Diagnostic Aspects of Neonatal Ascites: Report of 27 Cases

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1 Diagnostic Aspects of Neonatal Ascites: Report of 27 Cases N. THORNE GRISCOM, ARNOLD H. COLODNY,2 HENRIETTA K. ROSENBERG, CHRISTIAN P. FLIEGEL, BRIAN E. HARDY2 Review of 27 cases of massive ascites found in the first month of life. usually immediately after delivery. revealed a broad spectrum of causes. Seven newborns had urinary ascites. five had bowel disease. three had cardiac arrhythmias, two had liver disease. and one each had toxoplasmosis, ovarian cyst, and chylous ascites. In seven cases the cause was never established. Physical examination. a planned sequence of radiologic examinations, and paracentesis for fluid analysis when necessary are the major steps in differential diagnosis. A systematic approach to the problem is proposed. The discovery of ascites in a newborn startles obstetrician, pediatrician. radiologist, and surgeon alike. The causes are many, although nearly all reports on the subject are based on only one or two cases. This review of 27 live-born neonates with massive ascites suggests the approximate frequency and characteristics of the various causes. A systematic diagnostic approach to a newborn in whom ascites is suspected is proposed. Case Material and Findings The salient features of the 27 cases are presented in table 1. The cases are divided into urinary gastrointestinal [9, 101. cardiac 11 1, 121. hepatic systemic infectious , chylous , and ovarian [2] categories, plus a group of cases of indeterminate cause [26]. The infants were referred for care to Children s Hospital Medical Center. (24 patients), were born at the Boston Hospital for Women (10 patients), or both. Abdominal distension was discovered at birth in 24 cases, and later in the first month in the three others. The two newborns with posterior urethral valves were boys, but four of the other five cases of urinary ascites occurred in girls. The children with cardiac and hepatic ascites were all boys. Five of the seven cases of ascites of unknown cause occurred in girls. Except in the child with familial cirrhosis, the family histories were not helpful. The five examples of gastrointestinal ascites were selected from a far larger group of newborns with peritonitis due to clear-cut or suspected perforation of the bowel, mostly with obstruction [9, 271. In these five, however, the initial presentation was that of ascites without good preoperative clinical or radiologic evidence of obstruction (figs. 1 and 2); thus they are included as part of the spectrum of neonatal ascites. None had a pneumoperitoneum, distinct gaseous distension of bowel, or an intestinal or peritoneal pseudocyst [271. Seven of these 27 infants had some degree of edema of the extremities or abdominal wall, including two of the three cases of cardiogenic ascites. In each case ascites was the dominant finding, and in no case was it clear on presentation that the ascites was of circulatory etiology; these cases are therefore also included in the description of neonatal ascites. Infants with erythroblastosis fetalis and other types of hydrops fetalis 1281 were excluded. The peritoneal fluid was readily displayed by radiographs. In eight of the 23 patients with available plain films of the abdomen, the liver was visible (at least in retrospect) within the slightly more lucent abdominal fluid, and this excellent sign of ascites was questionably present on the plain films of three others (fig. 3) 129, 531. Thin, clear ascitic fluid tended to be lucent by x-ray, whereas the cloudy or bloody collections were not. Our fragmentary data do not show a correlation between lucency and low specific gravity; however, it is evident that lucency of peritoneal fluid is far from pathognomonic of chylous ascites Most of the cases showed the other plain film signs of ascites 130, 31 1, often with striking clarity (figs. 1-3). During the total body opacification phase of intravenous urography 1321, the fluid contrasted with the denser