~an?, ped;,,Ir. 4 5 ' 81. IP'tg
CONGENITAL P Y L O R I C S T E N O S l S IN INFANTg* A,M. G~rooz AND A.M. LAIIARI Bhopal
Hypertrophic pyloric itenosis is quite a common congenital anomaly and is one of those conditions wherein surgical intervent|0n offers good results. It is one of the commonest cat,ses of vomiting in neonates preceded only by faulty feeding. Calder (17S3) is credited to be the first to describe this clinical entity. However, it was Hirschsprung r of Copenhagen who coined the term congenital hypertrophic pyloric stenosis for feat~tres common to the disease. The incidence o f this disease is quite ~gh in Western co,Jmries like the U.S.A. ,nd the U.K. and is low in the I n d i a n subcontinent; the figures for U.S.A. (Must~,rd ~# al. 1969) being 3 cases per thousand live-births, for the U.K. {Rickham and ,Johnston 1969) 1:900. T h e corresponding figures in India are ! :3500. T h e Indian literature is also meagre in this respect. 35 cases have been reported by Fenn et al. in 1963 and l.t cases by T a h i i a n i and Fazil in 197t. Talwalker {1967} has c o m m e n t e d on the low incidence of the condition in Bombay. A low incidence has also been reported amongst the American and African Negroes. Material and Methods In the present series, 23 cases a d m i t t e d to the Gandhi Medical College and *Ffom the Departmenl r,f Surgery, Gandhi .Medical Colleg9 and Hamidia Hospital, Bhopal. Received on July 31, }9?7.
Hamidia Hospital, Dhopal, from 1965 to 1974 were studied under the headings of (i) Age and sex distribution (iS) Order of birth (iii) Symptomatology (iv) Time of o~set nf symptoms (v) Physical findings (vi) T r e a t m e n t administered (vii) Results of treatment and complications. Observations Table ! shows the age and sex incldence of congemtal hypertrophic pyloric stenosis. It is clear from the figures that the incidence is quite high in male infants and that in a predominantly high percentage of infams the age at which the child was brought to the hospital was between 5 to 8 weeks. T a b l e 2 shows the order of birth of the infants who were at~icted with the disease. As seen, the first-born child was more likely to have congenital pyloric stenosis. The symptomatology according to age group of infants born with congenital pyioric stenosis is given in Table 3. Vomiting is present in all cases as a cardinal symptom o~ the anomaly, and it is this persistent vomiting which ~.o alarms the parents that the child is brought to the hospital. Concurrent constipation is also a feature in many cases. In two cases diarrhoea was present which may be said to be unrelated to pyloric stenosis. Pyrexia was a featme of a few cases which can be accounted for by gastritis finding its way easily inlo a child already debilitated.
82
VOL. 45, No
INDgAN~OUitNAL 01~ PmDIATIUCg
T a b l e 1.
~4ge and u x distribution. Male
Age groups in weeks
Female
0
--
4
1
1
5
--
8
10
5
9
--
12
4
1
12
1
Above
T a b l e 2.
Birth ordtr of the c-Jes.
Birth o r d e r
Male
First Second Third Fourth
Table 3.
Female
I1
4
5
2
Nil
!
--
--
Clinical p/~tur# in d~elent oge groups.
Age group in week Symptoms
0
5
--
8
9
-- 12
I2&above
Vomiting
2
15
5
!
Constipation
t
6
3
1
Dehydration
2
14
4
Lump
2
15
2
1
1
8
3
1
--
4
2
Diarrhoea
W e i g h t loss Visible peristalsis Fever Abdominal
distension
83
OHOUI AND LAHAR[ --CONGENITAl. PYl,(.)ltlC | T E N O S | |
4.
