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Congenital atresia of the ileum, spontaneous perforation and multiple intussusception
CONGENITAL ATRESIA OF THE ILEUM, SPONTANEOUS PERFORATION AND MULTIPLE INTUSSUSCEPTION* TWO CASE REPORTS BERNARD
J.
FICARRA,
Resident Surgeon, Kings County
M.D.
AND
BROOKLYN,
A
surgery of infancy and chiIdhood has received accentuated attention from the genera1 surgeon in recent years. Some surgeons have stressed this branch of surgery as a distinct speciaIty. It cannot be denied that extreme deIicacy is necessary in pediatric surgery, even as the cases encountered present most interesting features. In the course of our residency in pediatric surgery, we have seen two unusua1 cases which we are presenting herewith. BDOMINAL
CASE
WILLIAM
Hospital
B.
DEGEN,
M.D.
Resident Surgeon, Kings County HospitaI NEW
YORK
visuaIized by x-ray. This mass measured 355 by 234 inches and was found in the right mid-abdomen. At this point the iIeum was
REPORTS
CASE I. The patient was a premature infant (eight months) born to a primiparous mother. The mother had hypertension and poIyhydramnios. At the time of birth the child cried IoudIy and breathed spontaneousty. The birth weight was 4 pounds, 4 ounces. The general appearance of the baby apparently was satisfactory. On the first postpartum day marked abdomina1 distention occurred. A Levine tube attached to a Wangensteen suction apparatus plus a rectal tube failed to reIieve the distention. At this time the temperature was I02OF. and the infant regurgitated yellow green fluid. During the twenty-four hours foIlowing birth no stoo1 was wssed. Enemas gave no resuIts. An emergency ffat pIate of the abdomen was taken at this time. (Fig. I.) Surgical consultation was requested. The infant was subjected to surgery thirty-six hours foIIowing birth. The abdomina1 cavity was entered via a right paramedian incision. When the peritoneal cavity was entered many organized adhesions were found between the parietal and visceral layers. The entire small intestine was found to be matted together by an organized fibrinous pIastic exudate. The iIeum was bound down forming the mass
FIG. I. Flat plate of abdomen demonstrating tremendous distention of the smaI1 intestine in right half of abdomen. Distended loops are also visibIe in left haIf indicating an adynamic iIeus compatible with peritonitis. A catheter is noted in the stomach, another is seen in the rectum (Case 1).
perforated for its entire diameter. Meconium fluid seeped through this opening. The intestina perforation occurred five inches from the ileoceca1 vaIve. Three and a half inches from the iIeoceca1 junction was found it non-perforated membrane completely occIuding the Iumen of the iIeum. The small intestine was distended to the area of the mass and the per-
* From the Surgical Services of Dr. NichoIas II. Ryan and Dr. Ottokar Tenopyr, 123
Kings County HospitaI, BrookIyn.
124
American Journal of Surgery
Ficarra,
Degen-Atresia
foral Zion. The Iarge intestine was colIapsed throl ughout its entire length. The matted ileum, in& [ding the membranous area was resected.
FIG. 2. Flat pIate x-ray of abdomen showing a mass in right upper half of the abdomen. Intussusception suggested in transverse colon (Case II).
A
side-to-side iIeocoIostomy was then performed. Routine cIosure of the abdomen was accomplished. The condition of the infant on Ieaving the operating room was very poor. Ten hours after the operation the infant died. Autopsy study attributed the death to meconium peritonitis. Comment. CongenitaI atresia of the iIeum is not seen frequentIy. Without surgica1 intervention death from dehydration and inanition is inevitabIe. Atresia of the smaI1 intestine occurs in two forms. One type is an interna membrane occIuding the Iumen. The second type is a sudden ending of the intestina1 tract as a bIind sac.’ Ladd and Gross report forty-one cases in which the patients were operated upon for congenita1 atresia of the ileum with onIy five patients surviving. Hence it can
of IIeum
JULY,
be appreciated how uncommon is t;his entity and how high is the operative m(ortaIity. It is rare to find this condit ion
FIG. 3. Preoperative barium enema revealing an obstructive process due to an intussusception in the ascending colon. Barium has pushed the intussusception from the transverse to the ascending coton (Case II).
