P r i m a r y V o l v u l u s o f the S m a l l I n t e s t i n e in I n f a n t s Tetsuaki KURASHIGEand Shiro MATSUYAMA A B S T R A C T : Primary small bowel volvulus which no definite cause can be detected surgically is rare. In this paper, we present five cases of primary small bowel volvulus and discuss the clinical features including etiology. KEY W O R D S : infant and neonate, intestinal obstruction, malrotation, small intestine, volvulus. INTRODUCTION
V o l v u l u s is found in various parts of the gastrointestinal tract even in infants and children. However, most of them are midgut volvulus associated with malrotation of the intestine. Localized small bowel volvulus is also seen with certain organic lesions, such as vitelline duct remnant, 1,15 intestinal polyp, TM mesenteric cyst, TM etc. There are only a small number of reported cases of primary small bowel volvulus. Such volvuli of the intestine are what some authors would call "volvulus without malrotation. ''2,7,14,19,21 We reviewed those case reports and found that the implicated intestines involved almost the entire small bowel in the majority of the reports. During nine years we experienced five cases of primary volvulus of the small intestine (Table 1). Among these five cases a localized loop of the small bowel was involved in four cases and in one case almost entire small bowel was twisted. Namely, four cases were localized small bowel volvulus and one case might be a so called "volvulus without malrotation." CASE REPORTS
Case 1. K.M., a male was born at 36 weeks of gestation weighing 3,270 g. with a maternal history of hydramnios. He was admitted at three days of age to our institution with gradually developing abdominal distention and bilious vomiting starting 24 hours after birth, and passed a small amount of bloody stool per rectum immediately after admission. A scout film showed the distended stomach and relatively long fluid levels. Contrast enema revealed normal colon, but the terminal segment of ileum showed a circular configuration (Fig. 1). At laparotomy a gangrenous localized volvulus of the distal ileum measuring 20 cm in length was found. The gangrenous segment was resected and an end-to-end anastomosis was performed. Any causative factors could not be found. The postoperative course was uneventful and the patient has been well for four years. On microscopic examination, the specimen showed partial loss of mucosa, marked capillary dilatations and severe inflammatory changes. Regenerated epithelia with atypia were also found, suggesting that the occurrence of the volvulus was probably prior to delivery (Fig. 2). From the Department of Surgery I, Gumma UniversitySchool of Medicine (Director: Prof. Takuji Nakamura), Maebashi, Japan. JAPANESEJOURNAL OF SURGERY, VOL. 8, No. 3, pp 228--235, 1978
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T a b l e 1. Case No. 1.
229
Primary Small Bowel Volvulus in Infants
Age (day) 3
Cases of small bowel volvulus in infants (1967-1976)
Sex
Birth weight (g.)
M
3,270
Melena
Site & length (era)
Perforation
Result
Postop. ileus
(+)
ileum
( -- )
alive
(--)
(+ )
died
(+ )
alive
(+ )
(--)
alive
(+)
( -- )*
alive
(+ )
(20) 2.
3
F
3,180
(4-)
jejunum
(4o) 3.
4
M
3,500
(§ )
4.
55
M
3,160
(--)
almost entire ileum
(20) 5.
3
M
3,500
( -- )
ileum
(65) * almost perforated
Fig. 1.
Contrast enema of case 1. Distal ileum shows circular configurations.
Case 2. H.S., a female w e i g h i n g 3,180 g. was b o r n at t e r m b y a m o t h e r with polyh y d r a m n i o s . V o m i t i n g b e g a n a t nine hours o f age a n d g r a d u a l l y the vomitus t u r n e d bilious. A p l a i n film o f the a b d o m e n r e v e a l e d a m o d e r a t e l y d i s t e n d e d s t o m a c h a n d a few relatively long fluid levels in the left u p p e r a b d o m e n . C o n t r a s t e n e m a was i n t e r p r e t e d as n o r m a l . She was transferred to o u r institution three days later. O n admission the b a b y was lethargic a n d her a b d o m e n was d i s t e n d e d r e m a r k a b l y . O n e x a m i n a t i o n there were evidences o f severe d e h y d r a t i o n a n d m a r k e d j a u n d i c e . C o f f e e - g r o u n d m a t e r i a l was a s p i r a t e d via a nasogastric tube. L a p a r o t o m y was done on the d a y o f admission a n d j e j u n a l volvulus was found. T h e l o c a t i o n was a p p r o x i m a t e l y 10 c m distal to the l i g a m e n t o f Treitz. T h e length o f the twisted intestine e x t e n d e d a b o u t 40 cm, a n d was gangrenous a n d perforated. T h e r e was no definite a n a t o m i c a l findings to e x p l a i n the cause o f this
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230
Fig. 2.
