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Pneumatosis Intestinalis in the Infant Ademakinwa M. Adekunle, MD, Albert Z. Holloway, MD, Trevor Golding, MD, and Harry C. Press, Jr., MD Washington, D.C.
Clinical Background This male infant was delivered by a 26-year-old mother with two previous abortions. The gestational age was 30 weeks and the infant weighed 1,020 gm at birth. His Apgar score was 8 at one minute and 9 in five minutes. Because of his prematurity, grunting respiration, and intercostal retraction, he was admitted into the Nursery Intensive Care Unit. Respiratory supportive measures were instituted and an umbilical arterial catheter, the tip of which was at T 10,
From the Departments of Radiology and Pediatrics, Howard University Hospital, Washington, D.C. Requests for reprints should be sent to Dr. Harry C. Press, Department of Radiology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, D.C. 20060.
was inserted. He was stabilized and did fairly well in the first 24 hours. Fortyeight hours after birth, he developed respiratory distress syndrome which was evidenced both clinically and radiographically. On the third day of life, he developed left pneumothorax for which a chest tube was inserted. He began to have apneic spells which increased in frequency. His abdomen was distended and tense and his stool was bloody. Because the umbilical arterial catheter was blocked, it was removed and replaced with an umbilical venous catheter. The accompanying radiograph was then obtained. Which one of the following is the most likely diagnosis? 1. Meconium peritonitis. 2. Meconium ileus. 3. Necrotizing enterocolitis. 4. Aganglionosis 5. Meconium plug syndrome.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 1, 1978
Radiographic Findings The chest demonstrates the fine reticulonodular pattern seen in respirat-
Figure 1. Three-day-old infant with apneic spells, abdominal distension, and bloody stools. 63
ory distress syndrome. The chest tube was inserted for pneumothorax. The endotracheal tube is fairly low within the trachea. An umbilical venous catheter is noted in situ. In the infant, it is usually difficult to differentiate the small bowel from colon gas. However, notice the distention of the ascending and proximal descending colon at the periphery of the abdominal cavity. The bubbly appearance represents a combination of air, fecal material, blood, and sloughed mucosa within the lumen of the colon. The colon is well outlined here because of intramural air in the wall.
caliber distal to the obstruction. Intramural gas is not a feature of this condition, but the bubbly appearance may be seen proximal to the obstruction. The meconium plug is relieved by enemas and the infants do not have this clinical presentation. Infants do not exhibit the "formed-stool appearance" seen in adults, because their stools are frequently liquid in consistency. Such a "bubbly" pattern, as seen in this case, is highly suggestive of necrotizing enterocolitis, which is the correct diagnosis in this case. There is no evidence of hepatic portal venous gas or pneumoperitoneum.
Differential Diagnosis
Discussion
Meconium peritonitis is almost always associated with proximal smallbowel obstruction. There is perforation of the bowel in utero proximal to the obstruction and there is spillage of meconium into the peritoneal cavity. An inflammatory reaction follows and there is subsequent calcification of the meconium. These flecks of calcification can usually be seen at birth. These are absent in this case. Meconium ileus is seen in infants with mucoviscidosis. The plain film of the abdomen usually shows several loops of dilated small bowel, which may contain air-fluid levels on erect or lateral decubitus roentgenograms. A "frothy" appearance may also be seen in meconium ileus as a result of admixture between the tanacious meconium and gas in the terminal ileum. Aganglionosis does not present this early in life. However, because necrotizing enterocolitis may sometimes complicate aganglionosis, it makes it necessary to evaluate all survivors of this disease for aganglionosis. The timing of the onset of clinical and radiographic features makes it unlikely to be secondary to an underlying aganglionosis. The meconium plug syndrome is seen in newborn infants and the colon is usually normal or near normal in
Necrotizing enterocolitis (NEC) was first described by Siebold in 1825. It is a very serious disease, with a mortality rate of well over 75 percent" 2 The patient who develops NEC is typically premature by weight and gestational age. Eighty percent weigh less than 2,000 gm and the gestational age is 34 weeks or less. Respiratory distress syndrome occurs in 75 percent of these patients.3 The average age of onset of NEC is 6.5 days. Nearly half of all cases occur between the 3rd and 4th days.4 The etiology of this disorder is not specifically known. It is seen most often in infants who have survived a period of a severe, shock-like state. Exchange transfusion, umbilical arterial or venous catheterization, various causes of perinatal hypoxia, or asphyxia have been cited as contributing factors in development of intestinal necrosis.' It may be idiopathic, associated with a mechanical functional obstruction or Hirschsprung's disease. Clinically an infant with NEC presents with abdominal distention, bile emesis, and hematochezia. Other clinical findings such as peritonitis with or without perforation, shock, or disseminated intravascular coagulopathy were present in patients having extensive involvement .4
A radiographic finding of pneumatosis intestinalis (PI) and adynamic ileus, although not specific for NEC, confirms the diagnosis if there is a high clinical index of suspicion. A patient may have NEC without pneumatosis intestinalis. These cases are confirmed at surgery or autopsy. There is no relationship of clinical severity or outcome either to pneumotosis intestinalis or adynamic ileus. There may be extensive disease found at surgery without severe radiologic findings or extensive pneumatosis intestinalis with a benign clinical course not requiring surgery.4 The development of PI had been explained on the basis of intraluminal gas entering bowel walls mechanically after the mucosa had been ulcerated. Other authors have shown that carbohydrate in the intestinal tract plus the presence of bacteria in the duodenum are required for PI to develop.5 Both substrate and bacteria are necessary for the production of hydrogen gas. Hydrogen is a major constituent of intramural gas found in NEC.4 A roentgenographic finding of PT occurs in 75 percent of patients.3 With progression of the disease perforation into the abdominal cavity is likely. In addition, gas may enter the portal system and be carried to the liver. The demonstration of gas within the hepatic portal venous system indicates a grave prognosis. Vollman et al reported a case of an infant who survived two separate episodes of PI and hepatic portal venous gas.6 Franz et al4 also reported the reappearance of signs of NEC in several of their patients in whom enteric feedings were restarted prior to 10 days of medical therapy.