associated with a perforation proxima1 to the atresia as recorded in this case. In the discussion of this subject Ladd and Gross mention Farber’s test as an aid in diagnosis. This is a microscopic test whereby the meconium of an infant can be quickIy examined and the presence or absence of swaIIowed vernix ceIIs can be determined. This is performed by staining a fresh specimen of meconium on a sIide with gentian vioIet and then examining the specimen. under a microscope. In any newIy born chiId with intestinal obstruction, the absence of cornified epitheIia1 ceIIs in the meconium is presumptive evidence that an intestina1 atresia exists.’ CASE II. This is the case history of a twenty-three month old coIored boy who was admitted to the pediatric medical service on
NEW LXV. VOL. SERIES
No. I
Ficarra,
Degen-Atresia
June 23, 1943.The history was one of constipation of five days’ duration. Small fecal particles were passed by rectum on aIternating days during this tive-day period. It was noted that the child wouId hold his abdomen with his hands and flex his Iegs on the abdomen. He would do this innumerabIe times. Associated with this procedure was the facia1 expression of pain. Vomiting occurred twice during the physica examination. OIiguria was present since the onset of symptoms. No bIood was passed by rectum; none was found on the examiner’s finger. On admission the temperature, pulse and respirations were within norma limits. The abdomina1 findings at this time were nonrevealing. The abdomen was soft, no tenderness could be eIicited, no masses were palpable. A smaI1 umbiIica1 hernia was present. Laboratory and bIood studies were normaI. On June 2&h, five days after admission, a surgica1 consuhation was requested. The patient was accepted on the surgica1 service with the tentative diagnosis of intestinal obstruction possibly due to an intussusception. X-ray studies were requested. (Figs. 2 and 3.) At this time the patient had severa Ioose, brown, watery stooIs. He retained ffuids by mouth but no soIid food. The next day the abdomen became moderateIy rigid on the right side; tenderness couId be eIicited in the right lower quadrant. A questionable mass couId be feIt in the right lower quadrant. There was no blood per rectum and no mass couId be felt by digita rectal examination. AI1 this time the temperature and pulse rate did not rise. Surgery was advised after intravenous Auids and a blood transfusion had been given. The patient underwent an expIoratory Iaparotomy on the seventh day of admission to the hospital. The abdomen was opened through a right A moderate amount of paramedian incision. cIear, yeIIow Auid was aspirated from the peritonea1 cavity. A doubIe intussusception was found in the right coIon extending up to the hepatic flexure. The termina1 iIeum was invaginated into the cecum. The cecum in turn was invaginated into the ascending colon up to the hepatic flexure. The appendix was drawn into the ascending coIon by the cecum. The appendix was reddened, edematous, congested and macroscopicaIIy enlarged. The lateral Iigamentous foId of the ascending colon was absent. The right half of the coIon was ex-
of IIeum
AmericanJournal of Surgery
I25
tremely mobile. The intussusception was reduced in the orthodox fashion. The intestine was viable. A smaI1 amount of hemorrhage in
4. Postoperative bar&n examinaaion revealing no obstruction to the passage of the opaque material through the entire c&n.
FIG.
The intussusception formerly pletely reduced (Case II).
present is com-
the serosa of the cecum was present. SIight to moderate edema of the cecum was noted. After the reduction of the intussusception an appendectomy was performed. The terminal portion of the iIeum and the cecum were sutured down to the posterior parieta1 peritoneum. AbdominaI cIosure was accompIished in the routine fashion. PostoperativeIy the patient was given intravenous fluids. On the second postoperative day the temperature rose to 102'~. on one occasion. AbdominaI distention on the third and fourth days was combatted with WangenSteen suction. No other compIication deveIoped. Four weeks after the operation a barium study of the coIon showed compIete reduction of the intussusception. (Fig. 4.) Intussusception is a surgica1 Comment. disease infrequentIy seen., Over a thirtyyear period Ladd and Gross reported 484 cases. Of this number onIy I per cent showed muItipIe intussusception. Thus the rarity of this state is readiIy appreciated.
