cantrell et al . reported five cases in 1958 , describing a pentad of findings that included : ( 1 ) supraumbilical wall defect , ( 2 ) defect of the lower sternum , ( 3 ) deficiency of the anterior diaphragm , ( 4 ) defect of the diaphragmatic pericardium , and ( 5 ) various intracardiac defects .
occurrence of the full pentalogy is rare and the survival rate of the patients with complete pentalogy is low , with outcome being dependent on the severity of the cardiac malformation , extra cardiac defects , and other associated anomalies .
the child was the second for the parents ; the first had died at one year of age from ill health .
the baby was born in a peripheral health center and was 18 hours old when she was transferred to our hospital .
pregnancy and delivery were supervised and there was no history of maternal illness during the pregnancy .
examination revealed a non - cyanosed anicteric baby , who was not in any obvious respiratory distress .
the chest examination showed an obvious anterior chest wall defect covered by skin , which was pink and membranous over the epigastrium .
breath sounds were well heard all over the chest , only the first and second heart sounds were heard over the precordium .
child with epigastric omphalocoele echocardiography could not be performed in our center because the echocardiography machine could not be adapted for pediatric use , and the parents could not afford the investigation in a private diagnostic center .
the child was on admission for seven weeks , and 70% alcohol ( ethanol ) dressing of the membranous covering over the epigastrium was done daily to encourage epithelialization .
the father who was a carpenter could not afford any further investigations , or the cost of admission , and requested for child 's discharge against medical advice .
a 36-hour - old female child was brought to our hospital following referral from a health center .
the child cried spontaneously at birth , and the weight on presentation was 2.5 kg .
the mother was a 30-year - old para 3 ( two were alive ) woman .
physical examination showed an acyanosed , anicteric child who had a right subconjuctival hemorrhage and was not in any obvious respiratory distress .
only heart sounds i and ii were heard . a thin membranous covering of the epigastrium
about 3 cm in diameter was noted , with a raised fibrous raphe , of about 5 mm extending from the membranous covering of the epigastrium to a normal appearing umbilical stump [ figure 2 ] .
noticeable midline fibrous raphe a chest radiograph revealed a centrally placed globular heart , with good lung expansion and markings , bilaterally [ figure 3 ] .
echocardiography could not be done in our center , and the parents could not afford the investigation in a private diagnostic center .
parents who were peasants requested for the child 's discharge , against medical advice , on financial grounds .
the child was discharged on the sixth day of admission and has been lost to follow up .
chest x - ray showing centrally placed globular heart this patient was referred to our unit from a peripheral health center at six weeks of age .
the child was referred on account of a chest wall defect , which was noticed at birth .
the mother was a remarried widow who had three other children who were alive and well .
chest wall examination revealed a complete sternal defect with visible cardiac impulse beneath the anterior chest wall skin covering .
the heart rate was 144 per minute , and only heart sounds i and ii were heard , no murmur .
a chest x - ray revealed a centrally placed heart , with normal lung fields .
the child was offered admission , with a view to have an early surgical intervention , but the parents refused this on financial grounds .
the child was the second for the parents ; the first had died at one year of age from ill health .
the baby was born in a peripheral health center and was 18 hours old when she was transferred to our hospital .
pregnancy and delivery were supervised and there was no history of maternal illness during the pregnancy .
examination revealed a non - cyanosed anicteric baby , who was not in any obvious respiratory distress .
the chest examination showed an obvious anterior chest wall defect covered by skin , which was pink and membranous over the epigastrium .
breath sounds were well heard all over the chest , only the first and second heart sounds were heard over the precordium .
child with epigastric omphalocoele echocardiography could not be performed in our center because the echocardiography machine could not be adapted for pediatric use , and the parents could not afford the investigation in a private diagnostic center .
the child was on admission for seven weeks , and 70% alcohol ( ethanol ) dressing of the membranous covering over the epigastrium was done daily to encourage epithelialization .
the father who was a carpenter could not afford any further investigations , or the cost of admission , and requested for child 's discharge against medical advice .
a 36-hour - old female child was brought to our hospital following referral from a health center .
the child cried spontaneously at birth , and the weight on presentation was 2.5 kg .
the mother was a 30-year - old para 3 ( two were alive ) woman .
physical examination showed an acyanosed , anicteric child who had a right subconjuctival hemorrhage and was not in any obvious respiratory distress .
only heart sounds i and ii were heard . a thin membranous covering of the epigastrium
about 3 cm in diameter was noted , with a raised fibrous raphe , of about 5 mm extending from the membranous covering of the epigastrium to a normal appearing umbilical stump [ figure 2 ] .
noticeable midline fibrous raphe a chest radiograph revealed a centrally placed globular heart , with good lung expansion and markings , bilaterally [ figure 3 ] .
echocardiography could not be done in our center , and the parents could not afford the investigation in a private diagnostic center .
