Short reports 395in which there was rapidly progressive unilateralexophthalmos. In the report of Galyean andRobertson (1970), the patient presented in much thesame way as ours with puffy eyes, irritability, andpoor feeding. This child had glaucoma whichresolved along with the hyperostosis over a periodof 4 months.The occurrence of thrombocytosis in Caffey's

disease is now well recognized. Pickering andCuddigan (1969) described 3 cases, all with raisedplatelet counts. In their review of reports pub-lished in English they found 3 cases with plateletcounts >500 000/mm3, this measurement havingbeen made in only 9 of 132 cases. Our patient'splatelets rose to 910 000/mm3 but she suffered nothrombotic complications and no anticoagulant orantimegakaryocytic agents were used.The reason for the thrombocytosis in Caffey's

disease is not known. The low vitamin E levelsshown in our patient may be a fortuitous finding butit is tempting to postulate a possible associationbetween the thrombocytosis and the vitamin Edeficiency in our patient. Ritchie et al. (1968)described a syndrome of oedema, haemolyticanaemia, and thrombocytosis in association withvitamin E deficiency. Thrombocytosis was alsonoted by Hassan et al. (1966) in their description ofa syndrome in premature infants associated withlow plasma vitamin E levels and a high poly-unsaturated fatty acid diet. Our patient was fedon Cow and Gate 'Premium', then changed to Oster-milk II. She received Abidec drops (vitamins A,B, C, D) but no vitamin E. The plasma vitamin Elevel rose spontaneously to reach the lower level ofnormal after 3 months. There was no evidence ofhaemolysis.Whether vitamin E deficiency is a factor in the

pathogenesis of Caffey's disease remains to be seen.It is hoped that this report will stimulate furtherstudies of vitamin E levels in infantile corticalhyperostosis.

SummaryAn infant is described who presented with ptosis

and periorbital oedema and was found to haveinfantile cortical hyperostosis with thrombocytosis,raised IgM, and vitamin E deficiency.

I am indebted to Dr. A Moosa for his advice in pre-paring this paper and to Dr. D. P. R. Muller, Instituteof Child Health, The Hospital for Sick Children, forthe measurements of vitamin E levels.

REaRNcESCaffey, J., and Silverman, W. A. (1945). Infantile cortical hypero-

stosis: preliminary report on a new syndrome. AmericanJournal of Roentgenology, Radium Therapy, and Nuclear Medicine,54, 1.

Galyean, J., and Robertson, W. 0. (1970). Caffey's syndrome: someunusual ocular manifestations. Pediatrics, 45, 122.

Hassan, H., Hashim, S. A., van Italie, T. B., and Sebrell, W. H.(1966). Syndrome in premature infants associated with lowplasma vitamin E levels and high polyunsaturated fatty aciddiet. American Journal of Clinical Nutrition, 19, 147.

Iliff, C. E., and Ossofsky, H. J. (1962). Infantile cortical hyperosto-sis: an unusual cause of proptosis. American Journal ofOphthalmology, 53, 976.

Minton, L. R., and Elliott, J. H. (1967). Ocular manifestations ofinfantile cortical hyperostosis. American Journal of Ophthal-mology, 64, 902.

Pickering, D., and Cuddigan, B. (1969). Infantile cortical hypero-stosis associated with thrombocythaemia. Lancet, 2, 464.

Ritchie, J. H., Fish, M. B., McMasters, V., and Grossman, M.(1968). Edema and hemolytic anemia in premature infants.A vitamin E deficiency syndrome. New England J'ournal ofMedicine, 279, 1185.

B. R. KEETONDepartment of Paediatrics and Neonatal Medicine,Hammersmith Hospital, Du Cane Road, LondonW12.

Gastric pneumatosis in infancyGastric pneumatosis (defined as gas within the

wall of the stomach) may be easily detected byradiography. It presents as a fine lucent stripeconforming to the contour of the stomach andenveloping any intraluminal gas and fluid content.The finding, though quite rare, is of utmost clinicalimportance. In adults, gastric pneumatosis iscaused either by gas-forming bacterial infection(emphysematous gastritis), or in association withbullous emphysema (Holgersen, Borns, and Srouji,1974). In infancy, isolated gastric pneumatosis hasbeen seen very rarely in gastric outlet obstruction.It has also been reported in neonatal necrotizingenterocolitis, in association with intestinal pneuma-tosis (Bell, Graham, and Stevenson, 1971; Santulliet al., 1975) though it may rarely be isolated (Robin-son, Grossman, and Brumley, 1974). Thus, radio-graphic detection of gastric pneumatosis indicatesserious underlying disease and determination of itscause will depend on the associated clinical findings,a point illustrated by the 2 cases presented here.