Table
Tirnt of first appcarance of symptoms in cJse.~ undel study.
f T i m e o f onset in weeks
T o t a l cases
0
--
2
Nil
3
--
4
1
5 7
---
6 8
3 11
9
--
10
7
11
--
12
1
12 a n d a b o v e
A m o n g s t the signs, the finding o f a lump is the most p a t h o g n o m o n l c of. this
b r o u g h t early to tl,e hospital were in a c o m p a r a t i v e l y better condition. T a b l e 4 shows that tF.e age at onset o f the s y m p t o m s varies from as early as 4 weeks to as late as the l l t h week o f life. T h e m a x i m u m n u m b e r o f cases d e v e l o p e d the s y m p t o m s b e t w e e n the 7th and 81h week o f life. T a b l e 5 depicts the course which the child has u n d e r g o n e d u r i n g the stay in the hospital including surgery and its complica-
dinlcal entity. I t has been noted t h a t not only one, but r e p e a t e d e x a m i n a t i o n o f the ;rrltable a n d u n c o - o p e r a t i v e child is indispensable. Visible neristalsis is seen in wine cases but it c a n be p a r t l y a t t r i b u t e d to the e m a c i a t e d c o n d i t i o n o f the child due to lack o f nutrition. Loss o f weight is a natural corollary o f v o m i t i n g , d e h y d r a tion and const;.pation. Infants who were
Table
5.
PoJt ope:ativr comO,i~atif.ns in di.~ereut age g,oups. Post operative complications
Age g r o u p in weeks Pyrexia
Gastroenteritis
W o u n d sepsis
0
--
4
2
2
I
5
-
8
11
1
5
9
~
12
2
1
1
--
--
A b o v e 12
1
Burst a b d o m e n
Death
a
~
IHDUAN ~01JRNAt. OP I~UnTATRVr~q
tleml,
All the infants, excluding four, were
9- ' p ~ a t e d upon, Nor.: of the children operated upon died nor developed a bur, t atglomen. The F r c d e t - R a m s t e d t pylorom.vot~rny was done in all the cases. Pyrexia wa~ the most c o m m o n post-operative compliealion fol!owed b 3, wound sepsis. Diarrhoea and cough were found in a few others. Discussion
In the present seri,'s the condition was furred to be c o m m o n in male infants; the percentage of males being 69.5 and females 30.5. T h e available literature also corroborates the fact that males shoiv a high in,'~dence oft.h!, anon,.z!y. Fur:h:r it has been noted that congenital hypertrophic pyloric stenosls is m o r e c o m m o n in the first-born rather than in the second or third infant. I n the present study, 69.5% of the children were first-born. T h e incidence of pyloric atenosis in the family members was not elicited in a majority of the cases. A s ~ c i a t e d congenital defects like cleft lip, syndactyly, hydrocephalus have been noted in a few cases. T h e average age of onset of the symptoms was between 7-8 weeks of life. T h e r e was invariably a per;.od of time between the onset of the disease and attendance at the hospital. This ca,', be attributed to lack of medical awareness and a conservative line of t r e a t m e n t instituted by quacks. T h e medical regimen of treatment with antispasmodics and eumydrine failed to afford much relief to the infants. T h e chddren were built up before any surgery was instituted because they were invariably d e h y d r a t e d a n d undernourished. T h e presenting feature of all the cases in the series was vomiting. However, it
V.~:.. 45, No. "~ was not projectile in nature in all of the I t was not associated with bile in any the cases. Concomitant constipation all deh~'dration was also seen in almost all tl, cases. As a corollary of the association von,iting with dehydration, loss of weilrl~ oec~Jrred in all cases. Many hypotheses have been advan 9 to explain the gencsh of this clinlcal emit It has been shown that beyond and p r o f real to the region of hypertrophy in U pyloric area tile gross and microscop' picture is absolutely normal. I n the regio of the pyiorus there is gross hypertrop of the muscles which has been desczib by m a n y as the pyloric turnout. Th thickened hypertrophied pylorus conshll of an actual increase in the n u m b e r muscle cells as well as enlargement of th individual fibres. These changes ar t, sually confined to the circular layer muscle fibres. Initially, there is no inflam marion, but later, mucosal oedema a,~ round cell