Literature Cited 1. Cohn R, Sunshine R, deVries P: Necrotizing enterocolitis in the newborn infant. Am J Surg 124:165-168, 1972 2. Rabinowitz JG, Wolf BS, Feller MR, et al: Colonic changes following necrotizing enterocolitis in the newborn. Am J Roentgenology 103:539-634, 1968 3. Roback SA, Foker J, Frantz IF, et al: Necrotizing enterocolitis. Arch Surg 109:314-319, 1974 4. Frantz lil ID, L'Heureux P, Engel RR, et al: Necrotizing enterocolitis. J Pediatr 86(2):259263, 1975 5. Coello-Ramirez P, Gutierrex-Topete G, Lifshitz F: Pneumatosis intesinalis. Am J Dis Child 120:3-9, 1970 6. Vollman JH, Smith WL, Tsang RC: Necrotizing enterocolitis with recurrent hepatic portal venous gas. J Pediatr 88(3):486-487, 1976
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 1, 1978
a
rS,, g .~ tL DU®mtfl
,~~~~~
Pneumatosis Intestinalis in the Infant Ademakinwa M. Adekunle, MD, Albert Z. Holloway, MD, Trevor Golding, MD, and Harry C. Press, Jr., MD Washington, D.C.
Clinical Background This male infant was delivered by a 26-year-old mother with two previous abortions. The gestational age was 30 weeks and the infant weighed 1,020 gm at birth. His Apgar score was 8 at one minute and 9 in five minutes. Because of his prematurity, grunting respiration, and intercostal retraction, he was admitted into the Nursery Intensive Care Unit. Respiratory supportive measures were instituted and an umbilical arterial catheter, the tip of which was at T 10,
From the Departments of Radiology and Pediatrics, Howard University Hospital, Washington, D.C. Requests for reprints should be sent to Dr. Harry C. Press, Department of Radiology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, D.C. 20060.
was inserted. He was stabilized and did fairly well in the first 24 hours. Fortyeight hours after birth, he developed respiratory distress syndrome which was evidenced both clinically and radiographically. On the third day of life, he developed left pneumothorax for which a chest tube was inserted. He began to have apneic spells which increased in frequency. His abdomen was distended and tense and his stool was bloody. Because the umbilical arterial catheter was blocked, it was removed and replaced with an umbilical venous catheter. The accompanying radiograph was then obtained. Which one of the following is the most likely diagnosis? 1. Meconium peritonitis. 2. Meconium ileus. 3. Necrotizing enterocolitis. 4. Aganglionosis 5. Meconium plug syndrome.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 1, 1978
Radiographic Findings The chest demonstrates the fine reticulonodular pattern seen in respirat-
Figure 1. Three-day-old infant with apneic spells, abdominal distension, and bloody stools. 63
ory distress syndrome. The chest tube was inserted for pneumothorax. The endotracheal tube is fairly low within the trachea. An umbilical venous catheter is noted in situ. In the infant, it is usually difficult to differentiate the small bowel from colon gas. However, notice the distention of the ascending and proximal descending colon at the periphery of the abdominal cavity. The bubbly appearance represents a combination of air, fecal material, blood, and sloughed mucosa within the lumen of the colon. The colon is well outlined here because of intramural air in the wall.
caliber distal to the obstruction. Intramural gas is not a feature of this condition, but the bubbly appearance may be seen proximal to the obstruction. The meconium plug is relieved by enemas and the infants do not have this clinical presentation. Infants do not exhibit the "formed-stool appearance" seen in adults, because their stools are frequently liquid in consistency. Such a "bubbly" pattern, as seen in this case, is highly suggestive of necrotizing enterocolitis, which is the correct diagnosis in this case. There is no evidence of hepatic portal venous gas or pneumoperitoneum.
