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Congenital eventration of the diaphragm
CONGENITAL
EVENTRATION SURGICAL
J.
DEWEY
BISGARD,
MANAGEMENT
M.D. AND GEORGE E. ROBERTSON, M.D. OMAHA,
A
OF THE DIAPHRAGM
NEBRASKA
of eventration of the diaphragm in an infant which was cured b,y operation is reported
Eventration of the diaphragm may be defined as an abnormaIIy high or elevated position of one leaf of the diaphragm as a
CASE
FIG. I. Preoperative film. The right diaphragm is elevated to the Ievel of the posterior portion of the fifth rib and the heart and mediastinum are displaced to the left. The area of cardiac dullness extends to the Ieft costal margin with only a smaI1 segment of unobscured lung in the left costalphrenic sinus. The inferior margin of the liver is ahove the eleventh rib.
because, (I) the writers are unable to find a report of a previous attempt to treat this condition in an infant by operation and (2) they suspect that some of the deaths in the newborns which are attributed to other causes of cyanosis and dyspnea are in reaIity the resuIt of unrecognized congenital eventration, which, as in our case, have prospect of surgica1 cure.
aplasia or atrophy of result of paralysis, the muscle fibers but with no break in the continuity of the Ieaf. Its unbroken continuity differentiates it from diaphragmatic hernia. Admitted by a11 authors an inappropriate term, eventration has been estabIished by general use and the Iack of a better name. The condition has been observed in peopIe of all ages and, although 95
96
American
Journal
ofSurgery Bisgard,
Robertson-Eventration
this paper is concerned with the probIem in infants, it is necessary to refer to subject matter which deals with the subject as a whole.
FIG. 2. Diagrammatic representation of the position of the diaphragm (dotted tine) and the resultant distortion of the intrathoracic viscera. This is afso illustrated in the procedure used in the writer’s case. Shortening was accomplished by pIication with three superimposed rows of interrupted mattress sutures through an intrathoracic approach.
Bibliographers have credited Petit with the first description “of the condition. A case report with postmortem findings appeared in the posthumous edition of his work published in 1774. Infrequently thereafter, isoIated cases were reported and in 1913 Bergman reviewed the Iiterature to that date and collected thirty-one cases. Three years later Bayne-Jones reported a coIIection of forty-five cases including one of his own. In 1921, Korns was abIe to assembIe sixty-five cases and in the most recent coIIection Reed and Borden, in 1935, reported 183 cases. Most of the reported cases were aduIts and it is probable that in most of them the lesion was acquired. A few were incidenta findings and presumably caused no symptoms. The remaining cases presented a variety of symptoms which fall into four general groups: (I) gastric, (2) cardiac, (3) pmmonary and (4) pleuropulmonary. If the number of case reports is a criterion, eventration in infants is indeed a rarity. In his review of the Iiterature up to rgr6 Bayne-Jones collected three cases:
OCTOB&R, 1949
a fetus, a newborn, one day old, and an infant of three months. In each instance the diagnosis was made at postmortem examination. In the eighty-three cases collected from the Iiterature from 192 I to 1933 by Reed and Borden there were only five infants. From 1935 to 1945, at Ieast nine additional cases in infants have been reported in&ding our case, Undoubtediy these figures give a very inaccurate conception of the incidence. In addition to the unreported cases it is very probable that some of the deaths in infants which have been attributed to other causes of cyanosis and dyspnea have in reality resuhed from eventration. The writers’ case was unusua1 in that the right haIf of the diaphragm was involved. Of the 183 cases coIIected by Reed and Borden only eighteen were on the right side. The symptoms in the infants, when recorded, were cyanosis and dyspnea as in our ‘case. At postmortem examination the elevated haIf of the diaphragm has presented varying degrees of atrophy; in the extreme a very thin fibrous membrane was present with compIete absence of muscIe fibers. Not infrequently there are associated maldeveIopments of the Iung, heart or fiver, faiIure of intestina1 rotation or unassociated congenital deformities as cIeft paIate. In the series of 183 cases reviewed by Reed and Borden direct operative attack upon the diaphragm was done in onIy four cases. Reports of two additional cases were found in the literature which has accumulated since 1935. In aI1 cases the diaphragm was Iowered by shortening, in one case by excision of a segment and in five by plication. One patient was plicated from the abdominaI side, the others from above through an intrathoracic exposure. One died three days after operation and the others obtained partial or compIete reIief of symptoms. The ages of these patients varied from ten to forty-four years. So far as we were able to ascertain from a carefui review of the available literature,
NEW
SERIES VOL. LXX, No. I
Bisgard,
our case is not only the first instance of a surgica1 cure of this in an infant but aIso the only
Robertson-Eventration recorded condition record of
FIG. 3. This roentgenogram made immediately foIIowing operation shows the right diaphragm Iowered to a position below the left with comptetr aeration of both lungs and normal intrathoracic reIations. The inferior border of the Iiver is severa centimeters beIow the twelfth rib.