Jpn. J. Surg. Sept. 1978
Microphotography of the resected loop of case 1 showing regenerated epithelia with atypia. • 35, H.E.
condition. The involved loop was resected and an end-to-end anastomosis carried out, but the patient expired soon after the operation. Case 3. M.Y., a four-day-old male weighing 3,500 g. was admitted because of bilious vomiting and abdominal distention. The course of pregnancy and delivery were normal. He passed blood-stained stool per rectum before admission. A plain film of the abdomen revealed pneumoperitoneum. At operation almost the entire small bowel was twisted except for the proximal 20 am of jejunum. After reduction of the volvulus, the intestine recovered its viability except for the distal portion of the ileum. Where a perforation was found, excised, and an end-to-end anastomosis of the remaining intestine performed. There was no abnormality found to predispose to the volvulus. The postoperative course was complicated by the malabsorption syndrome, but the patient was able to be discharged on the 33rd day after admission. Two weeks later further surgery was required to relieve strangulated small bowel secondary to adhesions. He has been well for three years. Case 4. N.T., a male weighing 3,160 g. was born after a normal pregnancy and delivery. No maternal history of polyhydramnios was noted. He was well until the 54th day, but on the 55th day of life was transferred by his pediatrician to our institution with a history of intermittent crying and vomiting for about 12 hours duration. On admission abdominal distention and mild dehydration were noted. X-ray examinations of the abdomen demonstrated multiple fluid levels. Contrast enema showed normal colon, but the terminal segment of ileum delineated an abnormal bending and "beak sign ''9 (Fig. 3). At laparotomy a segmental volvulus of the distal ileum measuring 20 cm was found. Reduction of the volvulus was prevented by a cord from the greater omentum adherent to the stalk of the twisted loop. After the removal of the cord the volvulus was reduced, but viability of the twisted bowel was doubtful. Resection and an end-to-end anastomosis was performed. There was no identifiable causative factor for this volvulus, except for the adhesion of the cord. Since the reduction was prevented by the cord, the adhesions might have developed after the occurrence of the volvulus. The postoperative course was uneventful and he was discharged on the 12th day after operation, but three
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Fig. 3.
Primary Small Bowel Vo!vulus in Infants
231
Contrast enema of case 4 showing an abnormal bending of the distal ileum and "beak sign" appearance.
Fig. 4. A plain radiograph of case 5 showing long fluid levels. months later he was reoperated upon for small bowel obstruction secondary to adhesions. Subsequently he has been well for four years. Case 5. H.T., a three-day-old male was admitted to our institution for persistent vomiting. He was delivered at 38 weeks of gestation weighing 3,500 g. with a maternal history ofpolyhydramnios. The fluid was turbid. He did well for six hours when vomiting began and persisted. A plain film of the abdomen showed the dilated stomach and long fluid levels (Fig. 4). No abnormal finding was revealed by contrast enema. At
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232
Fig. 5.
Jpn. J. Surg. Sept. 1978
Photograph of the excised intestine of case 5 showing a stenosis (arrowed). The intestine bends irregularily and has a tendency to form a spiral fashion.