Case reportsCase 1. A 6-week-old girl developed vomiting,

constipation, and oliguria 5 days before admission.She was a well-nourished infant, mildly dehydrated, andirritable. There was moderate epigastric distension butno visible peristaltic waves. No pyloric 'tumour' couldbe palpated. Laboratory studies including serumelectrolytes were normal, except for slightly raisedblood urea nitrogen (18-7 mg/100 ml). Radiographicexamination of the abdomen showed gastric distensionwith a long air fluid level in the stomach seen on the up-right film. The findings suggested gastric retention.

396 Short reports

-.;R$x,:i! ::^!:: .,;' -,. . 'e' T. :R . g '::: . . > .- <<< u*- ::iS:r:' ?. .a4.5lk.,. . < ... & i,v; P... ,. 9 'bEw6 .x::j>, i.,

'& tg C, .h.i. ,.

,,, ,,.-- X,. ,. W,-- -..... .:.S.,,z,,. :. ,: .., :E :. :e..::' .'.' .':: .: :. '' :lS. :'

t ,,.. ,:?..ep! .. :.* : :^ ,.::':: ... ::.

FIG. 1.-Case 1. Upright film. Gastric distension; intramural air around the fluid-filled gastric antrum.

A continuous curvilinear lucent stripe was noted to (gastric pneumatosis) (Fig. 1). Small amounts of gasenvelop the gastric contour, typical of intramural gas were scattered in the small bowel and colon.

N R

-iw

4i

Short reports

FIG. 2.-Case 2. Linear pneumatosis surrounids the air-filled fundus of the stomach (arrows).

The clinical and radiological findings led to the diag-nosis of gastric outlet obstruction. A nasogastriccatheter was passed into the stomach and copiousamounts of gastric contents were aspirated. Within afew hours a second radiographic examination showedmarked gastric decompression and disappearance ofgastric pneumatosis. Barium introduced in the stomachvia the catheter under fluroscopic control showed thetypical x-ray signs of hypertrophic pyloric stenosis.At operation a long leathery pyloric sphincter wasfound and pyloromyotomy carried out. The patienttolerated the procedure well and has remained well.

Case 2. A female infant was born to a 21-year-oldgravida 2, para 3 mother, after approximately 28 weeks'gestation. She was the second of twins and weighed1178 g. Dyspnoea, tachypnoea, expiratory grunt, andsubstemal retractions were noted soon after birth, andon auscultation of the lungs there were fine cracklingriles bilaterally. An umbilical artery catheter was

placed in the mid-thoracic aorta. Blood gases gavepH 7-23, Pao2 70 mmHg, and Paco2 36 mmHg inF,o2 30%. A chest x-ray was consistent with mildidiopathic respiratory distress syndrome. She respond-ded well to moderately increased concentration ofinspired 02 administered by continuous positive airwaypressure. A right upper lobe pneumonia was identifiedon chest x-rays on the 13th day and tracheal aspirategrew pseudomonas. She was treated successfully withcarbenicillin and gentamicin. Her course was alsocomplicated by hyperbilirubinaemia and a persistentductus arteriosus for which she was treated with digitalis.A transpyloric tube was used for nasojejunal feedings

on the 15th day but was discontinued 2 days later be-cause of abdominal distension. Diarrhoea, recurrentdistension, and diminished bowel sounds were noted onthe 33rd day. Abdominal films showed gastric pneuma-tosis (Fig. 2) and colonic dilatation, but there was noevidence of intestinal pneumatosis. Oral feedings werediscontinued, intravenous fluids were started, and she

397

398 Short reportswas given penicillin and gentamicin for 5 days. Duringthis time abdominal distension subsided, bowel soundsbecame active, and diarrhoea decreased. Gastricpneumatosis disappeared 2 days after onset of therapy.The infant had a protracted course with poor weightgain and periods of apnoea and bradycardia. She wasfinally discharged at the age of 31 months in good health.