I 26
American
Journal of Surgery
Ficarra,
Degen-Atresia
Intussusception in chiIdhood may find an etioIogica1 basis in a MeckeI’s diverticuIum, intestina1 polyp, Iymphoma or dupIication. In most instances no etioIogica1 agent can be found. ’ In the case reported, it is beIieved that the free mobiIity of the ascending coIon, due to the absence of a IateraI attachment, was a contributing factor. Intussusceptions are spoken of in compound terms which indicate the portion of the intestine (intussusceptum) which teIescopes into the intussuscipiens. l MuItipie types are reported. These are usuaIIy iieo-iIeo-colic.’ Ladd and Gross do not mention an iIeo-cecaI-cecaI-coIic type as found in this case. This we believe to be a rare condition. The other unusual features of this case were the deIayed physica findings, and the absence of bIoody stoo1.s with a compIex pathoIogica1 process. Another interesting aspect was the Iong interva1 between the onset of symptoms and the time of operation. Prolonged deIay such as this aImost always terminates fataIIy. The explanation
of IIeum
JULY, 1944
offered for the bizarre cIinica1 picture is that the intussusception reduced itseIf 0nIy to recur. SUMMARY
Tvo unusua1 pediatric cases are presented. The first is a congenita1 atresia of the iIeum associated with spontaneous intestinal perforation in a premature infant. This infant developed signs of an intestinal obstruction within twenty-four hours after birth. SurgicaI intervention faiIed in the presence of an extensive peritonitis. The second case is a doubIe intussusception in a twenty-three months old boy who presented a &e-day history prior to entering the hospita1. An additiona deIay of seven days occurred before surgica1 reIief of the intussusception was accomplished. Notwithstanding this proIonged delay, the patient had an uneventfu1 recovery. REFERENCE
WIL.LIAM and GROSS, ROBERT. AbdominaI Surgery of Infancy and Childhood. PhiIadeIphia, J941. W. B. Saunders Co.
I. LADD,
J.
FICARRA,
Resident Surgeon, Kings County
M.D.
AND
BROOKLYN,
A
surgery of infancy and chiIdhood has received accentuated attention from the genera1 surgeon in recent years. Some surgeons have stressed this branch of surgery as a distinct speciaIty. It cannot be denied that extreme deIicacy is necessary in pediatric surgery, even as the cases encountered present most interesting features. In the course of our residency in pediatric surgery, we have seen two unusua1 cases which we are presenting herewith. BDOMINAL
CASE
WILLIAM
Hospital
B.
DEGEN,
M.D.
Resident Surgeon, Kings County HospitaI NEW
YORK
visuaIized by x-ray. This mass measured 355 by 234 inches and was found in the right mid-abdomen. At this point the iIeum was
REPORTS
CASE I. The patient was a premature infant (eight months) born to a primiparous mother. The mother had hypertension and poIyhydramnios. At the time of birth the child cried IoudIy and breathed spontaneousty. The birth weight was 4 pounds, 4 ounces. The general appearance of the baby apparently was satisfactory. On the first postpartum day marked abdomina1 distention occurred. A Levine tube attached to a Wangensteen suction apparatus plus a rectal tube failed to reIieve the distention. At this time the temperature was I02OF. and the infant regurgitated yellow green fluid. During the twenty-four hours foIlowing birth no stoo1 was wssed. Enemas gave no resuIts. An emergency ffat pIate of the abdomen was taken at this time. (Fig. I.) Surgical consultation was requested. The infant was subjected to surgery thirty-six hours foIIowing birth. The abdomina1 cavity was entered via a right paramedian incision. When the peritoneal cavity was entered many organized adhesions were found between the parietal and visceral layers. The entire small intestine was found to be matted together by an organized fibrinous pIastic exudate. The iIeum was bound down forming the mass
FIG. I. Flat plate of abdomen demonstrating tremendous distention of the smaI1 intestine in right half of abdomen. Distended loops are also visibIe in left haIf indicating an adynamic iIeus compatible with peritonitis. A catheter is noted in the stomach, another is seen in the rectum (Case 1).