laparotomy a localized small bowel volvulus of the distal ileum, extending 65 cm in length was demonstrated. Reduction of the volvulus was prevented by a cord from the greater omentum adherent to the apex of the twisted loop. Reduction was successful after the removal of the cord. The involved intestine appeared healthy. However, pulsation of the mesenterie vessels to this loop was absent and portion of this loop was almost perforated. Therefore the involved intestinal loop was excised and an end-to-end anastomosis was carried out. Although there was the adhesive cord as in case 4, we could not define the etiology for the volvulus. The proximal end of the twisted segment was stenotic and its continuity of the lumen was merely kept with only a serosa (Fig. 5). The shape of the mesentery of the twisted loop was not normal, being band-like rather than fan-like. And only a marginal vessel was there. Furthermore, the detorted loop bent irregularily and had a tendency to form a spiral (Fig. 5). Postoperative course was complicated by diarrhea, jaundice and the second operation for adhesive small bowel obstruction, he was discharged on the 109th day after admission and has been well for seven months. Our present cases have following features in common; (1) None of them have a malrotation and/or a malfixation of intestine including "isolated incomplete rotation. ''4 (2) The involved loop is twisted on its mesentery as an axis with various degrees of clockwise rotation. (3) There was no evidence ofmeconium peritonitis found in the operative and microscopic examinations. D~scuss~oN
Affected Site: The site affected by volvulus is dependent on the region where its causative lesion is located. A majority of small bowel volvulus in adults is localized in the ileum. 10-12 Even in infants, volvulus with meconium ileus s or congenital intestinal atresia 13 predominantly occurs in the ileum. O f our five cases with unknown etiology,
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three cases were localized volvulus of the ileum and one case was confined to the j e j u n u m (Table 1). T h e remainder with the longest duration o f symptoms was almost entire small bowel volvulus, but it was presumed that distal ileum where perforated was affected initially. M c K e c h n i e a n d Priestley 11 stated that "distention a n d increased peristalsis usually aid in the formation of the p r i m a r y loop and produce further twisting of the intestine once volvulus has been formed." I n general, it is likely that volvulus tends to occur in the ileum. Incidence: Primary small bowel volvulus has been reported sporadically and it is difficult to estimate the true incidence. But Santulli 16 reported 10 cases of this condition out of 207 consecutive cases of neonatal intestinal obstruction. T h e volvulus with unknown cause affects y o u n g e r infants, a n d those with known cause affects older infants a n d children. 7 This is not true of a m i d g u t volvulus with malrotation. Males predominate regardless o f the cause. 7 Etiology : T h e causative factors o f local small bowel volvulus, has been reported as follows: vitelline duct r e m n a n t , 1,15 t u m o r and tumor-like mass 18 (i.e. polyp, duplication, etc.), internal hernia, 1 m e c o n i u m ileus, 8 adhesions, 2 congenital a b n o r m a l band, a6 HenochSch6nlein p u r p u r a a n d ascariasis. Furthermore there are case reports of localized small bowel volvulus associated with congenital intestinal atresia or m e c o n i u m peritonitis but it is not clear which lesion is p r i m a r y or secondary. I n infants and children p r i m a r y volvulus of the small bowel is rare. These cases m a y be w h a t some authors m a y call "volvulus without malrotation." T h e majority of these reported cases are male neonates and have a widespreaded volvulus extending over most of the small bowel. We reviewed the reported cases of this category of "volvulus without malrotation," a n d discovered that this category includes various subdivisions those in which the descriptions of the rotation of the duodenojejunal loop or cecocolic loop or both were obscure, in which the volvulus was caused by malfixation of only ascending colon a n d cecum, a n d in which isolated incomplete rotation was noted. Since this category is not an established entity we should use the term "volvulus without malrotation" with great care, because (1) the term of " m a l r o t a t i o n " m a y also be applied to the realfixation of ascending colon and cecum with normal rotation of duodenojejunal and cecocolic loop, 6 (2) in addition to malrotation and/or malfixation o f the intestine, there are some other u n k n o w n causes of volvulus, such as p r i m a r y local volvulus as in our and Santulli's cases. Swenson 18 described that " m o r e localized forms of volvulus are the result of redundancy o f only a portion o f the