infiltration m a y occur and the inte,~sify and provoke the symptoms ant signs. Vomiting is said to be initiated b these alterations in the pyloric mucosa. Studies of th~ nlye,teric plexus in the. normal h u m a n foetus, normal child and those with pyloric stenosis have indicated that it is failure of the d e v e l o p m e n t of the ganglion cells rather than degeneratior which is the cause of the condition. Belding and K e r n o h a n (I.953) reporte# that the cytoplasm of the ganglion cell a p p e a r e d to be in a state of distintegration in infants with co,~genital pyluric stenosis Theories of spasm coupled with primar~ hypertrophy have been advocated by somc workers. I n a couple of cases the tissue was taken and subjected to histopathologi
85
@i(t)01 A::n LAJIA~!--CONCJRNITALPYLORIC STKNOllSIN INFANTS
r examination. The ganglion ceils were found to be ill.deveh,ped and scanty. V,sihle peri~lal,is due to forcible confractions further became prominent due tn Ihe emaciated condition of these newl,vborn patients. The peristalsis is seen to r u , from left to right and iq easily di+cernible in an infant who ha,q been fed recently. The p,vlorie . t m o u r is palpable in almost all cases and this feature clinches the diagnosis. It is usually best feh between the umbilicus and the right subcostal margin. Besides the routine investigation of ]laematogram and urine analysis, barium meal examinations were carried o~t in all eases to confirm the diagnosis A large amount of residual barium in the stomach for a prolonged period is a point in support of the diagnosis. The conditions which can mimic co:tgenital hypertrophic pyloric stenosis are, mismanaged feeding, cardioachlasia of the ee~ophagus, pylorospasm, gastro-oesophageal reflux, neonatal intestinal obstruction e~pecially malrotation, intracraniai birth injuries, meningitis and other infections. Summary Congenital hypertrophic pyloric stenosls occurs three or four times in every 1000 births. The newborn infants are unable to retain the feeds and hence are undernourished and ill-developed. The 9;'omitus has no bile and is initially reg~rgirant but later projectile. First born males are the usual victims of this congenital anomaly. In the present series the family hhtory of similar cases could not be elicited in a majority of the patients. The exact aetiology and pathogenesis of this condition
is still obscure but many theories have been put forth to explain the genesis of it. The pathology i~ confined remarkably to the region of the p,vlorus which shows hypertrophy of its circular muscle fibres reducing the lumen of the pylorus to virtually a potential space. Vomiti,,~, dehydration , presence of - lump, visible peristalsis and constipation leave no doubt about the diagnosis which is confirmed by barium meal examination. The FredetRamstedt pylorom,votomy was performed in a majority of the infants with good results. The post operative results were encouraging. 14eferencea Belding, H.H. and Kernohan. V.W. (1953). A morphological study of myenteric plexus and m,,,culalure of the pylorus with special references to the changes in hypertrophic pylorir stenosis. Surf. Gy.*c. & ObJt. 9"/, 322, Benso., C.D. a,~d Warden, .~'[..J. (1957). $urg. Obse. and GV~c. IOS, 34S, Donovan, E.J. and atanlcy Brown, E.G., (1962). Sur&. G3net. & Obsf, 115 403. ]Penn, A.S., Sahai, O.B., Webb, J.K.G.,Bhatt, M.S. and Joseph, L.B.M. (1963), Indian 3. $wg. 25, 399. Mus|ard, W.T, Ravltch, M.M., Snyder, W.H., Welch, K.J. and Benson, C.D. (19~,9j Pocdiatr. 5utg. Vol. 2, 2~,d Ed. ?'tar Book Medical Publisher 1~. Chicago 818, 1969. Ram Kumar, L. and Gupta, S.L. (1957). Congenital hypertrophic pylorlc steno-*is, lndi~ 3. C'Md. lihh. g, 235.
Rickham P.P., Johnston, J.H (lgGS~. Neonatal, Surgery, Butttru~thf London, 271. Schaff"r. A,A, Erbs, J, (1948). Hypertrophic pylorlc uenosis. ~w+. G),~e+.06,t. 86, 4:). Swan,T.T. (1961). Congenital pyloric stenosis in African infAntSB.M.~. I, 545. ['abilani, N., Fazil, M. (1972}. Congenital pyloric slenosis, Indian Pntdialr. 9, 552, TahvalluLr, V. (1967). Hypertrophic pyloric stenosis in infants. Pard. Chlr. I, 19"/, 203.
