Disseminated Eosinophilic Infiltration of a Newborn Infant, with Perforation of the Terminal Ileum and Bile Duct Obstruction

Arch Dis Child: first published as 10.1136/adc.56.1.66 on 1 January 1981. Downloaded from

66 Shinozaki, Saito, and Shiraki

infant who acquired hepatitis from her mother. Br Med J 6 Yoshida A, Tozawa M, Furukawa N, Oya N, Kusunoki T, 1970; iv: 719-21. Kiyosawa N. HBsAg-positive chronic active hepatitis in 3 Bancroft W H, Warkel R L, Talbert A A, Russell P K. a 1 and 1/2 year-old-child (in Japanese). Shonika Shinryo Family with hepatitis-associated antigen. JAMA 1971; 1977;40: 1246-50. 217:1817-20. McCarthy J W. Hepatitis B antigen (HBAg)-positive chronic aggressive hepatitis and cirrhosis in an 8-month- Correspondence to Dr T Shinozaki, Department of old infant. A case report. JPediatr 1973; 83: 638-9. Paediatrics, Teikyo University School of Medicine, 11-1 5 Fujiwara T, Abe M, Tachi N, Jo M, Shiroda M. Kaga, 2 Chome, Itabashi-ku, Tokyo 173, Japan. HBsAg-positive infantile hepatitis associated with chronic aggressive hepatitis (in Japanese). Shonika Rinsho 1975; 28:1303-6. Received 26 November 1979

Disseminated eosinophilic infiltration of a newborn infant, with perforation of the terminal ileum and bile duct obstruction

S M MURRAY AND C J WOODS Department ofPathology and Department ofPaediatrics, Victoria Hospital, Blackpool

Case report SUMMARY A preterm boy died 4 days after delivery from septicaemia which at necropsy was found to be A white boy, weighing 1490 g, was born by spon- due to perforation of an eosinophilic lesion of the taneous vertex delivery at 35 weeks' gestation to a copyright. terminal ileum. Eosinophilic infiltration was also healthy 19-year-old rubella-immune group A Rh- found in kidney, lymph node, bone marrow, portal positive mother, who had a severe antepartum tracts of liver, gall bladder, and bile duct with associ- haemorrhage due to placenta praevia. The pregnancy ated obstruction of the cystic duct and mucocele of had been uneventful, apart from vaginal bleeding at the gall bladder. No allergic cause for the infiltrate 13 and 18 weeks which had settled without treatment was found in either the infant or his mother. Eosino- on each occasion. No drugs had been taken during the philic infiltration of neonatal spleen, lymph node, pregnancy. http://adc.bmj.com/ intestinal mucosa, epicardium, thymus, pancreas, There was no birth asphyxia, regular respirations portal tracts of the liver, and skin has been reported were established within 1 minute and, apart from but the aggressive behaviour of the infiltrate in this being preterm, there were no abnormalities on patient bears more resemblance to the eosinophilic clinical examination. 1-mg of vitamin K1 was gastroenteritis that has been described in older given intramuscularly. Intermittent intragastric tube children and adults. feeding with low solute milk was started and, apart from occasional regurgitation of small amounts of Eosinophilic infiltration of the alimentary tract of the feed, the infant made satisfactory progress. on September 30, 2021 by guest. Protected adult occurs in two forms-namely eosinophilic Meconium was first passed at age 8 hours. gastroenteritis and inflammatory fibroid polyp of the At age 48 hours the infant's condition deteriorated gastrointestinal tract-each has been compre- suddenly with the clinical picture of peripheral hensively reviewed by Johnstone and Morson.1-2 circulatory failure. There was no significant The condition is described much less often in children abdominal distention. Cerebrospinal fluid showed and in two reviews3-4 only 13 cases were recorded in only 1 lymphocyte/mm3 and no organisms were children under 12 years, the youngest being a 2-year- found on microscopical examination or culture. old boy. Eosinophilic infiltration of the gastro- The haemoglobin was 10 9 g/dl, packed cell volume intestinal tract has also been described in neonatal 33.9%/, and white blood count 1000 x 109/l. The necrotising enterocolitis5 but the significance of this scanty nucleated cells seen in the blood film were infiltrate is unknown. No reference to disseminated either lymphocytes or normoblasts with no increase eosinophilic infiltration in the neonate followed by in eosinophils. intestinal perforation has been found in a search of Septicaemia was diagnosed and treatment with the recent literature. intravenous gentamicin and cloxacillin was started. Arch Dis Child: first published as 10.1136/adc.56.1.66 on 1 January 1981. Downloaded from