mesentery." Chamberlain 3 reported three neonatal cases having normal rotation o f the duodenojejunal loop and a b n o r m a l kinkings in duodenal loop due to localized a b n o r m a l fixations to the posterior a b d o m i n a l wall. I f these abnormal kinking and fixations develop in the mesentery of the small bowel, they m a y cause localized small bowel volvulus. None of our cases h a d such a b n o r m a l fixations. An abnormality in the mesentery in case 5 was considered a result of impaired blood supply to the area. T h e pathological findings in case 5 drew our attention, because this might set a stage for congenital intestinal atresia or stenosis. Disturbance o f intestinal passage can be one of the causes ofvolvulus. But none of our cases demonstrated the findings suggesting this etiology such as Hirschsprung's disease, m e c o n i u m ileus and m e c o n i u m plug syndrome. The Time of Occurrence: As mechanical factors which p r o d u c e small bowel volvulus, Vaez-Zadeh 22 cites sudden changes of posture and i n t r a a b d o m i n a l pressure, rapid filling o f an e m p t y bowel and hyperperistalsis. All these factors are present during the course
234
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Jpn. J. Surg. Sept. 1978
of delivery. Our two cases (cases 2 and 3) were considered to have developed the volvulus shortly after delivery. In other two cases (cases 1 and 5) it was considered by the macroscopic and microscopic findings that the volvulus had alreadly occurred in utero. The occurrence of volvulus of the remainder (case 4) may be a day before admission. Symptoms: In-all of our cases the main symptoms were vomiting and abdominal distention, and preoperatively in three out of five cases there was a history of bloody stool and hematemesis. On the other hand, in the cases of midgut volvulus with malrotation, abdominal distention, hematemesis and/or bloody stool are not so frequently seen, 17 but vomiting is always present. The different symptoms found between localized small bowel volvulus and midgut volvulus may be attributed to the level of obstruction and the degree of twisting. 2~ For example, in the latter abdominal distention may be confined to the upper abdomen even if it is recognized, because its stalk involving the colon which may act as a role of cushion so that the damage from the volvulus may be less severe than in the former. 20 In the former the twisting is more intense than in the latter, and the involved loop more easily becomes gangrenous. Therefore resection of the involved loop is more frequently required in the former. Diagnosis: In many of the reports 10-1e it is generally agreed that a diagnosis of small bowel volvulus is very difficult to make because there is no specific findings in the clinical picture to distinguish from simple strangulating bowel obstruction. The preoperative diagnosis of our cases are strangulated obstruction in three, intestinal perforation and congenital ileal atresia in one, respectively. In Juler's series 10 of adult small bowel volvulus, an interesting finding was the occurrence of albuminuria in a high percentage (65 per cent). This finding was not elucidated in our cases. A plain film of the abdomen merely shows a picture of simple or strangulated obstruction which is so inconclusive that radiographic findings preclude from correct diagnosis. The described x-ray findings, most helpful in making the diagnosis are, "arcade pattern," "long fluid levels," etc.5, a0 Long fluid levels were noticed in three of our five cases and thought to be helplhl findings to some extent. Contrast enema should be performed with great care, because the affected portion of intestine tends to fall into necrosis rather rapidly. A film of case 1 (Fig. 1) was interpreted as contrast material flowing into the ileum and directly delineated a part of twisted ileum. An abnormal kinking of the ileum on the film of case 4 (Fig. 3), although it resembles "beak sign ''9 or "snake head sign ''9 in sigmoid volvulus, was interpreted as formed by the extrinsic pressure of dilated loops surrounding it. Prognosis: Localized small bowel volvulus rapidly progresses to gangrene and perforation of the involved bowel. Therefore the ultimate survival of the neonates depends upon the severity of the concomitant peritonitis. I f laparotomy is carried out rapidly, we may say the result is more satisfactory. In our series, four cases were found to have a perforation of intestine and peritonitis at the time of surgery and resection was necessary in all cases. However, all of them recovered except for one treated nine years ago. O f our survived four patients, three were complicated with postoperative small bowel obstruction due to adhesions necessitating careful follow-up. CONCLUSION
The characteristic clinical features of localized small bowel volvulus with unknown etiology were as follows; (1) Male predominance.