CONGENITAL P Y L O R I C S T E N O S l S IN INFANTg* A,M. G~rooz AND A.M. LAIIARI Bhopal
Hypertrophic pyloric itenosis is quite a common congenital anomaly and is one of those conditions wherein surgical intervent|0n offers good results. It is one of the commonest cat,ses of vomiting in neonates preceded only by faulty feeding. Calder (17S3) is credited to be the first to describe this clinical entity. However, it was Hirschsprung r of Copenhagen who coined the term congenital hypertrophic pyloric stenosis for feat~tres common to the disease. The incidence o f this disease is quite ~gh in Western co,Jmries like the U.S.A. ,nd the U.K. and is low in the I n d i a n subcontinent; the figures for U.S.A. (Must~,rd ~# al. 1969) being 3 cases per thousand live-births, for the U.K. {Rickham and ,Johnston 1969) 1:900. T h e corresponding figures in India are ! :3500. T h e Indian literature is also meagre in this respect. 35 cases have been reported by Fenn et al. in 1963 and l.t cases by T a h i i a n i and Fazil in 197t. Talwalker {1967} has c o m m e n t e d on the low incidence of the condition in Bombay. A low incidence has also been reported amongst the American and African Negroes. Material and Methods In the present series, 23 cases a d m i t t e d to the Gandhi Medical College and *Ffom the Departmenl r,f Surgery, Gandhi .Medical Colleg9 and Hamidia Hospital, Bhopal. Received on July 31, }9?7.
Hamidia Hospital, Dhopal, from 1965 to 1974 were studied under the headings of (i) Age and sex distribution (iS) Order of birth (iii) Symptomatology (iv) Time of o~set nf symptoms (v) Physical findings (vi) T r e a t m e n t administered (vii) Results of treatment and complications. Observations Table ! shows the age and sex incldence of congemtal hypertrophic pyloric stenosis. It is clear from the figures that the incidence is quite high in male infants and that in a predominantly high percentage of infams the age at which the child was brought to the hospital was between 5 to 8 weeks. T a b l e 2 shows the order of birth of the infants who were at~icted with the disease. As seen, the first-born child was more likely to have congenital pyloric stenosis. The symptomatology according to age group of infants born with congenital pyioric stenosis is given in Table 3. Vomiting is present in all cases as a cardinal symptom o~ the anomaly, and it is this persistent vomiting which ~.o alarms the parents that the child is brought to the hospital. Concurrent constipation is also a feature in many cases. In two cases diarrhoea was present which may be said to be unrelated to pyloric stenosis. Pyrexia was a featme of a few cases which can be accounted for by gastritis finding its way easily inlo a child already debilitated.
82
VOL. 45, No
INDgAN~OUitNAL 01~ PmDIATIUCg
T a b l e 1.
~4ge and u x distribution. Male
Age groups in weeks
Female
0
--
4
1
1
5
--
8
10
5
9
--
12
4
1
12
1
Above
T a b l e 2.
Birth ordtr of the c-Jes.
Birth o r d e r
Male
First Second Third Fourth
Table 3.
Female
I1
4
5
2
Nil
!
--
--
Clinical p/~tur# in d~elent oge groups.
Age group in week Symptoms
0
5
--
8
9
-- 12
I2&above
Vomiting
2
15
5
!
Constipation
t
6
3
1
Dehydration
2
14
4
Lump
2
15
2
1
1
8
3
1
--
4
2
Diarrhoea
W e i g h t loss Visible peristalsis Fever Abdominal
distension
83
OHOUI AND LAHAR[ --CONGENITAl. PYl,(.)ltlC | T E N O S | |
4.
