Unexplained Pneumoperitoneum Sclerema Neonatorum By Edward
G. Stanley-Brown New
and
York,
Dennis
and
J. Allendorf
N.Y.
A white male infant, product of an entirely normal pregnancy, was delivered by vacuum extraction. His mother was 29 yr old, gravida II, para II. No resuscitation was needed; the Apgar score was 9 at I min and 10 at 5 min. Examination at I hr of age revealed a 3374-g white male who appeared pale and not too active. The heart rate was 160/min and the rectal temperature was 37°C. Meconium was passed 3 hr after birth, and the physical examination was essentially normal. No obvious congenital anomalies were apparent. Because of pallor and listlessness, a chest x-ray was obtained along with other studies to rule out sepsis. Free air was noted beneath the diaphragm and a pediatric surgical consultation was requested. Examination at 7 hr of age revealed a pale, obviously sick male. The abdominal examination was almost completely normal. No organs or masses could be felt. The bowel sounds were normal. There was slight but soft abdominal distension. Meconium stooling continued and there was no vomiting. The erect film of the abdomen also revealed free air and showed a normal cardiothymic silhouette with clear lung fields and unremarkable pulmonary vasculature. At this juncture the working diagnosis was sepsis with pneumoperitoneum. Ampicillin and kanamycin were administered empirically. He was placed in a warm isolette with humidity but never required oxygen. Cultures of blood, cerebrospinal fluid, urine, gastric aspirate. and stool all revealed no growth or normal flora. Serial x-rays of the abdomen showed gradual diminution of the free intraperitoneal air and at no time was any gas seen within the bowel wall. At 3 days of age the patient exhibited sclerema neonatorum spreading from the abdomen to involve the entire body. His general condition was very poor but the abdomen remained normal to physical examination. The entire body was cold with tight skin and generalized marked induration. Since there was little to lose with this seriously ill baby, an exchange transfusion was carried out during the evening of the third day of life. Five hundred cubic centimers of A-negative blood was used and by the fourth day of life the baby seemed markedly improved. The extremities were notably softer to the touch, and he was much more alert, crying and sucking vigorously, and passing stools. By day 5 the sclerema seemed to be subsiding. We then entered a trying period of I5 days which followed a similar pattern. The baby’s general condition would worsen and, following an exchange transfusion, would impressively improve. Over these 2 wk he received four more exchange transfusions for a total of five during his hospitalization. Attempts at feeding met with failure before exchange transfusions and then progressed well after each exchange. There was regurgitation but never frank vomiting. At age 26 days the worst seemed to be over. He was feeding fairly well and stooling normally. Throughout this patient’s hospital course, many of the staff involved in his care were convinced that he had suffered a GI perforation with peritonitis to explain the free intraperitoneal air. Despite his obvious well being he was subjected to thorough x-ray examination. A barium swallow showed marked chalasia, which explained the regurgitation, and this promptly subsided when the patient was propped up in a chalasia chair and given thickened feedings. Upper GI series, barium enema, IVP, and voiding cystourethrogram were all normal. By June IO, 1974, the patient weighed 3650 g and he was entirely well. Antibiotics had been discontinued IO days previously and he was discharged home. At this writing, he is enjoying normal growth and development and regurgitation has almost ceased.
0 1976 by Crune & Stratton,
Inc.
Journal of Pediatric Surgery, Vol. 11, No. 1 (February),
1976
123
124
CASE REPORTS
It is difficult to explain the origin of free intraperitoneal air in a patient who had no resuscitation at birth and had neither acute nor chronic respiratory disease.’ At no time was there any clinical evidence of peritonitis, and exhaustive x-rays performed after his serious illness gave no hint that perforation of the GI tract had occurred. Recovery from sclerema neonatorum is infrequent. Exchange transfusions have been reported in Europe to be helpful.’ We have not tried this approach before. The marked clinical improvement that followed each exchange transfusion was impressive indeed. What part the antibiotic treatment played in this picture is unclear, since none of the innumerable cultures ever grew any offending organisms. Recovery from sclerema usually depends on successful treatment of the underlying cause. Presumably this was accomplished in this patient. REFERENCES I. Leonidas SG, Hall RT, Holder TM, et al: Pneumoperitoneum associated with chronic respiratory disease in the newborn. Pediatrics 51:933.1973
2. Prod’hom LS, Choffat JM, Frenck N, et al: .Care of the seriously ill neonate with hyaline membrane disease and with sepsis (sclerema neonatorum). Pediatrics 53: 170, 1974
G. Stanley-Brown New
and
York,
Dennis
and
J. Allendorf
N.Y.