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235
(2) S c o u t film d e m o n s t r a t i o n o f e i t h e r p a t t e r n s o f d i s t a l s m a l l b o w e l o b s t r u c t i o n i n t h e m a j o r i t y o f cases o r l o n g f l u i d levels. (3) V o l v u l u s t e n d i n g to a f f e c t i:he i l e u m . (4) R a p i d p r o g r e s s i o n to i n t e s t i n a l i n f a r c t i o n a n d p e r f o r a t i o n r e q u i r i n g s u r g i c a l intervention. (5) S a t i s f a c t o r y r e s u l t s f o l l o w i n g e m e r g e n c y s u r g e r y e x c e p t for f r e q u e n t p o s t o p e r a t i v e ileus d u e to a d h e s i o n s . (Received for p u b l i c a t i o n on J a n u a r y 12, 1977) References 1. Benson, C.D. and Linkner, L.M. : The surgical complications of Meckel's diverticulum in infants and children, Arch. Surg. 73 : 393-398, 1956. 2. Benson, C.D., Lloyd, J.R. and Krabbenhoft, K.L.: The surgical and metabolic aspects of massive small bowel resection in the newborn, J. Pediatr. Surg. 2: 227-240, 1967. 3. Chamberlain, J.W.: Partial intestinal obstruction in the newborn due to kinking of the proximal small bowel, N. Engl. J. Med. 275: 1241-1242, 1966. 4. Firor, H.V. and Harris, V.J.: Rotational abnormalities of the gut, re-emphasis of a neglected facet, isolated incomplete rotation of the duodenum, Am. J. Roentgenol. 120: 315-321, 1974. 5. Frimann-Dahl, J.: Roentgen examinations in acute abdominal disease, 3rd ed., pp. 264278, C.C. Thomas Publisher, Springfield, 1974. 6. Gross, R.E. : The surgery of infancy and childhood, pp. 192-203, W.B. Saunders Company, Philadelphia, 1953. 7. Hamanaka, Y., Narita, N., Tsuboi, K., Onoda, K. and Murakami, Y. : A survived neonatal case of intestinal volvulus without malrotation, Geka Shinryo (Surgical Diagnosis & Treatment) 18" 703-706, 1976 (in Japanese). 8. Holsclaw, D.S., Eckstein, H.B. and Nixon, H.H.: Meconium ileus, a 20-year review of 109 cases, Am. J. Dis. Child. I09: 101-113, 1965. 9. Hunter, J.G. and Keats, T.E.: Sigmoid volvulus in children, a case report, Am. J. Roentgenol. 108: 621-623, 1970. 10. Juler, G.L., Stemmer, E.A. and Connolly, J.E. : Preoperative diagnosis of small bowel volvulus in adults, Am. J. Gastroenterol. 56: 235247, 1971. 11. McKechnie, R.E. and Priestley, J.T.: Volvulus of small intestine, a report of thirtyseven cases, Am. J. Surg. 34: 286-291, 1936.
12. Moretz, W.H. and Morton, J.J.: Acute volvulus of small intestine, analysis of 36 cases, Ann. Surg. 132: 899-912, 1950. 13. Nixon, H.H. and Tawes, R." Etiology and treatment of small intestinal atresia: analysis of a series of 127 jejunoileal and comparison with 62 duodenal atresias, Surgery 69 : 41-51, 1971. 14. Ohkawa, H., Takahashi, H., Maie, M., Ohnuma, N., Saito, K., Sato, H. and Kazuma, K. : Small bowel volvulus in newborn period, Geka (Surgery) 34: 688-694, 1972 (in Japanese). 15. Rutherford, R.B. and Akers, D.R.: Meckel's diverticulum: a review of 148 pediatric patients, with special reference to the pattern of bleeding and to mesodiverticular vascular bands, Surgery 59: 618-626, 1966. 16. Santulli, T.V.: Intestinal obstruction in the newborn, Bull. N.Y. Acad. Med. 33: 175-194, 1957.
17. Stewart, D.R., Colodny, A.L. and Dagget, W.C.: Malrotation of the bowel in infants and children, Surgery 79: 716-720, 1976. 18. Swenson, O.: Pediatric surgery, 3rd ed., pp. 660-664, Appleton-Century-Crofts Inc, New York, 1969. 19. Tsuchida, Y., Makino, S., Honna, T. and Saito, S.: Three cases of volvulus without malrotation, Nippon Shinseo'i Gakkai Zasshi (Acta NeonatologicaJaponica) 13: 79-80, 1977 (in Japanese). 20. Tsunoda, A.: Malrotation and volvulus of small intestine, Geka Shinryo (Surgical Diagnosis & Treatment) 1 8 : 6 0 7 612, 1976 (in Japanese). 21. Wilkinson, T.S. and Stone, H.H." Intestinal volvulus without malrotation in a fourmonth-old infant, Am. Surg. 3 3 : 3 6 5 366, 1967. 22. Vaez-Zadeh, K., Dutz, W. and NowroozZadeh, M. : Volvulus of the small ~ntestine in adults: a study of predisposing factors, Ann. Surg. 169:265 271, 1969.