Disseminated eosinophilic infiltration ofa newborn infant 67 Oral feeding was stopped and intravenous dextrose be patent even a fine probe could not be passed along saline started. Blood culture subsequently grew the cystic duct. The lungs were severely congested. Escherichia coli sensitive to gentamicin. The other organs appeared normal. At age 53 hours the infant had a severe apnoeic attack requiring endotracheal intubation and inter- Histological examination. The perforated segment of mittent positive pressure ventilation. 30-ml of blood terminal ileum showed intense congestion and were transfused and metabolic acidosis corrected oedema with infiltration of the entire thickness of the with intravenous sodium bicarbonate. However, at wall by large numbers of eosinophils together with age 72 hours abdominal distension developed and some lymphocytes and plasma cells (Figs 1 and 2). the limbs showed sclerematous changes. Refractory The serosal surface showed a fibrino-purulent exu- hypoxaemia and acidosis ensued and despite con- date containing, in addition to polymorphonuclear tinued mechanical ventilation the infant died. He was leucocytes, numerous clumps of Gram-negative aged 90 hours. bacilli and moderate numbers of eosinophils. The gall bladder showed mild focal infiltration of its wall Necropsy findings by mononuclear cells and eosinophils while the bile ducts, particularly the cystic duct, showed severe Perforation of a severely congested short segment of congestion and oedema with cellular infiltration terminal ileum had caused acute peritonitis. The gall resembling that of the ileum. The liver showed bladder was distended with clear mucus and although haematopoiesis of normal extent for a preterm infant the common bile duct and hepatic ducts appeared to of this size but eosinophils were absent from these copyright. http://adc.bmj.com/ on September 30, 2021 by guest. Protected

Fig. 1 Full thickness section of - terminal ileum near point of l_- N perforation showing cellular infiltration. Prominent dilated blood vessels are seen in the submucosa. (H and E x 178). Arch Dis Child: first published as 10.1136/adc.56.1.66 on 1 January 1981. Downloaded from

68 Murray and Woods

Fig. 2 Greater magnification of boxed area ofFig. 1. 49 % ofthe

cells in this field are eosinophils. copyright. (H and E x 1650). http://adc.bmj.com/

haematopoietic foci. The portal tracts contained normal. In all the tissues except bone marrow the numerous eosinophils but there was no significant eosinophils were of mature form. Mast cells were not increase in portal fibrous tissue. greatly increased in any of the tissues.

The subcapsular region of the kidney showed on September 30, 2021 by guest. Protected infiltration by eosinophils which extended deeply into the cortex in a linear manner associated with the Discussion nephrogenic formations at the margins of persistent fetal lobules (Figs 3 and 4). The lungs showed The sequence of events in this infant appeared to be intense congestion and intra-alveolar haemorrhage eosinophilic infiltration of various organs, particu- but there was no inflammatory cell infiltration and no larly the terminal ileum, followed by perforation of eosinophil infiltration. Extra medullary haemato- the terminal ileum and death from E. coli peritonitis. poiesis was not present in the lung. Bone marrow The main interest is in the nature of the eosinophilic from sternum and upper end of femur was modera- infiltrate and this suggests an inflammatory rather tely cellular and contained large numbers of mature than a neoplastic process. The abnormal histiocytic and immature eosinophils. The subcapsular sinuses cells characteristic of Letterer Siwe's disease are not of a lymph node from the neck were infiltrated by seen and the findings do not support a diagnosis of eosinophils but these were not seen elsewhere in the eosinophilic leukaemia. node. Salivary gland and myocardium appeared The infant lived for only 4 days and it is unlikely Arch Dis Child: first published as 10.1136/adc.56.1.66 on 1 January 1981. Downloaded from