Table
Tirnt of first appcarance of symptoms in cJse.~ undel study.
f T i m e o f onset in weeks
T o t a l cases
0
--
2
Nil
3
--
4
1
5 7
---
6 8
3 11
9
--
10
7
11
--
12
1
12 a n d a b o v e
A m o n g s t the signs, the finding o f a lump is the most p a t h o g n o m o n l c of. this
b r o u g h t early to tl,e hospital were in a c o m p a r a t i v e l y better condition. T a b l e 4 shows that tF.e age at onset o f the s y m p t o m s varies from as early as 4 weeks to as late as the l l t h week o f life. T h e m a x i m u m n u m b e r o f cases d e v e l o p e d the s y m p t o m s b e t w e e n the 7th and 81h week o f life. T a b l e 5 depicts the course which the child has u n d e r g o n e d u r i n g the stay in the hospital including surgery and its complica-
dinlcal entity. I t has been noted t h a t not only one, but r e p e a t e d e x a m i n a t i o n o f the ;rrltable a n d u n c o - o p e r a t i v e child is indispensable. Visible neristalsis is seen in wine cases but it c a n be p a r t l y a t t r i b u t e d to the e m a c i a t e d c o n d i t i o n o f the child due to lack o f nutrition. Loss o f weight is a natural corollary o f v o m i t i n g , d e h y d r a tion and const;.pation. Infants who were
Table
5.
PoJt ope:ativr comO,i~atif.ns in di.~ereut age g,oups. Post operative complications
Age g r o u p in weeks Pyrexia
Gastroenteritis
W o u n d sepsis
0
--
4
2
2
I
5
-
8
11
1
5
9
~
12
2
1
1
--
--
A b o v e 12
1
Burst a b d o m e n
Death
a
~
IHDUAN ~01JRNAt. OP I~UnTATRVr~q
tleml,
All the infants, excluding four, were
9- ' p ~ a t e d upon, Nor.: of the children operated upon died nor developed a bur, t atglomen. The F r c d e t - R a m s t e d t pylorom.vot~rny was done in all the cases. Pyrexia wa~ the most c o m m o n post-operative compliealion fol!owed b 3, wound sepsis. Diarrhoea and cough were found in a few others. Discussion
In the present seri,'s the condition was furred to be c o m m o n in male infants; the percentage of males being 69.5 and females 30.5. T h e available literature also corroborates the fact that males shoiv a high in,'~dence oft.h!, anon,.z!y. Fur:h:r it has been noted that congenital hypertrophic pyloric stenosls is m o r e c o m m o n in the first-born rather than in the second or third infant. I n the present study, 69.5% of the children were first-born. T h e incidence of pyloric atenosis in the family members was not elicited in a majority of the cases. A s ~ c i a t e d congenital defects like cleft lip, syndactyly, hydrocephalus have been noted in a few cases. T h e average age of onset of the symptoms was between 7-8 weeks of life. T h e r e was invariably a per;.od of time between the onset of the disease and attendance at the hospital. This ca,', be attributed to lack of medical awareness and a conservative line of t r e a t m e n t instituted by quacks. T h e medical regimen of treatment with antispasmodics and eumydrine failed to afford much relief to the infants. T h e chddren were built up before any surgery was instituted because they were invariably d e h y d r a t e d a n d undernourished. T h e presenting feature of all the cases in the series was vomiting. However, it
V.~:.. 45, No. "~ was not projectile in nature in all of the I t was not associated with bile in any the cases. Concomitant constipation all deh~'dration was also seen in almost all tl, cases. As a corollary of the association von,iting with dehydration, loss of weilrl~ oec~Jrred in all cases. Many hypotheses have been advan 9 to explain the gencsh of this clinlcal emit It has been shown that beyond and p r o f real to the region of hypertrophy in U pyloric area tile gross and microscop' picture is absolutely normal. I n the regio of the pyiorus there is gross hypertrop of the muscles which has been desczib by m a n y as the pyloric turnout. Th thickened hypertrophied pylorus conshll of an actual increase in the n u m b e r muscle cells as well as enlargement of th individual fibres. These changes ar t, sually confined to the circular layer muscle fibres. Initially, there is no inflam marion, but later, mucosal oedema a,~ round cell