V o l v u l u s is found in various parts of the gastrointestinal tract even in infants and children. However, most of them are midgut volvulus associated with malrotation of the intestine. Localized small bowel volvulus is also seen with certain organic lesions, such as vitelline duct remnant, 1,15 intestinal polyp, TM mesenteric cyst, TM etc. There are only a small number of reported cases of primary small bowel volvulus. Such volvuli of the intestine are what some authors would call "volvulus without malrotation. ''2,7,14,19,21 We reviewed those case reports and found that the implicated intestines involved almost the entire small bowel in the majority of the reports. During nine years we experienced five cases of primary volvulus of the small intestine (Table 1). Among these five cases a localized loop of the small bowel was involved in four cases and in one case almost entire small bowel was twisted. Namely, four cases were localized small bowel volvulus and one case might be a so called "volvulus without malrotation." CASE REPORTS
Case 1. K.M., a male was born at 36 weeks of gestation weighing 3,270 g. with a maternal history of hydramnios. He was admitted at three days of age to our institution with gradually developing abdominal distention and bilious vomiting starting 24 hours after birth, and passed a small amount of bloody stool per rectum immediately after admission. A scout film showed the distended stomach and relatively long fluid levels. Contrast enema revealed normal colon, but the terminal segment of ileum showed a circular configuration (Fig. 1). At laparotomy a gangrenous localized volvulus of the distal ileum measuring 20 cm in length was found. The gangrenous segment was resected and an end-to-end anastomosis was performed. Any causative factors could not be found. The postoperative course was uneventful and the patient has been well for four years. On microscopic examination, the specimen showed partial loss of mucosa, marked capillary dilatations and severe inflammatory changes. Regenerated epithelia with atypia were also found, suggesting that the occurrence of the volvulus was probably prior to delivery (Fig. 2). From the Department of Surgery I, Gumma UniversitySchool of Medicine (Director: Prof. Takuji Nakamura), Maebashi, Japan. JAPANESEJOURNAL OF SURGERY, VOL. 8, No. 3, pp 228--235, 1978
Volume 8 Number 3
T a b l e 1. Case No. 1.
229
Primary Small Bowel Volvulus in Infants
Age (day) 3
Cases of small bowel volvulus in infants (1967-1976)
Sex
Birth weight (g.)
M
3,270
Melena
Site & length (era)
Perforation
Result
Postop. ileus
(+)
ileum
( -- )
alive
(--)
(+ )
died
(+ )
alive
(+ )
(--)
alive
(+)
( -- )*
alive
(+ )
(20) 2.
3
F
3,180
(4-)
jejunum
(4o) 3.
4
M
3,500
(§ )
4.
55
M
3,160
(--)
almost entire ileum
(20) 5.
3
M
3,500
( -- )
ileum
(65) * almost perforated
Fig. 1.
Contrast enema of case 1. Distal ileum shows circular configurations.
Case 2. H.S., a female w e i g h i n g 3,180 g. was b o r n at t e r m b y a m o t h e r with polyh y d r a m n i o s . V o m i t i n g b e g a n a t nine hours o f age a n d g r a d u a l l y the vomitus t u r n e d bilious. A p l a i n film o f the a b d o m e n r e v e a l e d a m o d e r a t e l y d i s t e n d e d s t o m a c h a n d a few relatively long fluid levels in the left u p p e r a b d o m e n . C o n t r a s t e n e m a was i n t e r p r e t e d as n o r m a l . She was transferred to o u r institution three days later. O n admission the b a b y was lethargic a n d her a b d o m e n was d i s t e n d e d r e m a r k a b l y . O n e x a m i n a t i o n there were evidences o f severe d e h y d r a t i o n a n d m a r k e d j a u n d i c e . C o f f e e - g r o u n d m a t e r i a l was a s p i r a t e d via a nasogastric tube. L a p a r o t o m y was done on the d a y o f admission a n d j e j u n a l volvulus was found. T h e l o c a t i o n was a p p r o x i m a t e l y 10 c m distal to the l i g a m e n t o f Treitz. T h e length o f the twisted intestine e x t e n d e d a b o u t 40 cm, a n d was gangrenous a n d perforated. T h e r e was no definite a n a t o m i c a l findings to e x p l a i n the cause o f this
Kurashige and Matsuyama
230
Fig. 2.