Disseminated eosinophlilic infiltration ofa newborn infant 69

Fig. 3 Renal cortex showing band-like cellular infiltrate at interface between 2 persistent fetal lobules. (H and E x 143). copyright.

that such a widespread and severe eosinophilia et al.11 described eosinophilic infiltration of the could be an allergic response to the reconstituted oesophagus in a 3-month-old infant. Eosinophilic milk with which he had been fed for 48 hours before infiltration of the pancreas in infants born to diabetic http://adc.bmj.com/ the sudden collapse. The membranes had been mothers is well documented.12 ruptured for several hours before delivery but in the Eitzman and Smith'3 have produced evidence that absence of inflammatory cell infiltration of the lungs some infants between ages 2 and 21 days respond to this is an unlikely cause of the abnormal cellular nonspecific stimuli with a pronounced eosinophilia. infiltration of other tissues. Careful search of all the Perhaps this is the explanation for the widespread fetal tissue taken at necropsy showed no fungi or eosinophilia seen in our patient and perhaps the initial

parasites, or any other causative agents. No eosino- lesion was necrotising enterocolitis. This view is sup- on September 30, 2021 by guest. Protected phil count was performed on the mother but she had ported by Tait and Kealy5 who stressed the frequent shown no signs of any allergic or other disorder occurrence of eosinophilia in the affected segment of during or after the pregnancy. intestine in this form of enterocolitis. However, A common eosinophilic disorder of the newborn although necropsy was performed on 1 of their 5 infant is erythema toxicum neonatorum in which the infants, there was no mention of eosinophilia of any erythematous skin lesions show intense eosinophilic organ other than the intestine. In our patient, in infiltration.6 Other references to eosinophilic in- addition to eosinophilia of the perforated segment of filtration of the neonate are scanty. Eosinophilic bowel, there was an increase in eosinophils in the infiltration of the thymus7 and portal tracts of the kidney, portal tracts of liver, bone marrow, lymph liver8 are normal findings in the neonatal period node sinuses, gall bladder, and cystic duct but not while eosinophils may persist in spleen, lymph in the lung, salivary gland, myocardium, or blood. nodes, and intestinal mucosa for several months Perhaps this report will draw attention to the after birth.9 Eosinophilic infiltration of the epi- possibility that apparently localised eosinophilic cardium has been described in 3 infants, 2 of whom lesions in the neonate may in fact be part of a more had multiple congenital abnormalities,10 and Forget widespread spectrum of eosinophilic infiltration. Arch Dis Child: first published as 10.1136/adc.56.1.66 on 1 January 1981. Downloaded from

70 Murray and Woods

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References Tait R A, Kealy W F. Neonatal necrotising enterocolitis. J Clin Pathol 1979; 32: 1090-9. ' Johnstone J M, Morson B C. Eosinophilic gastroenteritis. 6 Freeman R G, Spiller R, Knox J M. Histopathology of Histopathology 1978; 2: 335-48. erythema toxicum neonatorum. Arch Dermatol 1960; 82: 2 Johnstone J M, Morson B C. Inflammatory fibroid polyp 586-9. of the gastrointestinal tract. Histopathology 1978; 2: 7 Bhathal P S, Campbell P E. Eosinophil leucocytes in the 349-61. child's thymus. Australas Ann Med 1965; 14: 210-3. 3 Jona J Z, Belin R P, Burke J A. Eosinophilic infiltration 8 Potter E L, Craig J M. Pathology of the fetus and the of the gastrointestinal tract in children. Am J Dis Child infant. London: Lloyd-Luke, 1976: 395-6. 1976; 130: 1136-9. 9 Dourov N. Les eosinophiles tissulaires au cours de 4 Hoefer R A, Ziegler M M, Koop C E, Schnaufer L. la periode p6rinatale humaine. Pathol Eur 1967; 2: Surgical manifestations of eosinophilic gastroenteritis in 124-45. the pediatric patient. JPediatr Surg 1977; 12: 955-62. 10 Moragas A, Vidal M T. Granulomatous eosinophilic Arch Dis Child: first published as 10.1136/adc.56.1.66 on 1 January 1981. Downloaded from