liver in 13 of 17 cases (figs. 4-6). Although the presence of ascites was thus easily confirmed, finding its cause was often more difficult. Cystography and intravenous urography were quite useful in defining urinary ascites and its pathogenesis (figs. 7-10). Two children showed contrast leakage from one or both kidneys (figs. 8 and 9) [341, and one girl displayed leakage through a bladder perforation (fig. 10) 15, Total body opacification showed the avascular nature of the ovarian cyst (fig. 6). Chest films demonstrated cardiomegaly in all three cases of cardiogenic ascites but also in three of the seven cases of ascites of unknown cause. Other imaging techniques were not used systematically. A barium enema easily showed the one instance of malrotation. In another patient, a peritoneogram (fig. 7A) provided excellent demonstration of the ascites but otherwise advanced the diagnostic process only slightly. Cardiac catheterization and angiocardiography were used to show the disordered physiology in two of the three infants with arrhythmias. An aortogram via the umbilical artery 1371 Received November 17, 1976; accepted after revision February Department of Radiology. Children s Hospital Medical Center, Boston, Massachusetts Address reprint requests to N T. Griscom 2 Department of Surgery. Children s Hospital Medical Center, Boston, Massachusetts Present address: Department of Radiology. Children s Hospital of Philadelphia. Philadelphia, Pennsylvania Present address: Department of Radiology. Children s Hospital. CH-4000 Basel 5. Switzerland Am J Roentgeno/ 128: June

2 962 GRISCOM ET AL. TABLE 1 Summary of Cases Differences of Peritoneal Fluid from Category and Case No. Delivery vomiting Edema Mass Tissue Fluid #{149} Cause of Ascites Follow.up Data Urinary: 1 (fig.7) Dystocia 2(fig.8) 3 Oligohydramnios; dystocia 4(fig. 10) 5 7(fig.5) Gastrointestinal: (fig.9) 9(fig. 1) 11 12(fig.2) Cardiac: 13(fig.3) Polyhydramnios Polyhydramnios 14 Polyhydramnios 15 Hepatic: (fig.4) Systemic 18 Chylous: 19 Ovarian: 20 (fig. 6) infectious: - - Enlarged kidneys and bladder Enlarged bladder ?Central Brown abdominal mass - - Hepato- Serosanguinous and splenomegaly Polyhydramnios, dystocia Indeterminate: Dystocia Bowel contents Ileal atresia, perforation - - Thin meconium Ileal perforation,?cause Dark brown Cloudy yellow - ++ Hepatomegaly - ++ Hepatomegaly Hepatoand splenomegaly Milky - Left upper quadrant mass - Posterior urethral valves - Posterior urethral valves - Urethral, anal atresia, perforated bladder - Bladder perforation,?cause - Bladder perforation,?cause -.. Neurogenic bladder, myelocele, reflux - Reflux,?neurogenic bladder Ileal volvulus, perforation ?Infectious,??rubella Serosanguinous?Infectious Ileal duplication, perforation Well, age 26 months Malrotation, volvulus, ischemia Well, age 8 - Paroxysmal supraventricular tachycardia. Wolff-Parkinson-White phenomenon -.. Paroxysmal supraventricular tachycardia -.. Paroxysmal supraventricular tachycardia - Familial cirrhosis - Probable hepatitis Toxoplasmosis Lymphatic leak, base of mesentery Light brown Ovarian cyst, torsion, local rupture?infectious Doing well, age 6 Renal failure, age 1 3 Died Well, age 10 Died at 7 days Died at 3 months Microcephaly, age 8 Died at 10 weeks Well, age 4 Cystic fibrosis; died at 9 days Negative laparotomy; well, age 1 5 months Well, age 3 Well, age 1 Died at 8 days; small liver Borderline liver tests, age 7 Died at 1 days Well, age 11 months Well, age S Squint, retarded, age 4 Nonspecific autopsy at 4 weeks Ascites disappeared; tuberous sclerosis. age 3