operative group.
intervention
CASE
in a case in this
age
REPORT
Baby W., a male infant six weeks oId, was brought to the Bishop Clarkson Memorial HospitaI in an improvised oxygen chamber. He was born at fulI term without operative assistance and appeared normal at birth. On about the tenth day after birth the mother became was somewhat impressed that respiration labored and that cyanosis developed when the infant cried. Feeding became progressively more diff%uIt and he faiIed to gain weight. By the fifth week after birth the dyspnea and cyanosis had increased to such an alarming degree that it was necessary to administer oxygen. The father, who is an automobiIe mechanic in a rura1 community, fashioned a very satisfactory oxygen tent with the help of the family physician. AIthough the tent made
American
Journal
of Surger
97
surviva1 possible there was no improven lent in the infant’s condition after one week so he was brought to the hospital.
FIG. 4. At the time of dismissal
from the hospita1, ten days after operation, the Iowered position of the right diaphragm was still we11 retained.
Examination showed a well developed but poorly nourished infant of six weeks showing moderate dyspnea and rather marked cyanosis both of which became very marked with crying and exertion. The right side of the chest moved less than the Ieft and there was Iateral retraction with inspiration, aIso obvious use of the accessory muscles of respiration. There was duIIness to percussion and absence of breath sounds beIow the level of the second rib anteriorIy on the right side and in the upper twolthirds of the left chest. The liver was not palpable but the spleen extended 4 cm. beIon the Ieft costal margin. The weight was six pounds and fifteen ounces, the temperature p’.2’~., the urine negative, the bIood hemogIobin 12.7 Gm. and white blood ceIIs 24,800 with 35 per cent segmented, 20 per cent staff forms, 5 per cent monocytes, 2 per cent eosinophils and 18 per cent lymphocytes. An x-ray fiIm of the chest and abdomen (Fig. I) showed the right diaphragm elevated to the Ievel of the fourth rib posteriorly. There
98
AmericanJournal of Surgery
Bisgard,
Robertson-Eventration
was increased density of the right lung, probably from compression and an enlarged heart shadow which was displaced to the left to the
FIG. 5. This roentgenogram made five months folIowing operation shows the right diaphragm at the Ievel of the ninth rib, one interspace higher than the Ieft, presumabIy indicating that some giving way or relaxation has taken place. The writers are unable to explain the wedge-shaped shadow extending out from the right hiIus and the broadened area of supracardiac density; possibly thymus. Note that the eighth and ninth ribs have become broader and fused as a resuIt of the operative trauma.