Disseminated eosinophilic infiltration of a newborn infant 71 epicarditis in the newborn. Report of three cases. Arch 13 Eitzman D V, Smith R T. The nonspecific inflammatory Pathol 1969; 88: 459-62. cycle in the neonatal infant. Am J Dis Child 1959; 97: 11 Forget P, Eggermont E, Marchal G, Geboes K, Jaeken J, 326-34. Melchior S. Eosinophilic infiltration of the oesophagus in an infant. Acta Paediatr Beig 1978; 31: 91-3. Correspondence to Dr S M Murray, Department of 12 Pedersen L M, Tygstrup I. Cell infiltration in the pancreas Pathology, Victoria Hospital, Blackpool FY3 8NR. of newborn infants of diabetic mothers. Acta Pathol Microbiol Scand 1968; 73: 537-48. Received 29 January 1980.

Spontaneous oesophageal rupture with duodenal atresia in a newborn infant

HAJIME NAKAMURA, YOSHIHIKO KANAZAWA, MASAKI HAYANO, AND SHINICHIRO MIMASU Department ofPaediatrics, Kobe University School of Medicine, Japan

was rapid, shallow, and grunting. He was transferred suMMARY A newborn infant with spontaneous to this hospital. oesophageal rupture associated with duodenal On initial examination he was moribund, in severe atresia is described. The diagnosis was established by respiratory distress, with pronounced cyanosis and the pattern ofhydropneumothorax on the chest x-ray cold extremities. The abdomen was distended and copyright. film and by the aspiration of gastric juice by thora- movement was diminished on the right side cocentesis. Necropsy showed a tear, which extended of the chest. Petechiae were found on the chest from the oesophagogastric junction to the upper and abdominal walls. Plain x-ray film showed portion ofthe stomach. tension hydropneumothorax on the right (Figure). In addition, the stomach and duodenum were Spontaneous oesophageal rupture in infancy is rare, distended, distal to which no gas was seen. Thoraco-

but it is important to differentiate it from any other centesis was performed; considerable amounts of air http://adc.bmj.com/ disease that causes serious respiratory distress or and 30 ml of bile-stained fluid were obtained. cyanosis shortly after birth. Since 1952, when the Respiratory effort was poor and he received assisted first case of oesophageal rupture was reported,' ventilation: pH 6 98, Pao2 94 mmHg (12.5 kPa), only 14 cases have been cited.2-3 The disease shows a Paco2 56 mmHg (7A4 kPa), base excess -16 mmol/l. pattern of hydropneumothorax, a finding charac- At age 24 hours, a general haemorrhagic tendency teristic on chest x-ray films. It should therefore not be and sclerema neonatorum were noted. After exchange too difficult to make an early diagnosis if one knows transfusion with fresh heparinised blood, the of the existence of the disease. Some patients survive tendency to bleed and the metabolic acidosis, as well on September 30, 2021 by guest. Protected early surgical operation. as his general condition were improved, but an x-ray film showed pneumoperitoneum. Case report At age 92 hours, laparotomy was performed through an upper midline incision. Purulent matter A Japanese boy was born after 38 weeks' gestation was attached to the upper portion of lesser curvature after induction for hydramnios. Delivery had been of stomach. When the stomach was pulled down, a spontaneous with a vertex presentation. Birthweight longitudinal rupture 1 5 cm in length was found in was 2 * 75 kg and he was the first child of a 26-year-old the anterolateral aspect of the lower end of the mother. He cried spontaneously and required no oesophagus, spanning the diaphragmatic hiatus and resuscitation. Mucus was suctioned from the nose extending to the stomach. The rupture was closed. and nasopharynx, but no tube was passed into the Atresia of the duodenum was found, the second oesophagus. Three hours after birth, breathing was portion of which ended blindly at an annular noted to have become laboured and he was placed in pancreas. A duodenojejunostomy was fashioned. A an oxygen tent. By 19 hours, the infant was spitting gastrostomy was performed for feeding. The infant small amounts of bright red blood and the respiration tolerated the procedures and chest x-ray showed