3 NEONATAL ASCITES 963 TABLE 1 (Continued) Category and Case No Delivery Vomitin g Edema Mass Differences Peritoneal Fluid Tissue Fluid of from #{149} Cause of Ascites Follow-up Data 24 Polyhydramnios Unknown Nonspecific autopsy at 1 day Unknown Nonspecific autopsy at 1 day 26 Dystocia Unknown Ascites disappeared; well, age 2 months Unknown Ascites disappeared; well, age 4 Note.-Ascites was found at birth in all cases except from the authors. Case 3 [1]; case 20 [2] Minus sign indicates no differences found k... :Fg. 1..T-: Case. Upright anteroposteriof age showing no indication of intestinal obstruction. Plaques and lumps of peritoneal calcification are present (arrows). Laparotomy showed large quantity of thin green peritoneal fluid, collapse of small bowel, and ileal perforation of unknown cause, leading to meconium peritonitis. Cystic fibrosis was never tested for, but child was well at age 4. and 12 where it was found at 23. 7, and 4 days. respectively. Further details on all cases available Fig. 2.-Case 12. Upright anteroposterior film of abdomen in 4-dayold child with abdominal distension and a few hours of vomiting. Film suggests that bowel, though abnormal, does not account for abdominal dis tension and that ascites is present. Malrotation of midgut. a 7200 volvulus. ischemia of bowel without perforation or appreciable dilatation, and large quantity of cloudy yellow culturenegative fluid were found at laparotomy. showed a small liver and copious ascites in the child with intestines in the case of hepatitis. cirrhosis, but ultrasonography would probably have been However, radiologic techniques often gave only a a better method. Ultrasound was useful in excluding a partial answer to the question of etiology. Most of the mesenteric cyst or some other gross lymphatic abnor- infants had some type of chemical, bacteriologic, or cytomality in the child with chylous ascites and would probably logic examination of the ascitic fluid, sometimes quite have been informative in other cases. A 131l,rose bengal extensive. Table 1 lists only those characteristics of diagliver scan showed failure of passage of bile into the nostic usefulness. The most worthwhile determinations

4 964 GRISCOM ET AL. Fig. 3 -Case 13. Plain film of abdomen showing contrast of liver (arrows) with ascitic fluid Gas-containing bowel has risen to center of distended abdomen Child had episodes of supraventricular tachycardia in first few weeks of life, ascites attributed to intrauterine paroxysmal supraventricular tachycardia Fig 5 -Case 7 Anteroposterior film of abdomen several minutes after injection for urography. Right lobe of liver (arrows) contrasts with.,:-. peritoneal fluid by total body opacification effect as well as by its inherent radiodensity.; Fig 4 -Case 17. Recumbent anterioposterior view of abdomen on first day of life 10 mm after intravenous injection of 15 ml Renografin 60. Arrows mark interface between liver. opacified by circulating iodine Liver biopsy suggested hepatitis..41c -. Fig. 6.-Case 20. Left posterior oblique film of abdomen at several hours of age 2 mm after intravenous injection for urography, 400 ml of peritoneal fluid already removed. Open arrows indicate left lobe of liver, opaque against avascular ascitic fluid Laparotomy showed more fluid (50 ml) and twisted left ovarian cyst (giant corpus luteum, closed arrows). Lucency in center of mass indicates avascularity: specimen showed central hemorrhage and necrosis and apparently had ruptured locally Other films published in [2]

5 NEONATAL ASCITES 965 F I I, C.. were the gross appearance of the fluid and the presence or absence of white and red blood cells. In the four children with intestinal perforation (fig. 1), the peritoneal fluid was highly abnormal and quite unlike tissue fluid. As the bleeding disorder of the girl with toxoplasmosis became more severe, the fluid became bloody. The fluid of the child with chylous ascites became milky after feedings were begun. However, peritoneal fluid tends to reach obstruction. Our series also fails to show the expected predominance of posterior urethral valves among causes of urinary ascites. Only two of the seven cases of urinary ascites were shown to be caused by valves. Furthermore, these two children were the only two cases of ascites among 27 neonates with hydronephrosis due to valves seen at this hospital Although ureteropelvic obstruction appears to be the leading cause of neonatal hydronephrosis no case of, I