parents who were peasants requested for the child 's discharge , against medical advice , on financial grounds .
the child was discharged on the sixth day of admission and has been lost to follow up .
this patient was referred to our unit from a peripheral health center at six weeks of age .
the child was referred on account of a chest wall defect , which was noticed at birth .
the mother was a remarried widow who had three other children who were alive and well .
chest wall examination revealed a complete sternal defect with visible cardiac impulse beneath the anterior chest wall skin covering .
the heart rate was 144 per minute , and only heart sounds i and ii were heard , no murmur .
a chest x - ray revealed a centrally placed heart , with normal lung fields .
the child was offered admission , with a view to have an early surgical intervention , but the parents refused this on financial grounds .
cantrell 's pentalogy ( cp ) is a rare congenital anomaly in which defects of the anterior abdominal wall , sternum , and diaphragm are associated with cardiac anomalies.[14 ] full pentalogy occurs rarely .
presence of the pentad is considered by some to be the full spectrum , with absence of some of the components seen as incomplete forms of the pentalogy .
cp is 2.7 times more common in boys , and african americans may be more predisposed .
the error in the embryology appears to occur in a segment of the lateral mesoderm .
the cardiac abnormalities are due to a faulty development of the epimyocardium and the sternal and abdominal wall defects represent a faulty ventromedial migration of the paired mesodermal primordial structures .
the diaphragmatic and the pericardial defects are secondary to a developmental failure of the transverse septum .
these abnormalities seem to occur around day 14 to 18 of embryonic life . through the resulting sternal and abdominal wall defects
many factors may play a role in the pathogenesis of cp and ventral midline defects .
judging from the phenotypic variability of these defects , factors such as genetic aberrations and external mechanical forces may be implicated .
some workers believe that the pathogenesis of this syndrome can be linked to mechanical teratogenesis ( band disruptions ) following rupture of the chorion or yolk sac .
the mechanical teratogenesis concept seeks to explain the numerous malformations that could be seen in these infants , exclusive of the torso .
toyama , after a review of 61 cases of cp , suggested the following classifications : class 1 , definite diagnosis , with all five defects present ; class 2 , probable diagnosis , with four defects , including intracardiac and ventral abdominal wall abnormalities present ; and class three , incomplete expression , with various combinations of defects , including a sternal abnormality present .
our three cases may be regarded as incomplete expression of the syndrome , as we were not able to demonstrate an intracardaic defect or a deficiency of the anterior diaphragm in any of them , due to constraints in performing echocardiography .
patients should be reviewed by a pediatric anesthetist before surgery , due to the peculiarities of anesthesia that may arise from the component anomalies .
we intended a repair of the sternal cleft in these patients , to provide a bony protection for the mediastinal structures .
the technical and financial constraints we encountered , however , precluded this line of management .
the constraints we experienced in the management of these patients are rather sadly recurrent in pediatric surgical practice in this region , where a majority of the population survive on less than 1 us dollar daily , and only about 2  3% of government expenditure is allocated to health . due to a non - encompassing health insurance ,
therefore , healthcare is in direct competition with the basic needs of food , shelter , and clothing .
this will explain the willingness of our patients relatives to seek for discharge against medical advice on financial grounds .
policy makers do not regard surgery as a component of basic healthcare and often dismiss pediatric surgery as being capital intensive and catering to an insignificant minority .
this inclination has accounted for the paucity of equipments needed for the optimal care of our patients .
surgical repair of the sternal cleft should be done early to protect the mediastinal structures and eliminate a cosmetic deformity .
 pentalogy of cantrell is a rare upper midline syndrome that may present in association with anomalies outside the torso . 
 the pentad  the supraumbilical body wall defect , sternal defect , deficiency of the anterior diaphragm , defect of the diaphragmatic pericardium , and the intracardiac anomalies  was first described by cantrell et al . , in 1958 . 
 the defect is said to be more common in males , and survival is dependent on the cardiac malformations and on the degree of completeness of the syndrome . 
 we report three cases of cantrell 's pentalogy managed in our unit . 
 two of the patients were females and one a male . 
 all were seen at peripheral health centers before being referred to us . 
 age at presentation for the girls was 18 hours and 36 hours , respectively , the boy presented at the age of six weeks . 
 all of their parents were unschooled manual workers . 
 all patients presented with a defect in the supraumbilical body wall , bifid sternum , and a visible cardiac impulse . 
 we were unable to do echocardiography to rule out intracardiac anomalies in the three patients . 
 the thin membranous covering of the epigastrium in the female patients was managed conservatively . 
 both female patients were discharged against medical advice as requested by their parents , due to financial constraints . 
 the male patient was lost to follow up after two clinic visits . 
 a multidisciplinary approach to the management of this syndrome is recommended . 
INTRODUCTION
CASE REPORTS
Case 1
Case 2
Case 3
DISCUSSION