extent that it was in contact with the left costal border. Evidence of aeration of the left lung was apparent at the inferior angle only. The liver shadow was displaced upward with its lower border above the costal margin. A diagnosis of either eventration or hernia of the right diaphragm was made and surgical exploration undertaken July 29, 1944. Oxygen was administered with positive pressure through a mask in a closed system and the skin and underlying tissues of the right ninth intercosta1 space infiltrated with 0.5 per cent novocaine solution. Through this interspace the pleural cavity was opened and adequate exposure obtained with a small rib spreader. The right diaphragm was intact throughout; that is, there was no break in its continuity. Its apex extended to the second rib anteriorly. Its excursion, though almost negligible, was not paradoxical. Except for pallor it appeared to be fairly normal and obviously it consisted of
OcroaER,1945
muscle. On casual observation there was discerned no abnormahty of the right lung. The diaphragm was shortened and brought down to the level of the tenth rib by plication with three superimposed rows of interrupted mattress sutures of silk extending diagonally from the costal attachment in the region of the nipple line posteromesially to the aperature for the vena cava as illustrated in Figure 2. The pleat was turned downward between the diaphragm and liver. The chest wall was cIosed with pericosta1 sutures and silk was used in the muscles and skin. There was complete relief of dyspnea and cyanosis immediately foIlowing operation and from then until the time of this report (seven months after operation) respirations have continued to be normal. Also in every other respect the infant appears to be normal and is growing and gaining weight at a normal rate. Roentgenograms of the chest immediately following operation and at periods subsequently are illustrated in Figures 3, 4 and 3. It will be observed that with the return of the diaphragm to its normal position the intrathoracic organs have Iikewise resumed their normal positions and both lungs are completely aerated. In the last roentgenogram the right diaphragm is one interspace higher than the left. COMMENT
It is probabIe that the writers’ case was more amenable to surgical therapy than most cases because the diaphragm contained muscIe and had considerabIe substance in which the sutures were weI1 retained against tension. Much Iess might be expected of cases in which the diaphragm is a very thin fibrous membrane as has been revealed in a few instances at postmortem examination. CONCLUSIONS I. A successfu1 operation and an apparent cure of eventration of the right diaphragm in an infant is reported. 2. As judged from reported cases, it is a reIativeIy rare condition in infancy. The writers beIieve that some infant deaths resuIt from unrecognized eventration of the diaphragm.
New
SERIES VOL.LXX, No.
Bisgard,
I
Robertson-Eventration
327, 1913.
3. CHAVDHURI, K. C. and REAI)>-, D. V. X. Indian J. Pediat., 7: I, 1940. 4. CLARKE, T. W., POWERS, hl. T. and WILSON, J. R. Arch. Pediat., 52: 798, 1935. f. 5. COSACK, G. and WEIN.RECI<, J. Monatscbr. Kinderb., 70: 161, 1937. 6. FELDMAN. L.. TRACE. I. nl. and KAPLAN. ,Il. I. Ann. Int. Med., 9: 62, 1935. 16: 193, 1913. 7. HAASE, H. Wien. 8. JAUBERT DE BEAUJERI A. J. de radial. et d. elecml., 21: 123, 1937.
thin, be of blush hue. They they become
tender
29, 1784.
presents increase
Company).
or when the abdominal
in size at the menstrual
They
with periodic external bIeeding. From “Textbook of Gynecology” Wilkins
in the form of small nodules
the surface
and painful.
99
17. SAKUL.~, J. Proc. Roy. Sot. Med., 33: 629, 1940. 18. SANTE, L. R. Am. J. Roentgenol., 44: 350, 1940. 19. WHITMORE, 0. S. and KABLSTROM, S. C. R;ew York Slate J. Med., 42: 1587, 1942. 20. YAKHCINA,R. S. Klin. Med., 13: 1724, ,935.
,
UMBILICAL endometriosis
of Surgery
KOESTER, F. Rhtgenpraxis, 7: 31 I, 1935. KORNS, H. bl. Arch. Int. Med., 28: 192, 1921. LANDOIV,J. F. J. Pediat., 8: 593, 1936. LANDON, J. F. Am. J. Dis. Child., 50: 284, 1935. LERCHE, \?I.Surg., Gynec. 0 Obst., 34: 244, 1922. 14. MECKEL. Cited by Zwauzinger, H. Inaug. Dissert., HalIe, 1819. 15. NICHOLAS, F. G. and NUSSBRECHER,A. hl. Lancet, 2: 61 I, 1935. 16. PYL, J. T. Aufsarse u. Beobacbt. a. d. per. Med., 5:
I. BAYNE-JONES, S. Arch. Inc. Med., 17: 221, 1916. 2. BERGMAX, J. Ergebu. d. inn. Med. u. Kinderb., 12:
may, when they approach
Journal
9. IO. I I. 12. 13.
REFERENCES
I
American
sometimes by Emil
break Novak
which
wall is verl periods,
through (The
when
the skin,
Williams
&
EVENTRATION SURGICAL
J.