6 966 GRISCOM ET AL. urinary ascites due to this type of obstruction occurred in our series or was found in an incomplete review of the literature. The obstructions leading to ascites all occurred in the urethra, bladder, or distal ureter, even though the actual leak may have been in the kidney (figs. 8 and 9). In seven of the 27 newborns the cause of the ascites was never established despite many diagnostic tests, nor did we know which organ system was at fault. In the three fatal cases, autopsies were unrevealing; in the other four the ascites spontaneously disappeared. Perhaps in some the ascites was caused by systemic infection present at birth, but this was not verified by culture, serologic studies, or convincing findings at autopsy. One of the four survivors is mentally slow and may have had congenital rubella; another recovered completely from her abdominal problems but was later found to have tuberous sclerosis, presumably an unrelated illness. The third and fourth seemed quite well at the age of 2 months and 4 years, respectively. Two other cases of spontaneously remitting benign neonatal ascites of unknown cause have recently been reported [26]. Presumably these 27 cases are reasonably representative of ascites as it is found in live-born infants on active delivery and newborn referral services. Causes of neonatal ascites not found in the present series are listed in table 2. A thorough review of the related subject of hydrops

7 TABLE 2 Causes of Neonatal Ascites Not Found in This Series 1 Fig Case 6 Cystogram of girl born with myelomeningocele (small arrow) and ascites. Intravenous urography performed earlier in day showed bilateral hydronephrosis and passage of contrast material into right subcapsular space. Note bilateral reflux (worse on left) nd passage of contrast material into right s.bcapsular space (large arrows) Artifact above left kidney is opaque pact protecting myelomeningocele. which caused hydrocephalus and death at 3 months of age - _ - I Cause Reference Urinary: Ureterocele [3, 42] Bladder neck obstruction [6] Urethral stricture [3. 34] Pelvic neuroblastoma, bladder perforation [6. 36] Congenital nephrosis [43] Ureteric stenosis [44] Renal vein thrombosis [ ]t Gastrointestinal: Appendicitis, perforation [10] Perforation of Meckel s diverticulum [45] Imperforate anus [46] Cardiac and circulatory: Hypoplastic left heart syndrome [12] Hypoproteinemia [4O]t Many other cardiac and circulatory diseases, #{149} especially including erythroblastosis fetalis [ ]t Portohepatic: Polycystic disease of the liver [14] Meckel-Gruber syndrome, liver cysts [47] Biliary atresia [48] Common duct perforation [49] Portal vein obstruction, hypoplasia [4O.t 48.t SO] Galactosemia Systemic infectious: [4O]t Cytomegalovirus infection [21] Syphilis [17] Sepsis [3] Leptospirosis Chagas s disease [28]t Chylous: Constricting peritoneal band [23] Occlusion or stenosis of thoracic duct [22]t Genital: Hydrometrocolpos, imperforate anus [SO] Others. some of indeterminate cause [1 2, 28]t #{149} Coexisting with hydrops fetalis. t Listings only fetalis (fluid accumulation throughout the tissues and body cavities of a fetus or newborn infant) is given by Driscoll [28]. A diagnostic approach to a newborn suspected of having ascites is outlined in figure 1 1. Plain films will usually distinguish ascites from ordinary intestinal obstruction and from undetected tumors. If the plain films indicate ascites and the child is critically ill, paracentesis should probably be performed next rather than cystography and intravenous urography, both for relief of respiratory cornpromise and for diagnosis. If the peritoneal fluid resembles intestinal contents, laparotomy for repair of bowel perforation is urgently necessary. If the fluid is hemorrhagic. consideration should be given to rupture of a solid viscus [51, 521, to toxoplasmosis or some other hemorrhagic disorder, or to rupture of an ovarian cyst. If the fluid resembles serum, cystography and intravenous urography should be performed. [28]t