Differential Diagnosis
Discussion
Meconium peritonitis is almost always associated with proximal smallbowel obstruction. There is perforation of the bowel in utero proximal to the obstruction and there is spillage of meconium into the peritoneal cavity. An inflammatory reaction follows and there is subsequent calcification of the meconium. These flecks of calcification can usually be seen at birth. These are absent in this case. Meconium ileus is seen in infants with mucoviscidosis. The plain film of the abdomen usually shows several loops of dilated small bowel, which may contain air-fluid levels on erect or lateral decubitus roentgenograms. A "frothy" appearance may also be seen in meconium ileus as a result of admixture between the tanacious meconium and gas in the terminal ileum. Aganglionosis does not present this early in life. However, because necrotizing enterocolitis may sometimes complicate aganglionosis, it makes it necessary to evaluate all survivors of this disease for aganglionosis. The timing of the onset of clinical and radiographic features makes it unlikely to be secondary to an underlying aganglionosis. The meconium plug syndrome is seen in newborn infants and the colon is usually normal or near normal in
Necrotizing enterocolitis (NEC) was first described by Siebold in 1825. It is a very serious disease, with a mortality rate of well over 75 percent" 2 The patient who develops NEC is typically premature by weight and gestational age. Eighty percent weigh less than 2,000 gm and the gestational age is 34 weeks or less. Respiratory distress syndrome occurs in 75 percent of these patients.3 The average age of onset of NEC is 6.5 days. Nearly half of all cases occur between the 3rd and 4th days.4 The etiology of this disorder is not specifically known. It is seen most often in infants who have survived a period of a severe, shock-like state. Exchange transfusion, umbilical arterial or venous catheterization, various causes of perinatal hypoxia, or asphyxia have been cited as contributing factors in development of intestinal necrosis.' It may be idiopathic, associated with a mechanical functional obstruction or Hirschsprung's disease. Clinically an infant with NEC presents with abdominal distention, bile emesis, and hematochezia. Other clinical findings such as peritonitis with or without perforation, shock, or disseminated intravascular coagulopathy were present in patients having extensive involvement .4
A radiographic finding of pneumatosis intestinalis (PI) and adynamic ileus, although not specific for NEC, confirms the diagnosis if there is a high clinical index of suspicion. A patient may have NEC without pneumatosis intestinalis. These cases are confirmed at surgery or autopsy. There is no relationship of clinical severity or outcome either to pneumotosis intestinalis or adynamic ileus. There may be extensive disease found at surgery without severe radiologic findings or extensive pneumatosis intestinalis with a benign clinical course not requiring surgery.4 The development of PI had been explained on the basis of intraluminal gas entering bowel walls mechanically after the mucosa had been ulcerated. Other authors have shown that carbohydrate in the intestinal tract plus the presence of bacteria in the duodenum are required for PI to develop.5 Both substrate and bacteria are necessary for the production of hydrogen gas. Hydrogen is a major constituent of intramural gas found in NEC.4 A roentgenographic finding of PT occurs in 75 percent of patients.3 With progression of the disease perforation into the abdominal cavity is likely. In addition, gas may enter the portal system and be carried to the liver. The demonstration of gas within the hepatic portal venous system indicates a grave prognosis. Vollman et al reported a case of an infant who survived two separate episodes of PI and hepatic portal venous gas.6 Franz et al4 also reported the reappearance of signs of NEC in several of their patients in whom enteric feedings were restarted prior to 10 days of medical therapy.
Literature Cited 1. Cohn R, Sunshine R, deVries P: Necrotizing enterocolitis in the newborn infant. Am J Surg 124:165-168, 1972 2. Rabinowitz JG, Wolf BS, Feller MR, et al: Colonic changes following necrotizing enterocolitis in the newborn. Am J Roentgenology 103:539-634, 1968 3. Roback SA, Foker J, Frantz IF, et al: Necrotizing enterocolitis. Arch Surg 109:314-319, 1974 4. Frantz lil ID, L'Heureux P, Engel RR, et al: Necrotizing enterocolitis. J Pediatr 86(2):259263, 1975 5. Coello-Ramirez P, Gutierrex-Topete G, Lifshitz F: Pneumatosis intesinalis. Am J Dis Child 120:3-9, 1970 6. Vollman JH, Smith WL, Tsang RC: Necrotizing enterocolitis with recurrent hepatic portal venous gas. J Pediatr 88(3):486-487, 1976
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 70, NO. 1, 1978
