Nigerian Journal of Paediatrics 2012;39 (1): 31 - 34

CASE REPORT

Mukhtar-Yola M

Pentalogy of Cantrell - A Case Report

Mohammad AM

Faroul ZL

from Nigeria.

Alhassan SU

Adeleke SI

Aji AA

Asani MO

DOI: http://dx.doi.org/10.4314/njp.v39i1.7

Received: 5th April 2011

Abstract : FB was delivered at

The pericardium was absent and the

Accepted: 14th October 2011

home to a para three low income

great vessels were exposed. There

mother, at term after an

was an epigastric omphalocoele and

Mukhtar-Yola M ( )

unsupervised pregnancy. At birth

a ventral diaphragmatic defect. The

Faroul ZL, Adeleke SI

she was noticed to have an anterior

diagnosis of Pentallogy of Cantrell

Asani MO

chest wall defect with a protruding

was made. The challenges involved

Department of Paediatrics

p u l s a t i n g

m a s s .

F u r t h e r

in the evaluation and management

National Hospital Abuja.

examination revealed a defect

of this case in a Nigerian setting are

Email: mariyamukhtar@yahoo.com

from the suprasternal notch to the

discussed.

Tel: +234 802 309 2757

xiphoid process (bifid sternum)

with the heart exposed and

Keywords:

Pentallogy, Cantrell,

Mohammad AM, Alhassan SU,

pulsating (ectopia cordis).

Nigeria.

Aji AA

Department of Surgery

Bayero University and Aminu

Kano Teaching Hospital,

Kano - Nigeria.

Introduction

The pentalogy is said to be fully expressed when all

the five components are present, however other

variants have been described by Toyoma et al. He

3

The pentalogy of Cantrell is a rare syndrome with an

1

estimated incidence of 5.5 per 1 million live birth

2

suggested the following classification of the

This syndrome was first described by Cantrell et all

pentalogy of Cantrell: class 1, definite diagnosis, with

in 1958 to include a pentad of findings of a midline

all five defects present; class 2, probable diagnosis,

supra umbilical thoracoabdominal wall defect, a

with four defects present, including intracardiac and

defect in the lower sternum, a deficiency of the

ventral wall abnormalities; and class 3, incomplete

diaphragmatic pericardium, a deficiency of the

expression, with various combinations of defects

anterior diaphragm and various congenital cardiac

present, including a sternal abnormality. A review of

abnormalities including an ectopia cordis. The

the literature yielded 58 reported patients with

pentalogy of Cantrell between 1987 and April 2007

. 4

Ectopia cordis may be complete or partial. The

pathogenesis of pentalogy of Cantrell has not been

Thirty-three patients were described as complete and

fully elucidated. Cantrell et al.

2

suggested an

23 patients as incomplete. Two patients were not

embryologic developmental failure of a segment of

clearly defined as complete or incomplete.

the lateral mesoderm around gestational age 1418

days. Consequently, the transverse septum of the

The prognosis in these patients depends on the

diaphragm does not develop, and the paired

severity of the cardiac anomalies and is usually very

mesodermal folds of the upper abdomen do not

poor, however a few cases have been succesfully

migrate ventromedially. Organs may eviscerate

operated on. There is a paucity of local data from

through the resulting sternal and abdominal wall

Africa and to our knowledge this is the first case being

defects.

reported from Nigeria inAfrica.

32

Case summary

takes several days or weeks). Surgery was done on the

2

nd

day of life with the aim of mobilizing skin to

Baby FB was referred to the special care baby unit

temporarily cover the heart before transport. The

(SCBU) of Aminu Kano Teaching Hospital Kano

intra operative findings were those of an exposed

(AKTH) at the age of 4 hours. She was a product of

heart, epigastric omphalocele, liver dome in chest,

term gestation delivered to a 23 year old Para 3 (all

complete bifid sternum and suggestive of a left to

alive and well) non diabetic non hypertensive

right shunt.

mother. Pregnancy was uneventful; mother had 2

visits to a primary health care centre but delivered at

Baby was admitted in the intensive care unit,

home as she did with her other children. Ultrasound

however 3 hours after surgery her condition

scan was not done in pregnancy. Baby cried well at

deteriorated following cardiac arrest, she was

birth and was immediately noticed to have an

resuscitated, ventilated, stitches released but all

anterior chest wall defect with a protruding pulsating

efforts proved abortive and she died at the age of 39

mass, and she was transferred to AKTH. There was

hours. The parents did not consent to a post mortem

no history of congenital malformation in the family,

examination .

mother is a full time house wife and father is a petty

trader, both are educated to primary school level and

Fig.1

are low income earners.

Examination revealed an active, uniformly pink,

anicteric, acyanosed baby drooling saliva. She

weighed 3.2kg with a length of 50cm and

occipitofrontal circumference of 35cm which are all

appropriate for gestational age. There were no

dysmorphic facies, head and neck were normal. The

anterior chest wall revealed a defect from the

suprasternal notch to the xiphoid process (complete

bifid sternum) with the heart exposed and pulsating

supported only by the great vessels (complete

ectopia cordis). The pericardium was absent and the

Fig.2

great vessels were exposed. There was an epigastric

omphalocoele and a ventral diaphragmatic defect.