8 968 GRISCOM ET AL. NEWBORN, ABDOMINAL DISTENSION, NO PALPABLE MASS, NOT VOMiTING, DISTENSION MORE IMPRESSWE THAN ANY FLUID ACCUMULATION ELSEWHERE recumbent AP, upright AP, and lateral films of abdomen gaseous dilatation of bowel or solid mass, shown by tumor distension accounted for by neither pneumoperitoneum (intestinal calcification or bowel of those, apparently representing obstruction or perforation) displacement ascites yes laparotomy, for bowel perforation consider ovarian cyst, ruptured solid viscus, hemorrhagic disorder as in toxoplasmosis peritoneal /\ calcification? yes no / meconium peritonitis cystography intravenous sweat test normal urinary tract abnormal electrocardiogram, cardiomegaly by chest film, or both no abnormal (urinary appropriate urography consider systemic infectious and paracentesis for fluid inspection, hematocrit, white cell count, presence cardiac causes of ascites of chyle if baby has been given milk, culture fluid resembles bowel contents hemorrhagic but not chylous resembles serum resembling bowel contents A few differential diagnostic points follow which are not conveniently presented in flow chart form. (1 ) If a large bladder or a myelomeningocele is present, the ascites is probably urinary. (2) If vomiting occurs or if bowel loops are abnormal even though not distended, gastrointestinal causes are likely. (3) If there is impressive peripheral or body wall edema, consider infection, heart disease, liver disease, erythroblastosis, and other causes of hydrops fetalis [281. (4) If hepatomegaly is present, infectious, cardiac, and hepatic causes of ascites become more likely. Ultrasonography has yet to define its place in this diagnostic scheme; presumably it will be found very useful. Circumstances will frequently demand modification of the approach suggested in figure 1 1. However, passage of the data from the present series through the proposed system indicates that it is a reasonably efficient diagnostic plan. The use of such an approach will occasionally prevent needless laparotomy. ACKNOWLEDGMENTS We thank Drs. Shirley Driscoll, John Kirkpatrick, Robert Lebowitz, and Michael Oxman for comments on this report and Ms. Andrea Kelton for superlative secretarial assistance. Fig Flow chart illustrating proposed diagnostic approach. ascites) surgery barium studies, liver function tests; ultrasound for acute phase titers; mesenteric cyst, review entire situation; lymphangioma, consider hepatogenic malrotation; ascites, infectious consider ascttes, benign ascites exploration REFERENCES 1. Syrnonds DA, Driscoll SG: Massive fetal ascites, urethral atresia, and cytomegalic inclusion disease. Am J Dis Child 127: , Carlson DH, Griscom NT: Ovarian cysts in the newborn. Am J Roentgenol 1 16: , Cremin BJ: Urinary ascites and obstructive uropathy. Br J Radiol48: , Dockray KT: Preferred treatment for urinary ascites in newborns. J Urol 1 1 2: Howat JM: Urinary ascites complicating spina bifida. Arch Dis Child46:1O3-1OS, Mann CM, Leape LL, Holder TM: Neonatal urinary ascites: a report of two cases of unusual etiology and a review of the literature. J Urol : , McDonald J. Murphy AV: Neonatal ascites from spontaneous rupture of the bladder. Arch Dis Child 50: , 197S 8. Parker AM: Neonatal urinary ascites: a potentially favorable sign in bladder outlet obstruction. Urology 3: , Birtch AG, Coran AG, Gross RE: Neonatal peritonitis. Surgery 61 : , Nilforoushan MA: Fever and ascites in a newborn. Cl/n

9 NEONATAL ASCITES 969 Ped/atr(Phila) 14:878, Cowan RH, Waldo AL, Harris HB, Cassady G. Brans YW: Neonatal paroxysmal supraventricular tachycardia with hydrops. Ped/atr/cs 55: , 197S 12. Leake RD. Strimling B, Emmanouilides GC: Intrauterine cardiac failure with hydrops fetalis. Clln Pediatr (Phila) 12: , Bellin LB. Bailit 1W: Congenital cirrhosis of the liver associated with infectious hepatitis of pregnancy. J Ped/atr 40:60-63, Ehrlich JC, Goodfriend MJ, Shinohara Y, Seki M: Fetal ascites and portal dysplasia of the liver (polycystic disease without cysts). Ped/atrics 33: , Fletcher CB, Eakin EL, Rothman PE: Fetal ascites-liver giant-cell transformation. Am J D/s Child 108: , Thaler MM: Fatal neonatal cirrhosis, entity or end result? Pediatrics 33: , Aziz EM: Fetal ascites secondary to congenital syphilis. South MedJ67:81-84, Bain AD, Bowie JH, Flint WF, Beverley JKA, Beattie CP: Congenital toxoplasmosis simulating hemolytic disease of the newborn. J Obstet Gynaecol Br Commonw 63: Couvreur J, Desmonts G: Congenital and maternal toxoplasmosis, a review of 300 congenital cases. Dev Med Child Neurol4: , Embil JA, Krause VW, Haldane EV, Easterbrook KB, Crosby JM: Congenital cytomegalovirus infection: clinical, pathological, and virological studies of two fatal cases. Can Med AssocJ 101: , Frank DJ, DeVaux WD, Perkins JR. Perrin EV: Fetal ascites and cytomegalic inclusion disease. Am J Dis Child 112: , Gribetz D, Kanof A: Chylous ascites in infancy. Pediatrics 7: , GrossJl, Goldenberg ye, Humphreys EM: Venous remnants producing neonatal chylous ascites. Pediatrics 27: , Sanchez RE, Mahour GH, Brennan LP, Woolley MM: Chylous ascites in children. Surgery 69: , Wyatt GM, Gross RE: Chylous ascites: roentgenological observations from a case in infancy. Am J Roentgenol 45: , Bryan EM: Benign fetal ascites associated with maternal hydramnios. Cl/n Pediatr (Phila) 14:88-91, 197S 27. Effmann EL, Griscom NT, Colodny AH, Vawter G: Pseudocysts of bowel and peritoneum associated with neonatal intestinal obstruction. In preparation 28. Driscoll SG: Current concepts: hydrops fetalis. N Engl J Med 275: , Proto AV, Lane EJ: Visualization of differences in softtissue densities: the liver in ascites. Radiology 121:19-23, Franken EA Jr: Ascites in infants and children, roentgen diagnosis. Radiology 102: , Proto AV, Lane EJ. Marangola JP: A new concept of ascitic fluid distribution. Am J Roentgenol 126: , Martin DJ, Griscom NT, Neuhauser EBD: A further look at the total body opacification effect. Br J Radiol 4:18-192, Griscom NT, Cochran WC, Harris GBC. Easterday CL, Umansky I, Frigoletto FD: The processus vaginalis of the third trimester fetus. Radiology 96: , Moncada R, Wang JJ, Love L. Bush I: Neonatal ascites associated with urinary outlet obstruction (urine ascites). Radiology 90: , Leonidas JC, Leiter E, Gribetz D: Congenital urinary tract obstruction presenting with ascites at birth. Radiology 96: Weller MH. Miller K: Unusual aspects of urine ascites. Radiology 109: , Lindstrom A, Gyepes MT: Aortography through the umbilical artery in the newborn, Angiography in Infants and Children, edited by Gyepes MT. New York. Grune & Stratton, 1974, pp Winestock D, Macpherson RI: Neonatal ascites, a case for diagnosis. J Can Assoc Radiol 22: , Cywes S. Wynne JM, Louw JH: Urinary ascites in the newborn, with a report of two cases. J Pediatr Surg 3: , Mustard WT. Aavitch MM, Snyder WH, Welch KJ, Benson CD: Pediatric Surgery, 2d ed. Chicago. Year Book Medical Publishers, Lebowitz AL. Griscom NT: Neonatal hydronephrosis. 146 cases. Radiol Clin North Am. In press, North AF Jr. Eldridge DM. Talpey WB: Abdominal distention at birth due to ascites associated with obstructive uropathy. AmJDis Child 111: , Hallman N, Norio A, Rapola J: Congenital nephrotic syndrome. Nephron 11: , Linde NC: Neonatal ascites and urinary tract obstruction. Acta Paediatr Scand 55: , Abt IA: Fetal peritonitis. Med Cl/n North Am 15: , Clayton JW, Ford AM: A case of foetal ascites. MedJ Aust 1:537, Dorland WAN: Watery accumulations in the fetal abdomen obstructing labor. Am J Obstet 79: Baghdassarian OM, Koehler PR, Schultze G: Massive neonatal ascites. Radiology 76: , Aavitch MM: Neonatal ascites. Med Times 96: , Fordyce W: Intrauterine ascites. its obstetrical significance and pathology. Teratologia 1 : Cywes S. Cremin BJ: Roentgenologic features of hemoperitoneum in the newborn. Am J Roentgenol 106: , Marquis JR. Sun SC, Verasestakul S: Rupture of the spleen in a newborn infant. Radiology 119: , Love L, Demos TC, Aeynes CJ, Williams V. Shkolnik A, Gandhi V. Zerofos N: Visualization of the lateral edge of the liver in ascites. Radiology 122: , 1977