high-

lights an unusual thoracic duct anomaly as a cause of our

reported chylous pericardial effusion.

Keywords Pericardial effusion � Poland syndrome �Chylous formation � Pleural space

Introduction

Poland syndrome is a rare congenital anomaly, character-

ized by the under-development or absence of unilateral

chest wall muscle or breast [1]. These defects have been

postulated to be due to interruption of the embryonic blood

supply of the upper limb bud secondary to hypoplasia of

the ipsilateral subclavian artery or its branches [2]. Genetic

defects and autosomal dominant inheritance are causes of

congenital Poland syndrome; sporadic cases may be due to

trauma, viral infection, intrauterine insult from attempted

abortion or exposure to teratogenic xenobiotics [3] and

maternal smoking [4].

Previous case reports of chylous pericardial effusion, in

patients with known Poland sequence, are in patients who

have undergone breast surgery with/without axillary

clearance. The unlikely injury to the lymphatic trunk from

surgery has led to the conclusion that this complication is

caused by anatomical variation of the thoracic duct, which

is associated with Poland syndrome and other congenital

anomalies [1].

We present a rare case of spontaneous chylous pericar-

dial effusion in a 63-year-old man with known Poland

syndrome who required pericardiectomy for recurrent

pericardial effusion.

Case report

A 63-year-old man with known sporadic Poland syndrome,

absent left pectoralis major, presents with 2-week history of

intermittent right-sided pleuritic chest pain. This is on a

background of previous ischemic heart disease and percu-

taneous coronary intervention 6 years ago. There were no

exertional symptoms and no associated dyspnoea, palpita-

tions or presyncope. No evidence of previous cardiothoracic

or chest wall surgery from clinical history and examination.

The patient was initially investigated with chest radiog-

raphy that revealed mediastinal widening with subsequent

CT demonstrating a pericardial effusion (Fig. 1a, b).

Echocardiogram confirmed a moderate pericardial effusion

with otherwise normal bi-ventricular size and function.

Lymphoscintigram revealed potential duct anomaly

(Fig. 2a, b) with tracer drainage into the pericardial sac.

H. Chew � P. Shetty � M. Elahi � Z. Akhunji

Department of Cardiothoracic Surgery, Prince of Wales

Hospital, Barker Street, Randwick, NSW 2031, Australia

M. Elahi (&)

Division of Cardiothoracic Surgery, Department of Surgery,

Texas A and M Health Science Center at Scott and White

Memorial Hospital, 2401 S, 31st Street,

Temple, TX 76508, USA

e-mail: manzoor_elahi@hotmail.com

123

Gen Thorac Cardiovasc Surg

DOI 10.1007/s11748-013-0257-x

The patient was referred for surgical drainage of pericardial

effusion and this was performed via a subxiphoid incision

and division of pericardium to evacuate hemoserous effu-

sion. A 28 French right-angled drain was inserted into the

pericardial space, with pericardial biopsy and pericardial

window was re-apposed at the end of procedure.

Post-operatively, he was managed in the intensive care

unit. Pericardial drain output settled within 48 h and the

drain was removed after 72 h post procedure. On dis-

charge, repeat echocardiogram did not show any evidence

of recollection of pericardial fluid.

The patient represented after 2 months with recollection

of pericardial effusion requiring re-drainage of effusion.

Fluid biochemistry from previous collection confirms

chylous effusion. Pre-procedure echocardiogram reveals

normal left ventricular size and function with apical

hypertrophy, right atrial buckling and right ventricular

diastolic collapse.

Surgical drainage was again performed, with re-entry

through the subxiphoid incision and division of pericardium

with drainage of 600 ml of the fluid. The effusion was slightly

blood stained with the appearance of fat in the fluid. Next, an

incision was made in the diaphragmatic surface of the peri-

cardium to create a window into the peritoneal cavity. Again,

a pericardial drain was inserted. Post-procedure echocardio-

gram reveals residual moderate pericardial effusion with

otherwise no evidence of tamponade. He was discharged and

organized for follow-up with repeat lymphoscintigram. At

3 months routine follow-up, there was no further recollection

of pericardial effusion. Pericardial biopsy reveals normal

pericardial tissue without evidence of malignancy.