DiscussionX-ray diagnosis of gastric outlet obstruction in

infancy is very difficult without contrast media.Certain plain film signs are suggestive, but notdiagnostic (Riggs and Long, 1971). Althoughgastric pneumatosis is a very rare radiographicmanifestation of pyloric obstruction (e.g. fromhypertrophic pyloric stenosis) it should immediatelylead to the correct diagnosis, provided that sepsisand necrotizing enterocolitis can be excluded,readily accomplished on clinical grounds. Necro-tizing enterocolitis is seen among preterm infants,usually after perinatal stress. There are signs ofsepsis, abdominal distension, decreased or absentbowel sounds, bile-stained vomiting, and oftenbloody stools. In addition, in most cases there isradiographic evidence of generalized bowel dis-tension and intestinal pneumatosis, though occa-sionally necrotizing or ischaemic gastroenterocolitismay present radiographically by isolated gastricpneumatosis, as in Case 2.Depending on the differential diagnosis described

above, infants with suspected gastric outlet obstruc-tion should have a barium upper gastrointestinalstudy to show the exact nature of obstruction.Sepsis and ischaemic gastroenterocolitis should betreated immediately and vigorously as outlined bySantuli et al. (1975).

Speculating on the mechanism of gastric pneuma-tosis gastric outlet obstruction may lead to increasedintraluminal pressure and gas may be forced into thewall of the stomach through superficial mucosaltears (Holgersen et al., 1974). Disappearance ofgastric pneumatosis in our Case 1 within hours aftergastric decompression was in keeping with this.Mucosal tears are likely to occur with excessivevomiting, andindeed 3 infants described byHolgersenet al. (1974) had haematemesis or coffee-groundvomitus. In necrotizing enterocolitis presumably

breakdown of effective mucosal barrier leads toinvasion by gas-forming bacteria, which may thenproduce intramural gas (Stone etal., 1968). Regardingour Case 2, presumably she had emphysematousgastritis, as a form of ischaemic gastroenterocolitisof the newborn.

SummaryGastric pneumatosis (gas in the wall of the sto-

mach) is an uncommon but characteristic plain-filmradiographic sign. In infancy it is associated eitherwith gastric outlet obstruction, usually hypertrophicpyloric stenosis, or necrotizing gastroenterocolitis.Differential diagnosis is mainly based on associatedclinical findings, as exemplified by the 2 casesreported here. Both infants had isolated gastricpneumatosis as the principal radiographic finding.Correct diagnosis was greatly aided by recognitionof this x-ray sign so that appropriate therapy wasstarted without delay.

REFERENCES

Bell, R. S., Graham, C. B., and Stevenson, J. K. (1971). Roent-genologic and clinical manifestations of neonatal necrotizingenterocolitis. American Journal of Roentgenology, RadiumTherapy, and Nuclear Medicine, 112, 123.

Holgersen, L. O., Borns, P. F., and Srouji, M. N. (1974). Isolatedgastric pneumatosis. Journal of Pediatric Surgery, 9, 813.

Riggs, W., and Long, L. (1971). The value of the plain film roent-genogram in pyloric stenosis. American Journal of Roentgeno-logy, Radium Therapy, and Nuclear Medicine, 112, 77.

Robinson, A. E., Grossman, H., and Brumley, G. W. (1974).Pneumatosis intestinalis in the neonate. American Journal ofRoentgenology, Radium Therapy and Nuclear Medicine, 120,333.

Santulli, T. V., Schullinger, J. N., Heird, W. C., Gongaware, R. D.,Wigger, J., Barlow, B., Blanc, W. A., and Berdon, W. E. (1975).Acute necrotizing enterocolitis in infancy: a review of 64 cases.Pediatrics, 55, 376.

Stone, H. H., Allen, W. B., Smith, R. B., and Haynes, C. D. (1968).Infantile pneumatosis intestinalis. Journal of Surgical Research,8, 301.

JOHN C. LEONIDAS*Department of Radiology, Children's Mercy Hospitaland University of Missouri School of Medicine,Kansas City, Missouri, U.S.A.

*Present address: Dept. of Radiology, Boston Floating Hospital,New England Medical Center, 171 Harrison Avenue, Boston,Mass. 02111, U.S.A.