perforated for its entire diameter. Meconium fluid seeped through this opening. The intestina perforation occurred five inches from the ileoceca1 vaIve. Three and a half inches from the iIeoceca1 junction was found it non-perforated membrane completely occIuding the Iumen of the iIeum. The small intestine was distended to the area of the mass and the per-
* From the Surgical Services of Dr. NichoIas II. Ryan and Dr. Ottokar Tenopyr, 123
Kings County HospitaI, BrookIyn.
124
American Journal of Surgery
Ficarra,
Degen-Atresia
foral Zion. The Iarge intestine was colIapsed throl ughout its entire length. The matted ileum, in& [ding the membranous area was resected.
FIG. 2. Flat pIate x-ray of abdomen showing a mass in right upper half of the abdomen. Intussusception suggested in transverse colon (Case II).
A
side-to-side iIeocoIostomy was then performed. Routine cIosure of the abdomen was accomplished. The condition of the infant on Ieaving the operating room was very poor. Ten hours after the operation the infant died. Autopsy study attributed the death to meconium peritonitis. Comment. CongenitaI atresia of the iIeum is not seen frequentIy. Without surgica1 intervention death from dehydration and inanition is inevitabIe. Atresia of the smaI1 intestine occurs in two forms. One type is an interna membrane occIuding the Iumen. The second type is a sudden ending of the intestina1 tract as a bIind sac.’ Ladd and Gross report forty-one cases in which the patients were operated upon for congenita1 atresia of the ileum with onIy five patients surviving. Hence it can
of IIeum
JULY,
be appreciated how uncommon is t;his entity and how high is the operative m(ortaIity. It is rare to find this condit ion
FIG. 3. Preoperative barium enema revealing an obstructive process due to an intussusception in the ascending colon. Barium has pushed the intussusception from the transverse to the ascending coton (Case II).
associated with a perforation proxima1 to the atresia as recorded in this case. In the discussion of this subject Ladd and Gross mention Farber’s test as an aid in diagnosis. This is a microscopic test whereby the meconium of an infant can be quickIy examined and the presence or absence of swaIIowed vernix ceIIs can be determined. This is performed by staining a fresh specimen of meconium on a sIide with gentian vioIet and then examining the specimen. under a microscope. In any newIy born chiId with intestinal obstruction, the absence of cornified epitheIia1 ceIIs in the meconium is presumptive evidence that an intestina1 atresia exists.’ CASE II. This is the case history of a twenty-three month old coIored boy who was admitted to the pediatric medical service on
NEW LXV. VOL. SERIES
No. I
Ficarra,
Degen-Atresia
June 23, 1943.The history was one of constipation of five days’ duration. Small fecal particles were passed by rectum on aIternating days during this tive-day period. It was noted that the child wouId hold his abdomen with his hands and flex his Iegs on the abdomen. He would do this innumerabIe times. Associated with this procedure was the facia1 expression of pain. Vomiting occurred twice during the physica examination. OIiguria was present since the onset of symptoms. No bIood was passed by rectum; none was found on the examiner’s finger. On admission the temperature, pulse and respirations were within norma limits. The abdomina1 findings at this time were nonrevealing. The abdomen was soft, no tenderness could be eIicited, no masses were palpable. A smaI1 umbiIica1 hernia was present. Laboratory and bIood studies were normaI. On June 2&h, five days after admission, a surgica1 consuhation was requested. The patient was accepted on the surgica1 service with the tentative diagnosis of intestinal obstruction possibly due to an intussusception. X-ray studies were requested. (Figs. 2 and 3.) At this time the patient had severa Ioose, brown, watery stooIs. He retained ffuids by mouth but no soIid food. The next day the abdomen became moderateIy rigid on the right side; tenderness couId be eIicited in the right lower quadrant. A questionable mass couId be feIt in the right lower quadrant. There was no blood per rectum and no mass couId be felt by digita rectal examination. AI1 this time the temperature and pulse rate did not rise. Surgery was advised after intravenous Auids and a blood transfusion had been given. The patient underwent an expIoratory Iaparotomy on the seventh day of admission to the hospital. The abdomen was opened through a right A moderate amount of paramedian incision. cIear, yeIIow Auid was aspirated from the peritonea1 cavity. A doubIe intussusception was found in the right coIon extending up to the hepatic flexure. The termina1 iIeum was invaginated into the cecum. The cecum in turn was invaginated into the ascending colon up to the hepatic flexure. The appendix was drawn into the ascending coIon by the cecum. The appendix was reddened, edematous, congested and macroscopicaIIy enlarged. The lateral Iigamentous foId of the ascending colon was absent. The right half of the coIon was ex-
of IIeum
AmericanJournal of Surgery
I25
tremely mobile. The intussusception was reduced in the orthodox fashion. The intestine was viable. A smaI1 amount of hemorrhage in
4. Postoperative bar&n examinaaion revealing no obstruction to the passage of the opaque material through the entire c&n.