Other systems were essentially normal. The

diagnosis of Pentallogy of Cantrell was made.

Baby was admitted and barrier nursed. Complete

blood count, urea and electrolytes, urinalysis,

random blood sugar were all within normal limits.

The chest X ray ( CXR)is as shown in fig.1,

Transesophageal/Doppler echocardiography, video

oesophagogram and blood gases /oxygen saturation

could not be done as the facilities were unavailable.

So it was not possible to definately determine

presence of intracardiac abnormalities.

Fig.3

She was commenced on oxygen, she was suctioned

PRN and commenced on 10% dextrose water,

Vitamin K and IV ceftazidime and gentamicin. Strict

input and output chart was maintained and the chest

wall defect was covered with sofratulle.

A multidisciplinary team involving the

paediatrician, paediatric and vascular surgeons ,

anaesthetist, cardiologist, social welfare was

constituted. Parents were counseled on the pros and

cons for a temporizing surgery prior to transfer to a

cardiothoracic surgical centre abroad if a

government waiver was obtained (This normaly

33

Fig 4

diagnosis at birth was such a shock for the family and

a great challenge for the managing doctors. We had a

lot of challenges in managing this case, from

investigations to management. This family is a low

2

income family and did not have any form of

insurance. So all health expenses were out of pocket

spending and they had severe constrains. Though we

had requested that the government should assist with

the finances, we also knew that it will take a while

before all the procedures could be completed and

patient transfered abroad. Moreover we could not do

some investigations because of lack of equipments.

There is also no established cardiac centre in the

country that we could immediately transfer the

patient to. So in order to curb infection the

multrtidisciplinary team agreed on a temorizing

Discussion

surgery prior to transfer.

The pentalogy of Cantrell is estimated to have an

Controversies exist with regards to the best surgical

incidence of 1:65,000 live births

5.

The main

technique f or this rare syndrome necessitating

abdominal wall malformation associated with this

multiple operations.

5

The treatment of the POC

syndrome is omphalocele found in 74.5% of patients

consists of corrective or palliative cardiovascular

followed by deformed regions in the lower sternum

surgery, correction of ventral hernia and

(59.4%), diaphragm (56.8%), and pericardium

diaphragmatic defects and correction of associated

(41.8%). Cardiac anomalies occurs in 83% of cases

anomalies. The best treatment strategy depends on

with interventricular communications as the most

the size of the abdominal wall defect, the associated

common intracardiac abnormality. Intra-cardiac

heart anomalies, and the type of EC. In most cases,

anomalies described include ventricular septal

death occurs in the first days of life, usually from

defect (100%), atrial septal defect (53%), tetralogy

infection, cardiac failure or hypoxaemia.

6

Surgical

of Fallot (20%), and ventricular diverticulum (20%).

2

therapy for neonates without overwhelmingly severe

cardiac abnormalities consists of covering the heart

Our patient had an extrathoracic ectopia cordis but

with skin without compromising venous return or

we could not determine if he had an associated intra

ventricular ejection, palliation of associated defects is

cardiac lesion as we could not do a transoesophageal

also very necessary. Occasional patients with the

echocardiography. In the most common thoracic

abdominal type of EC have survived to adult hood.

6

forms of ectopia cordis, the sternum is split and the

heart protrudes outside the chest. In other forms, the

Clinical parameters such as birth weight and

heart protrudes through the diaphraghm into the

immaturity, associated congenital heart anomalies,

abdominal cavity or may be situated in the neck.

6

and size of the abdominal defects are the main

Associated anomalies have been reported with POC

elements to predict the outcome of these patients:

and include craniofacial and central nervous system

particularly the prognosis of POC depends on the

anomalies such as cleft lip and/or palate,

severity of the associated cardiac anomaly. The

encephalocele, hydrocephalus, craniorachischisis

7,8,9

prognosis seems to be poorer in patients with the

limb defects such as clubfoot, absence of tibia or

complete form of pentalogy of Cantrell, EC, and

radius, and hypodactyly

10,11

also reported are

patients with associated anomalies.

abdominal organ defects such as gallblader agenesis

and polysplenia .

12

African children face a great challenge as facilities to

diagnose and care for complex problems such as

With prenatal ultrasonography, POC usually can be

this, are non- existant. When the diagnosis of

diagnosed in the first trimester of pregnancy.

13

Use

pentalogy of Cantrell is suspected, a

of prenatal magnetic resonance imaging (MRI) may

multidisciplinary approach is essential. A prenatal

enhance the visualization of the fetal anomalies and

medical team consisting of a gynecologist, a

help in planning of surgeries. In our patient the

14

neonatologist, pediatric cardiologist, pediatric

mother did not have adequate antenatal care and did

surgeon and geneticist, should use their expertise in

not get an ultrasound done in pregnancy as such the

choosing the best approach to this severe disorder.

34

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