Clin. Radiol. (1979) 30, 25-31.

Strictures and other Late Complications of Neonatal Necrotising Enterocolitis j. p. VIRJEE*, G. J. GILL, D. DESA, S. SOMERS and G. W. STEVENSON From the Department o f Radiology, McMaster University Medical Centre, Hamilton, Ontario, Canada Eighty infants have been treated for neonatal necrotising enterocolitis within a period o f five years. Twentyfive per cent developed strictures o f the smail or large intestine. Four cases of atresia including two with multiple cyst formation were seen. Four patients developed severe malabsorption requiring hyperalimentation with slow recovery of small bowel function in two survivors. The radiological features of these complications is illustrated and the role of radiology in the management of these patients is discussed.

INTRODUCTION Necrotising enterocolitis has been described as a usually fatal (Berdon et al., 1964) or highly lethal (Touloukian et al_, 1967) ischaemic disease of bowel occurring in newborn infants. However, in recent years, the mortality rate has decreased considerably (Stevenson et al., 1969; Torma et al., 1973; Frantz et al., 1975; Bell et al., 1976); the reasons for this seem multifactorial (Santulli et al., 1975). As a result of a higher survival rate, late complications are now seen more frequently. The purpose of this pape r is to consider the strictures and other late complications that have occurred in a series of 80 consecutive cases of necrotising enterocolitis and to indicate the role of radiology in their,management.

MATERIAL AND METHOD Between February 1973 and September 1977, 80 patients with necrotising enterocolitis were treated in the Neonatal Unit of the McMaster University Medical Centre. The clinical and radiological records of these patients have been reviewed. The diagnosis was suggested clinicalJy by abdominal distension, vomiting or increased gastric residue, absent bowel sounds or blood in the stools. Characteristic radiological features included thickened intestinal wall (mucosal oedema or haemorrhage), intramural gas, dilated bowel loops with fluid levels, free intraperitoneal gas, portal vein gas and free intraperitoneal fluid. Pathological confirmation of the diagnosis was obtained at surgery in 23 cases, at autopsy in a further 13 and in the remaining 44 the diagnosis was made on clinical and radiological criteria alone. * Present address: Department of Radiodiagnosis, Bristol Royal Infirmary, Bristol.

Surgical or autopsy cases of suspected ischaemic bowel disease without a previous clinical syndrome compatible with necrotising enterocolotis have not been included in the present analysis.

RESULTS Thirty-three strictures were found in 20 patients (11 males and 9 females). Strictures have been reported previously (Rabinowitz et al., 1968; Krasna et al., 1970; Master et al., 1973; Lloyd and Cywes, 1973; Santulli, 1974; Bell et al., 1976) but in this series of 80 patients a surprisingly high incidence of 25% was encountered. The segments of the gastrointestinal tract involved are shown in Table l. More strictures occurred in the large intestine than in the small intestine. Though most patients had single strictured segments, in eight o f them (40%) there was more than one stricture, the most being five. The length of the stricture varied from 1 cm (Fig. la, b) to one involving most of the transverse, descending and sigmoid colon (Fig. 2). The overall mortality rate in the 80 patie~lts was 17.5% whereas in those who developed strictures the rate was 15%. In 16 of the 20 patients, surgical intervention to relieve the obstruction caused by the Table 1 - Sites involved by strictures in NEC (20/80 patients) Jejunum Ileum Caecum Ascending colon Hepatic flexure Transverse colon Splenic flexure Descending colon Sigmoid colon

2 8 1 4 2 4 4 6 6

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CLINICAL RADIOLOGY

Fig. 1 (a) A stricture 1 cm in length has developed in the upper descending colon and is demonstrated by barium enema. (b) Macroscopic appearances of the excised specimen of the strictured segment shown in Fig. la. -

stricture was necessary. Table 2 indicates in these cases the age at which the diagnosis of necrotising enterocolitis was made, the age at which the stricture was diagnosed and the time interval between them. In the other four patients the strictures progressed to complete obliteration (i.e. atresia) of the lumen. All four of these patients developed atretic segments in that part of the bowel that was distal to a defunctioning ileostomy or colostomy and diagnosis was made on barium examination of the distal segment prior to re-anastomosis. In two cases marked

Table 2 - Time sequence in the d e v e l o p m e n t o f strictures in NEC 60-

50-

40I---36 81 ~ 30-

8

r

ru

tm 20-

. lira

A22

: I

4-13

L

~

10-

g zx NEC

3-31

z~ Stricture

17-52

Interval 10-42

Fig. 2 - Barium enema demonstrates the longest stricture found in this series of patients. It extended from the:rectosigmoid to mid-transverse colon, with variable calibre.