FIG.
The intussusception formerly pletely reduced (Case II).
present is com-
the serosa of the cecum was present. SIight to moderate edema of the cecum was noted. After the reduction of the intussusception an appendectomy was performed. The terminal portion of the iIeum and the cecum were sutured down to the posterior parieta1 peritoneum. AbdominaI cIosure was accompIished in the routine fashion. PostoperativeIy the patient was given intravenous fluids. On the second postoperative day the temperature rose to 102'~. on one occasion. AbdominaI distention on the third and fourth days was combatted with WangenSteen suction. No other compIication deveIoped. Four weeks after the operation a barium study of the coIon showed compIete reduction of the intussusception. (Fig. 4.) Intussusception is a surgica1 Comment. disease infrequentIy seen., Over a thirtyyear period Ladd and Gross reported 484 cases. Of this number onIy I per cent showed muItipIe intussusception. Thus the rarity of this state is readiIy appreciated.
I 26
American
Journal of Surgery
Ficarra,
Degen-Atresia
Intussusception in chiIdhood may find an etioIogica1 basis in a MeckeI’s diverticuIum, intestina1 polyp, Iymphoma or dupIication. In most instances no etioIogica1 agent can be found. ’ In the case reported, it is beIieved that the free mobiIity of the ascending coIon, due to the absence of a IateraI attachment, was a contributing factor. Intussusceptions are spoken of in compound terms which indicate the portion of the intestine (intussusceptum) which teIescopes into the intussuscipiens. l MuItipie types are reported. These are usuaIIy iieo-iIeo-colic.’ Ladd and Gross do not mention an iIeo-cecaI-cecaI-coIic type as found in this case. This we believe to be a rare condition. The other unusual features of this case were the deIayed physica findings, and the absence of bIoody stoo1.s with a compIex pathoIogica1 process. Another interesting aspect was the Iong interva1 between the onset of symptoms and the time of operation. Prolonged deIay such as this aImost always terminates fataIIy. The explanation
of IIeum
JULY, 1944
offered for the bizarre cIinica1 picture is that the intussusception reduced itseIf 0nIy to recur. SUMMARY
Tvo unusua1 pediatric cases are presented. The first is a congenita1 atresia of the iIeum associated with spontaneous intestinal perforation in a premature infant. This infant developed signs of an intestinal obstruction within twenty-four hours after birth. SurgicaI intervention faiIed in the presence of an extensive peritonitis. The second case is a doubIe intussusception in a twenty-three months old boy who presented a &e-day history prior to entering the hospita1. An additiona deIay of seven days occurred before surgica1 reIief of the intussusception was accomplished. Notwithstanding this proIonged delay, the patient had an uneventfu1 recovery. REFERENCE
WIL.LIAM and GROSS, ROBERT. AbdominaI Surgery of Infancy and Childhood. PhiIadeIphia, J941. W. B. Saunders Co.
I. LADD,