DEWEY
BISGARD,
MANAGEMENT
M.D. AND GEORGE E. ROBERTSON, M.D. OMAHA,
A
OF THE DIAPHRAGM
NEBRASKA
of eventration of the diaphragm in an infant which was cured b,y operation is reported
Eventration of the diaphragm may be defined as an abnormaIIy high or elevated position of one leaf of the diaphragm as a
CASE
FIG. I. Preoperative film. The right diaphragm is elevated to the Ievel of the posterior portion of the fifth rib and the heart and mediastinum are displaced to the left. The area of cardiac dullness extends to the Ieft costal margin with only a smaI1 segment of unobscured lung in the left costalphrenic sinus. The inferior margin of the liver is ahove the eleventh rib.
because, (I) the writers are unable to find a report of a previous attempt to treat this condition in an infant by operation and (2) they suspect that some of the deaths in the newborns which are attributed to other causes of cyanosis and dyspnea are in reaIity the resuIt of unrecognized congenital eventration, which, as in our case, have prospect of surgica1 cure.
aplasia or atrophy of result of paralysis, the muscle fibers but with no break in the continuity of the Ieaf. Its unbroken continuity differentiates it from diaphragmatic hernia. Admitted by a11 authors an inappropriate term, eventration has been estabIished by general use and the Iack of a better name. The condition has been observed in peopIe of all ages and, although 95
96
American
Journal
ofSurgery Bisgard,
Robertson-Eventration
this paper is concerned with the probIem in infants, it is necessary to refer to subject matter which deals with the subject as a whole.
FIG. 2. Diagrammatic representation of the position of the diaphragm (dotted tine) and the resultant distortion of the intrathoracic viscera. This is afso illustrated in the procedure used in the writer’s case. Shortening was accomplished by pIication with three superimposed rows of interrupted mattress sutures through an intrathoracic approach.
Bibliographers have credited Petit with the first description “of the condition. A case report with postmortem findings appeared in the posthumous edition of his work published in 1774. Infrequently thereafter, isoIated cases were reported and in 1913 Bergman reviewed the Iiterature to that date and collected thirty-one cases. Three years later Bayne-Jones reported a coIIection of forty-five cases including one of his own. In 1921, Korns was abIe to assembIe sixty-five cases and in the most recent coIIection Reed and Borden, in 1935, reported 183 cases. Most of the reported cases were aduIts and it is probable that in most of them the lesion was acquired. A few were incidenta findings and presumably caused no symptoms. The remaining cases presented a variety of symptoms which fall into four general groups: (I) gastric, (2) cardiac, (3) pmmonary and (4) pleuropulmonary. If the number of case reports is a criterion, eventration in infants is indeed a rarity. In his review of the Iiterature up to rgr6 Bayne-Jones collected three cases:
OCTOB&R, 1949
a fetus, a newborn, one day old, and an infant of three months. In each instance the diagnosis was made at postmortem examination. In the eighty-three cases collected from the Iiterature from 192 I to 1933 by Reed and Borden there were only five infants. From 1935 to 1945, at Ieast nine additional cases in infants have been reported in&ding our case, Undoubtediy these figures give a very inaccurate conception of the incidence. In addition to the unreported cases it is very probable that some of the deaths in infants which have been attributed to other causes of cyanosis and dyspnea have in reality resuhed from eventration. The writers’ case was unusua1 in that the right haIf of the diaphragm was involved. Of the 183 cases coIIected by Reed and Borden only eighteen were on the right side. The symptoms in the infants, when recorded, were cyanosis and dyspnea as in our ‘case. At postmortem examination the elevated haIf of the diaphragm has presented varying degrees of atrophy; in the extreme a very thin fibrous membrane was present with compIete absence of muscIe fibers. Not infrequently there are associated maldeveIopments of the Iung, heart or fiver, faiIure of intestina1 rotation or unassociated congenital deformities as cIeft paIate. In the series of 183 cases reviewed by Reed and Borden direct operative attack upon the diaphragm was done in onIy four cases. Reports of two additional cases were found in the literature which has accumulated since 1935. In aI1 cases the diaphragm was Iowered by shortening, in one case by excision of a segment and in five by plication. One patient was plicated from the abdominaI side, the others from above through an intrathoracic exposure. One died three days after operation and the others obtained partial or compIete reIief of symptoms. The ages of these patients varied from ten to forty-four years. So far as we were able to ascertain from a carefui review of the available literature,
NEW
SERIES VOL. LXX, No. I
Bisgard,
our case is not only the first instance of a surgica1 cure of this in an infant but aIso the only
Robertson-Eventration recorded condition record of
FIG. 3. This roentgenogram made immediately foIIowing operation shows the right diaphragm Iowered to a position below the left with comptetr aeration of both lungs and normal intrathoracic reIations. The inferior border of the Iiver is severa centimeters beIow the twelfth rib.