C O M P L I C A T I O N S OF N E O N A T A L N E C R O T I S I N G E N T E R O C O L 1 T I S

27

F i g - 3 - Specimen of resected distal ileum and ascending colon. A thread-like zone of atresia is seen proximal to the caecum, and three other atretic zones are present in the terminal ileum. All are indicated by arrows and the cystic dilatation of the intervening segments of ileum can be seen. (a) and (b) represent opened areas in Fig. 4.

Fig. 5 This child recovered from the acute illness, but thereafter failed to gain weight and required hyperalimentation. Barium follow-through showed dilatation of loops of small bowel with increased width of valvulae conniventes. Histology shown in Fig. 12. Small bowel function recovered during six weeks hyperalimentation.

Fig. 4 - Same specimen as Fig. 3; opened segments (a) and (b) to show preservation of mucosa and mucous contents of (a).

mucous distension o f bowel b e t w e e n atretic segments resulted in the f o r m a t i o n of m u l t i p l e enteric cysts. In one case these cysts (three in n u m b e r ) were in the small intestine (Figs 3, 4) whereas in the other (five) t h e y were in the descending colon. Following recovery f r o m necrotising enterocolitis severe m a l a b s o r p t i o n occurred in four cases. This resulted in a progressive loss in weight w h i c h could o n l y be halted or reversed w i t h hyperalimentation. The radiological appearances on a followthrough e x a m i n a t i o n showed dilated loops and thickened valvulae as shown in Fig. 5. DISCUSSION Until recently the t r e a t m e n t for necrotising enterocolitis has been early surgical intervention with resection of involved bowel. This has been

superseded by an aggresive medical a p p r o a c h which entails t h e steps shown in Table 3 (Santulli, 1974). The McMaster University Medical Centre approach has followed these steps w i t h the e x c e p t i o n o f the use of low molecular weight dextran which was used in o t h e r centres to prevent platelet adherence and therefore the d e v e l o p m e n t o f m i c r o t h r o m b i . The surgical indications are n o w m o r e well defined and are those listed in Table 4 (Santulli et aL, 1975).

Table 3 - Treatment protocol for acute necrotising enterocolitis 1. Nil by mouth 2. Intravenous feeding with electrolyte and acid/base monitoring 3. Nasogastric decompression 4. Broad-spectrum antibiotics parenterally and topically

Table 4 - Surgical indications 1. 2. 3. 4. 5.

Pneumoperitoneum Signs of peritonitis Mass formation and localised perforation Intestinal obstruction Deterioration in spite of medical treatment

28

CLINICAL

RADIOLOGY

Table 5 - Role of radiology 1. 2. 3. 4.

Early diagnosis of the disease process Monitor the course of the disease Early detection of the surgical indications Indicate or demonstrate affected sites which may influence the surgical approach 5. Demonstrate late complications 6. Following apparent complete recovery, to demonstrate any residual bowel abnormalities which would influence future management

Fig. 6 - A horizontal beam supine lateral film shows large and small bowel dilatation with mutliple fluid levels. This child with clinical and radiological evidence of necrotising enterocolitis for 24 days, developed vomiting and renewed distension. Surgery revealed that this obstruction was due to a stricture of descending colon presenting acutely.

Fig. 8 - Barium enema in an infant with necrotising enterocolitis who developed a left iliac fossa mass and proximal colonic distension. At the distal margin of the stricture a little contrast is seen leaking into an abscess cavity.

Fig. 7 - Subacute presentation of stricture. Good clinical recovery from necrotising enterocolitis, but delay in resolution of large and small bowel distension. Barium enema reveals a stricture at the junction of sigmoid and descending colon.