A white male infant, product of an entirely normal pregnancy, was delivered by vacuum extraction. His mother was 29 yr old, gravida II, para II. No resuscitation was needed; the Apgar score was 9 at I min and 10 at 5 min. Examination at I hr of age revealed a 3374-g white male who appeared pale and not too active. The heart rate was 160/min and the rectal temperature was 37°C. Meconium was passed 3 hr after birth, and the physical examination was essentially normal. No obvious congenital anomalies were apparent. Because of pallor and listlessness, a chest x-ray was obtained along with other studies to rule out sepsis. Free air was noted beneath the diaphragm and a pediatric surgical consultation was requested. Examination at 7 hr of age revealed a pale, obviously sick male. The abdominal examination was almost completely normal. No organs or masses could be felt. The bowel sounds were normal. There was slight but soft abdominal distension. Meconium stooling continued and there was no vomiting. The erect film of the abdomen also revealed free air and showed a normal cardiothymic silhouette with clear lung fields and unremarkable pulmonary vasculature. At this juncture the working diagnosis was sepsis with pneumoperitoneum. Ampicillin and kanamycin were administered empirically. He was placed in a warm isolette with humidity but never required oxygen. Cultures of blood, cerebrospinal fluid, urine, gastric aspirate. and stool all revealed no growth or normal flora. Serial x-rays of the abdomen showed gradual diminution of the free intraperitoneal air and at no time was any gas seen within the bowel wall. At 3 days of age the patient exhibited sclerema neonatorum spreading from the abdomen to involve the entire body. His general condition was very poor but the abdomen remained normal to physical examination. The entire body was cold with tight skin and generalized marked induration. Since there was little to lose with this seriously ill baby, an exchange transfusion was carried out during the evening of the third day of life. Five hundred cubic centimers of A-negative blood was used and by the fourth day of life the baby seemed markedly improved. The extremities were notably softer to the touch, and he was much more alert, crying and sucking vigorously, and passing stools. By day 5 the sclerema seemed to be subsiding. We then entered a trying period of I5 days which followed a similar pattern. The baby’s general condition would worsen and, following an exchange transfusion, would impressively improve. Over these 2 wk he received four more exchange transfusions for a total of five during his hospitalization. Attempts at feeding met with failure before exchange transfusions and then progressed well after each exchange. There was regurgitation but never frank vomiting. At age 26 days the worst seemed to be over. He was feeding fairly well and stooling normally. Throughout this patient’s hospital course, many of the staff involved in his care were convinced that he had suffered a GI perforation with peritonitis to explain the free intraperitoneal air. Despite his obvious well being he was subjected to thorough x-ray examination. A barium swallow showed marked chalasia, which explained the regurgitation, and this promptly subsided when the patient was propped up in a chalasia chair and given thickened feedings. Upper GI series, barium enema, IVP, and voiding cystourethrogram were all normal. By June IO, 1974, the patient weighed 3650 g and he was entirely well. Antibiotics had been discontinued IO days previously and he was discharged home. At this writing, he is enjoying normal growth and development and regurgitation has almost ceased.
0 1976 by Crune & Stratton,
Inc.
Journal of Pediatric Surgery, Vol. 11, No. 1 (February),
1976
123
124
CASE REPORTS
It is difficult to explain the origin of free intraperitoneal air in a patient who had no resuscitation at birth and had neither acute nor chronic respiratory disease.’ At no time was there any clinical evidence of peritonitis, and exhaustive x-rays performed after his serious illness gave no hint that perforation of the GI tract had occurred. Recovery from sclerema neonatorum is infrequent. Exchange transfusions have been reported in Europe to be helpful.’ We have not tried this approach before. The marked clinical improvement that followed each exchange transfusion was impressive indeed. What part the antibiotic treatment played in this picture is unclear, since none of the innumerable cultures ever grew any offending organisms. Recovery from sclerema usually depends on successful treatment of the underlying cause. Presumably this was accomplished in this patient. REFERENCES I. Leonidas SG, Hall RT, Holder TM, et al: Pneumoperitoneum associated with chronic respiratory disease in the newborn. Pediatrics 51:933.1973
2. Prod’hom LS, Choffat JM, Frenck N, et al: .Care of the seriously ill neonate with hyaline membrane disease and with sepsis (sclerema neonatorum). Pediatrics 53: 170, 1974