Discussion

Our case demonstrates spontaneous chylopericardium in

the setting of Poland’s syndrome due to anatomical

deformities of the lymphatic system. There is however no

direct evidence or direct correlation between the syndrome

and thoracic duct anomaly.

Although there is no definitive evidence linking

Poland’s syndrome to pericardial effusion, the literature

suggests thoracic duct anomaly in patients with Poland’s

syndrome leading to chylous pericardial effusion post

breast reconstructive surgery [1]. Potential injury to the

lymphatic system during surgery resulting in formation of a

chylous fistula [5] has been postulated as the most likely

pathogenetic hypothesis for chylous pericardial effusion in

this setting.

Other reported cases of primary chylous pericardium

have been associated with congenital thoracic duct anom-

aly. In cardiac surgery, the etiology of chylopericardium

after surgery is thought to involve disruption of the cardiac

lymphatic channels in the pericardial reflections. It is

apparent that any dissection within the pericardium can

potentially cause this problem. Pollard and Bakay [6, 7],

who both reported chylopericardium after CABG, impli-

cated venous thrombosis as a causative factor; other

authors have also suggested this as a mechanism after other

cardiac surgical procedures. Pollard and associates suggest

that occlusion of thoracic duct drainage secondary to

venous thrombosis leads to a rise in pressure within the

lymphatic system, with subsequent back-flow through

ruptured lymphatic vessels as the pathophysiological basis

of postoperative chylothorax [6]. In one case study, aber-

rant lymphatic connection and a duplicate thoracic duct

lead to spontaneous chylopericardium was demonstrated on

routine lymphoscintigraphy [8]. The patient was treated

surgically with partial pericardiectomy and resection of

thoracic duct.

This case demonstrates a spontaneous occurrence of

chylopericardium in the absence of recent surgery to

explain injury to the thoracic duct or venous thrombosis.

Fig. 1 a Chest radiography demonstrating a widened mediastinum.

Globular enlargement of the cardiac shadow with loss of cardiac

border. The left diaphragm cannot be distinguished. b Computed

tomographic section shows circumferential pericardial effusion

Gen Thorac Cardiovasc Surg

123

Previous experiences with Poland’s syndrome patients

after breast reconstruction have suggested that the likely

cause of chylopericardium in the post-acute setting is

likely due to aberrant thoracic duct which is a postulated

defect related to Poland’s sequence. Our case exemplifies

aberrant lymph channels on lymphoscintigram and the

development of chylopericardium in the absence of

recent surgery. We therefore postulate that the aberrant

duct system being the most likely cause of spontaneous

chylopericardium in this patient. However, currently,

there is no direct evidence linking this syndrome and

thoracic duct anomaly to spontaneous chylopericardium.

Future reports of similar cases may help consolidate and

confirm the current association.

Furthermore, demonstration of a chylous pericardial

effusion does not elucidate the site of origin of the fluid. In

a brief look at the cited literature, we conclude that such

effusions can be idiopathic and some have responded to

ligation of the thoracic duct. Embryological studies

regarding the development and the formation of the lym-

phatic system may help elucidate whether an interruption

to the vasculature at a similar gestational stage (i.e., in

accordance to developmental defects in Poland’s syn-

drome) might perturb lymphatic vessel formation.

Acknowledgments We would like to thank Dr Eva Wegner, nuclear

medicine consultant at Prince of Wales Hospital for the expert

opinion on the figures.

Fig. 2 a The above scan is a

‘Tc-99m antimony sulfide

colloid lymphoscintigraphy’.

Tracer was injected in the web

spaces of both hands, 20 MBq

in each of the injections. Tracer

flowing up the arms; at

30 min—uptake in axillary

lymph nodes; at 1 h—

physiological uptake seen in

liver and spleen; at 4 h—small

amount of activity in the chest,

possibly in the pericardial

distribution. This is not

confirmed on SPECT/CT due to

very low counts. b 5 h post

injection—solid arrow shows

low grade uptake in the

pericardium

Gen Thorac Cardiovasc Surg

123

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