The role of radiology in necrotising enterocolitis is summarised in Table 5. With particular reference to strictures the role is two-fold. First, in acute cases there are signs of o b s t r u c t i o n as in Fig. 6 where multiple g a s - f l u i d levels are seen t h r o u g h o u t the colon but n o t in the sigmoid colon or rectum. Second, in the subacute cases the length o f the involved segment m a y be d e m o n s t r a t e d prior to surgery, aiding in planning the surgical approach (Fig. 7). Occasionally, unsuspected complications such as an abscess m a y also be d e m o n s t r a t e d (Fig. 8). We have used double and single contrast barium e n e m a techniques to study the c o l o n in these cases and have not f o u n d one to have any advantage over the other. F o l l o w i n g emergency e n t e r o s t o m y and prior to elective re-anastomosis, a barium study o f the distal d e f u n c t i o n e d segment o f the gastrointestinal tract, must be u n d e r t a k e n and this m a y well show develop, merit o f stricture or atresia. Fig. 9 is one such example where the barium enema showed arrest of the column

COMPLICATIONS OF NEONATAL NECROTISING ENTEROCOLITIS

29

Fig. 11 - Barium enema prior to closure of enterostomy disclosed complete arrest of the barium column, by acquired atresia, close to the mucous fistula (see text). Fig. 9 Prior to closure of the enterostomy, barium enema was performed to confirm recovery and adequacy of the defunctioned colon. Barium arrested as shown, and introduction of the lumen by partly calcified scar tissue (Fig. 10). of barium into the mucous fistula demonstrates the length of The possibilities that should be considered radio° the atretic segment at the splenic flexure (4 cm). See Fig. logically in this situation are (a) a stenotic segment 10 for histology. that has progressed to complete obliteration of the lumen, (b) enteric cyst formation, and (c) an associof contrast just distal to the splenic flexure. Barium ated abscess with intense spasm. This last possibility was then introduced via the mucous fistula and has not occurred in this series and only one case arrested proximal to the spenic flexure. Histology of report each of atresia (Chiba et aL, 1975) and enterothe excised specimen shows complete obliteration cyst formation (Lloyd and Cywes, 1973) has been reported previously. In one case (Fig. 11) complete obliteration of the lumen was demonstrated on barium enema 2cm from the mucous fistula, The fact that this segment of the bowel was mobilised to form the mucous fistula and that the obliterated segment is so near the skin surface suggested the possibility that surgical intervention may have further compromised the ischaemic state and hence resulted in obliteration of the lumen. Cases of malabsorption have been alluded to (Santulli, 1974) and predicted (Lloyd and Cywes, 1973) in the literature. The possible causes following necrotising enterocolitis are listed in Table 6. In the Table 6 - Causes of malabsorption fonowing NEC Fig. 10 Transversesection through the atretic bowel segment (Fig. 9) shows complete obliteration of the lumen by scar tissue. In addition to the fibrous tissue there are aggregates of inflammatory cells and calcified debris. The circular muscle (C) is relatively well preserved (H & E, × 400).

1. 2. 3. 4. 5.

Short gut syndrome Stricture causing stasis with bacterial overgrowth Failure of functional recovery Fistula Unrelated coexisting small bowel disease

30

CLINICAL RADIOLOGY

Fig. 12 - Histological section through macroscopically normal small bowel showing a large area of mucosa with ablated villi (between arrows). This area is bordered on each side by a zone of small bowel mucosa where the villi are oedematous and of irregular height and contour (H & E, x 400).

f o u r patients who developed this complication none had an extensive resection of bowel adequate to account for the malabsorption state. A small bowel follow-through examination (Fig. 5) on these patients showed no strictures, stasis or evidence of an internal fistula. Exhaustive investigation did not reveal any other cause for their severe malabsorption and the patients lost weight unless hyperalimentation was instituted. Fig.' 12 is the histology from a segment of apparently normal intestine taken at operation using a Crosby capsule. The section shows that there

is an abnormal villous pattern with severe loss of villi. The epithelium of the small intestine regenerates very rapidly and Fig. 13 is a section showing regenerated epithelium, but the striking abnormality is that morphologically these villi are atypical. Of these four cases two died and at autopsy no other cause for malabsorption was found apart from the effects o f previous necrotising enterocolitis. The remaining two patients, during extensive periods on hyperalimentation (six weeks and four m o n t h s respectively), steadily recovered their small bowel function and both are now progressing normally. It appears from these latter two cases, that following necrotising enterocolitis enough of the small intestine may be affected to produce at least a temporary malabsorption state which will persist until functional recovery of the affected bowel occurs. Internal fistula formation between diseased segments is a recognised possible complication, two such cases being described in the literature (Firor, 1970; Beck et aL, 1971). In this series no case of intestinal fistulae occurred. There was one case of external fistula formation following surgery. A further role of radiology is routine examination of the alimentary tract following apparent complete recovery from necrotising enterocolitis. This is to document any abnormalities such as asymptomatic stricture (one c a s e - n o t included in the present series) which would make the clinician aware o f the condition and could influence future management. Acknowledgement.- It is a pleasure to thank Dr G. Cameron for his enthusiastic advice and encouragement..