infiltration m a y occur and the inte,~sify and provoke the symptoms ant signs. Vomiting is said to be initiated b these alterations in the pyloric mucosa. Studies of th~ nlye,teric plexus in the. normal h u m a n foetus, normal child and those with pyloric stenosis have indicated that it is failure of the d e v e l o p m e n t of the ganglion cells rather than degeneratior which is the cause of the condition. Belding and K e r n o h a n (I.953) reporte# that the cytoplasm of the ganglion cell a p p e a r e d to be in a state of distintegration in infants with co,~genital pyluric stenosis Theories of spasm coupled with primar~ hypertrophy have been advocated by somc workers. I n a couple of cases the tissue was taken and subjected to histopathologi
85
@i(t)01 A::n LAJIA~!--CONCJRNITALPYLORIC STKNOllSIN INFANTS
r examination. The ganglion ceils were found to be ill.deveh,ped and scanty. V,sihle peri~lal,is due to forcible confractions further became prominent due tn Ihe emaciated condition of these newl,vborn patients. The peristalsis is seen to r u , from left to right and iq easily di+cernible in an infant who ha,q been fed recently. The p,vlorie . t m o u r is palpable in almost all cases and this feature clinches the diagnosis. It is usually best feh between the umbilicus and the right subcostal margin. Besides the routine investigation of ]laematogram and urine analysis, barium meal examinations were carried o~t in all eases to confirm the diagnosis A large amount of residual barium in the stomach for a prolonged period is a point in support of the diagnosis. The conditions which can mimic co:tgenital hypertrophic pyloric stenosis are, mismanaged feeding, cardioachlasia of the ee~ophagus, pylorospasm, gastro-oesophageal reflux, neonatal intestinal obstruction e~pecially malrotation, intracraniai birth injuries, meningitis and other infections. Summary Congenital hypertrophic pyloric stenosls occurs three or four times in every 1000 births. The newborn infants are unable to retain the feeds and hence are undernourished and ill-developed. The 9;'omitus has no bile and is initially reg~rgirant but later projectile. First born males are the usual victims of this congenital anomaly. In the present series the family hhtory of similar cases could not be elicited in a majority of the patients. The exact aetiology and pathogenesis of this condition
is still obscure but many theories have been put forth to explain the genesis of it. The pathology i~ confined remarkably to the region of the p,vlorus which shows hypertrophy of its circular muscle fibres reducing the lumen of the pylorus to virtually a potential space. Vomiti,,~, dehydration , presence of - lump, visible peristalsis and constipation leave no doubt about the diagnosis which is confirmed by barium meal examination. The FredetRamstedt pylorom,votomy was performed in a majority of the infants with good results. The post operative results were encouraging. 14eferencea Belding, H.H. and Kernohan. V.W. (1953). A morphological study of myenteric plexus and m,,,culalure of the pylorus with special references to the changes in hypertrophic pylorir stenosis. Surf. Gy.*c. & ObJt. 9"/, 322, Benso., C.D. a,~d Warden, .~'[..J. (1957). $urg. Obse. and GV~c. IOS, 34S, Donovan, E.J. and atanlcy Brown, E.G., (1962). Sur&. G3net. & Obsf, 115 403. ]Penn, A.S., Sahai, O.B., Webb, J.K.G.,Bhatt, M.S. and Joseph, L.B.M. (1963), Indian 3. $wg. 25, 399. Mus|ard, W.T, Ravltch, M.M., Snyder, W.H., Welch, K.J. and Benson, C.D. (19~,9j Pocdiatr. 5utg. Vol. 2, 2~,d Ed. ?'tar Book Medical Publisher 1~. Chicago 818, 1969. Ram Kumar, L. and Gupta, S.L. (1957). Congenital hypertrophic pylorlc steno-*is, lndi~ 3. C'Md. lihh. g, 235.
Rickham P.P., Johnston, J.H (lgGS~. Neonatal, Surgery, Butttru~thf London, 271. Schaff"r. A,A, Erbs, J, (1948). Hypertrophic pylorlc uenosis. ~w+. G),~e+.06,t. 86, 4:). Swan,T.T. (1961). Congenital pyloric stenosis in African infAntSB.M.~. I, 545. ['abilani, N., Fazil, M. (1972}. Congenital pyloric slenosis, Indian Pntdialr. 9, 552, TahvalluLr, V. (1967). Hypertrophic pyloric stenosis in infants. Pard. Chlr. I, 19"/, 203.