operative group.
intervention
CASE
in a case in this
age
REPORT
Baby W., a male infant six weeks oId, was brought to the Bishop Clarkson Memorial HospitaI in an improvised oxygen chamber. He was born at fulI term without operative assistance and appeared normal at birth. On about the tenth day after birth the mother became was somewhat impressed that respiration labored and that cyanosis developed when the infant cried. Feeding became progressively more diff%uIt and he faiIed to gain weight. By the fifth week after birth the dyspnea and cyanosis had increased to such an alarming degree that it was necessary to administer oxygen. The father, who is an automobiIe mechanic in a rura1 community, fashioned a very satisfactory oxygen tent with the help of the family physician. AIthough the tent made
American
Journal
of Surger
97
surviva1 possible there was no improven lent in the infant’s condition after one week so he was brought to the hospital.
FIG. 4. At the time of dismissal
from the hospita1, ten days after operation, the Iowered position of the right diaphragm was still we11 retained.
Examination showed a well developed but poorly nourished infant of six weeks showing moderate dyspnea and rather marked cyanosis both of which became very marked with crying and exertion. The right side of the chest moved less than the Ieft and there was Iateral retraction with inspiration, aIso obvious use of the accessory muscles of respiration. There was duIIness to percussion and absence of breath sounds beIow the level of the second rib anteriorIy on the right side and in the upper twolthirds of the left chest. The liver was not palpable but the spleen extended 4 cm. beIon the Ieft costal margin. The weight was six pounds and fifteen ounces, the temperature p’.2’~., the urine negative, the bIood hemogIobin 12.7 Gm. and white blood ceIIs 24,800 with 35 per cent segmented, 20 per cent staff forms, 5 per cent monocytes, 2 per cent eosinophils and 18 per cent lymphocytes. An x-ray fiIm of the chest and abdomen (Fig. I) showed the right diaphragm elevated to the Ievel of the fourth rib posteriorly. There
98
AmericanJournal of Surgery
Bisgard,
Robertson-Eventration
was increased density of the right lung, probably from compression and an enlarged heart shadow which was displaced to the left to the
FIG. 5. This roentgenogram made five months folIowing operation shows the right diaphragm at the Ievel of the ninth rib, one interspace higher than the Ieft, presumabIy indicating that some giving way or relaxation has taken place. The writers are unable to explain the wedge-shaped shadow extending out from the right hiIus and the broadened area of supracardiac density; possibly thymus. Note that the eighth and ninth ribs have become broader and fused as a resuIt of the operative trauma.