REFERENCES

Fig. 1 3 - Segment of small bowel showing loss of villous differentiation with oedema of the lamina propria and fusion of adjacent villi, excess inflammatory infiltration and a smaJrl ' crypt abscess (arrow) (Trichrome stain, X800).

Beck, J. M., Dinner, M. & Chappel, J. (1971). Enterocolitis foUowing exchange transfusion. South African Journal of Surgery, 9, 39-42. Bell, M. J. Ternberg, J. L. Askin, F. B., McAlister, W. & Shackelford, G. (1976). Intestinal stricture in necrorising enterocolitis. Journal of Paediatric Surgery, 11, 319-327. Berdon, W. E., Grossman, H., Baker, D. H., Mizrahi, A., Barlow, O. & Blanc, W. A. (1964). Neerotising enterocolitis in the premature infant. Radiology, 83,879-887. Chiba, T., Watanabe, I. & Kasai, M. (1975) Colonic atresia following necrotising enterocolitis. Journal o f Paediatric Surgery, 10, 965-966. Firor, H. V. (1970)_ Gastrojejunocolie fistula in an infant. Journal of Paediatric Surgery, 5,450-453. Frantz, I. D., III, L'Heureux, P., Engel, R. R. & Hunt, C. E. (1975). Necrotising enterocolitis. Journal of Paediatrics, 86,259-263. Lloyd, D. A. & Cywes, S. (1973). Intestinal stenosis and enterocyst formation as late complications of neonatal necrotising enterocolitis. Journal of Paediatric S~rgery, 8,479-486.

COMPLICATIONS OF NEONATAL NECROTISING ENTEROCOLITIS Krasna, I. H., Becket, J. M., Schneider, K. M. & Beck, A. R. (1970). Colonic stenosis following necrotising enterocolitis. Journal o f Paediatric Surgery, 5 , 2 0 0 - 2 0 6 . Master, S. P., Truscott, D. E., Templeton, A. C. & Middlemiss, J. H. (1973). Neonatal necrotising enterocolitis. British Journal o f Radiology, 46, 1063-1069. Rabinowitz, J- G., Wolf, B. S., Feller, M. R_ & Krasna, I. (1968). Colonic changes following necrotising enterocolitis in the newborn. American Journal o f Roentgenology, 1 0 3 , 3 5 9 - 3 6 4 . Santulli, T. V. (1974). Acute necrotising enterocolitis: recognition and management. Hospital Practice, 33, 129-135Santulli, T. V., SchuUinger, J. N., Heird, W. C., Gongavare, R. D., Wigger, J., Barlow,B_, Blanc, W. A. & Berdon,

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W. E. (1975). Acute necrotising enterocolitis in infancy,

Paediatrics, 5 5 , 3 7 6 - 3 8 7 . Stevenson, J. K., Graham, G. B., Oliver, T_ K., & Goldenbu~g, V. E. (1969). Neonatal necrotising enterocolitis.American Journal of Surgery, 118,260-272. Torma, M. J., Delemos, R. A., Rogers, J. R. & Diserens, H. M. (1973). Necrotising enterocolitis in infants. American Journal of Surgery, 126,758 761. Touloukian, R. J., Berdon, W. E., Amoury, R. A. & Santulli, T. V. (1967). Surgical experience with necrotising enterocolitis in infants. Journal of Paediatric Surgery, 2, 389 401. Wilson, S. E. & WoUey, M. M. (1969). Primary necrotising enterocolitis in infants. Archives of Surgery, 99, 563-566.

Strictures and other late complications of neonatal necrotising enterocolitis.

Clin. Radiol. (1979) 30, 25-31. Strictures and other Late Complications of Neonatal Necrotising Enterocolitis j. p. VIRJEE*, G. J. GILL, D. DESA, S...
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