extent that it was in contact with the left costal border. Evidence of aeration of the left lung was apparent at the inferior angle only. The liver shadow was displaced upward with its lower border above the costal margin. A diagnosis of either eventration or hernia of the right diaphragm was made and surgical exploration undertaken July 29, 1944. Oxygen was administered with positive pressure through a mask in a closed system and the skin and underlying tissues of the right ninth intercosta1 space infiltrated with 0.5 per cent novocaine solution. Through this interspace the pleural cavity was opened and adequate exposure obtained with a small rib spreader. The right diaphragm was intact throughout; that is, there was no break in its continuity. Its apex extended to the second rib anteriorly. Its excursion, though almost negligible, was not paradoxical. Except for pallor it appeared to be fairly normal and obviously it consisted of
OcroaER,1945
muscle. On casual observation there was discerned no abnormahty of the right lung. The diaphragm was shortened and brought down to the level of the tenth rib by plication with three superimposed rows of interrupted mattress sutures of silk extending diagonally from the costal attachment in the region of the nipple line posteromesially to the aperature for the vena cava as illustrated in Figure 2. The pleat was turned downward between the diaphragm and liver. The chest wall was cIosed with pericosta1 sutures and silk was used in the muscles and skin. There was complete relief of dyspnea and cyanosis immediately foIlowing operation and from then until the time of this report (seven months after operation) respirations have continued to be normal. Also in every other respect the infant appears to be normal and is growing and gaining weight at a normal rate. Roentgenograms of the chest immediately following operation and at periods subsequently are illustrated in Figures 3, 4 and 3. It will be observed that with the return of the diaphragm to its normal position the intrathoracic organs have Iikewise resumed their normal positions and both lungs are completely aerated. In the last roentgenogram the right diaphragm is one interspace higher than the left. COMMENT
It is probabIe that the writers’ case was more amenable to surgical therapy than most cases because the diaphragm contained muscIe and had considerabIe substance in which the sutures were weI1 retained against tension. Much Iess might be expected of cases in which the diaphragm is a very thin fibrous membrane as has been revealed in a few instances at postmortem examination. CONCLUSIONS I. A successfu1 operation and an apparent cure of eventration of the right diaphragm in an infant is reported. 2. As judged from reported cases, it is a reIativeIy rare condition in infancy. The writers beIieve that some infant deaths resuIt from unrecognized eventration of the diaphragm.
New
SERIES VOL.LXX, No.
Bisgard,
I
Robertson-Eventration
327, 1913.
3. CHAVDHURI, K. C. and REAI)>-, D. V. X. Indian J. Pediat., 7: I, 1940. 4. CLARKE, T. W., POWERS, hl. T. and WILSON, J. R. Arch. Pediat., 52: 798, 1935. f. 5. COSACK, G. and WEIN.RECI<, J. Monatscbr. Kinderb., 70: 161, 1937. 6. FELDMAN. L.. TRACE. I. nl. and KAPLAN. ,Il. I. Ann. Int. Med., 9: 62, 1935. 16: 193, 1913. 7. HAASE, H. Wien. 8. JAUBERT DE BEAUJERI A. J. de radial. et d. elecml., 21: 123, 1937.
thin, be of blush hue. They they become
tender
29, 1784.
presents increase
Company).
or when the abdominal
in size at the menstrual
They
with periodic external bIeeding. From “Textbook of Gynecology” Wilkins
in the form of small nodules
the surface
and painful.
99
17. SAKUL.~, J. Proc. Roy. Sot. Med., 33: 629, 1940. 18. SANTE, L. R. Am. J. Roentgenol., 44: 350, 1940. 19. WHITMORE, 0. S. and KABLSTROM, S. C. R;ew York Slate J. Med., 42: 1587, 1942. 20. YAKHCINA,R. S. Klin. Med., 13: 1724, ,935.
,
UMBILICAL endometriosis
of Surgery
KOESTER, F. Rhtgenpraxis, 7: 31 I, 1935. KORNS, H. bl. Arch. Int. Med., 28: 192, 1921. LANDOIV,J. F. J. Pediat., 8: 593, 1936. LANDON, J. F. Am. J. Dis. Child., 50: 284, 1935. LERCHE, \?I.Surg., Gynec. 0 Obst., 34: 244, 1922. 14. MECKEL. Cited by Zwauzinger, H. Inaug. Dissert., HalIe, 1819. 15. NICHOLAS, F. G. and NUSSBRECHER,A. hl. Lancet, 2: 61 I, 1935. 16. PYL, J. T. Aufsarse u. Beobacbt. a. d. per. Med., 5:
I. BAYNE-JONES, S. Arch. Inc. Med., 17: 221, 1916. 2. BERGMAX, J. Ergebu. d. inn. Med. u. Kinderb., 12:
may, when they approach
Journal
9. IO. I I. 12. 13.
REFERENCES
I
American
sometimes by Emil
break Novak
which
wall is verl periods,
through (The
when
the skin,
Williams
&