Jpn. J. Surg. Sept. 1978
Microphotography of the resected loop of case 1 showing regenerated epithelia with atypia. • 35, H.E.
condition. The involved loop was resected and an end-to-end anastomosis carried out, but the patient expired soon after the operation. Case 3. M.Y., a four-day-old male weighing 3,500 g. was admitted because of bilious vomiting and abdominal distention. The course of pregnancy and delivery were normal. He passed blood-stained stool per rectum before admission. A plain film of the abdomen revealed pneumoperitoneum. At operation almost the entire small bowel was twisted except for the proximal 20 am of jejunum. After reduction of the volvulus, the intestine recovered its viability except for the distal portion of the ileum. Where a perforation was found, excised, and an end-to-end anastomosis of the remaining intestine performed. There was no abnormality found to predispose to the volvulus. The postoperative course was complicated by the malabsorption syndrome, but the patient was able to be discharged on the 33rd day after admission. Two weeks later further surgery was required to relieve strangulated small bowel secondary to adhesions. He has been well for three years. Case 4. N.T., a male weighing 3,160 g. was born after a normal pregnancy and delivery. No maternal history of polyhydramnios was noted. He was well until the 54th day, but on the 55th day of life was transferred by his pediatrician to our institution with a history of intermittent crying and vomiting for about 12 hours duration. On admission abdominal distention and mild dehydration were noted. X-ray examinations of the abdomen demonstrated multiple fluid levels. Contrast enema showed normal colon, but the terminal segment of ileum delineated an abnormal bending and "beak sign ''9 (Fig. 3). At laparotomy a segmental volvulus of the distal ileum measuring 20 cm was found. Reduction of the volvulus was prevented by a cord from the greater omentum adherent to the stalk of the twisted loop. After the removal of the cord the volvulus was reduced, but viability of the twisted bowel was doubtful. Resection and an end-to-end anastomosis was performed. There was no identifiable causative factor for this volvulus, except for the adhesion of the cord. Since the reduction was prevented by the cord, the adhesions might have developed after the occurrence of the volvulus. The postoperative course was uneventful and he was discharged on the 12th day after operation, but three
Volume 8 Number 3
Fig. 3.
Primary Small Bowel Vo!vulus in Infants
231
Contrast enema of case 4 showing an abnormal bending of the distal ileum and "beak sign" appearance.
Fig. 4. A plain radiograph of case 5 showing long fluid levels. months later he was reoperated upon for small bowel obstruction secondary to adhesions. Subsequently he has been well for four years. Case 5. H.T., a three-day-old male was admitted to our institution for persistent vomiting. He was delivered at 38 weeks of gestation weighing 3,500 g. with a maternal history ofpolyhydramnios. The fluid was turbid. He did well for six hours when vomiting began and persisted. A plain film of the abdomen showed the dilated stomach and long fluid levels (Fig. 4). No abnormal finding was revealed by contrast enema. At
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Fig. 5.
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Photograph of the excised intestine of case 5 showing a stenosis (arrowed). The intestine bends irregularily and has a tendency to form a spiral fashion.
laparotomy a localized small bowel volvulus of the distal ileum, extending 65 cm in length was demonstrated. Reduction of the volvulus was prevented by a cord from the greater omentum adherent to the apex of the twisted loop. Reduction was successful after the removal of the cord. The involved intestine appeared healthy. However, pulsation of the mesenterie vessels to this loop was absent and portion of this loop was almost perforated. Therefore the involved intestinal loop was excised and an end-to-end anastomosis was carried out. Although there was the adhesive cord as in case 4, we could not define the etiology for the volvulus. The proximal end of the twisted segment was stenotic and its continuity of the lumen was merely kept with only a serosa (Fig. 5). The shape of the mesentery of the twisted loop was not normal, being band-like rather than fan-like. And only a marginal vessel was there. Furthermore, the detorted loop bent irregularily and had a tendency to form a spiral (Fig. 5). Postoperative course was complicated by diarrhea, jaundice and the second operation for adhesive small bowel obstruction, he was discharged on the 109th day after admission and has been well for seven months. Our present cases have following features in common; (1) None of them have a malrotation and/or a malfixation of intestine including "isolated incomplete rotation. ''4 (2) The involved loop is twisted on its mesentery as an axis with various degrees of clockwise rotation. (3) There was no evidence ofmeconium peritonitis found in the operative and microscopic examinations. D~scuss~oN
Affected Site: The site affected by volvulus is dependent on the region where its causative lesion is located. A majority of small bowel volvulus in adults is localized in the ileum. 10-12 Even in infants, volvulus with meconium ileus s or congenital intestinal atresia 13 predominantly occurs in the ileum. O f our five cases with unknown etiology,
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three cases were localized volvulus of the ileum and one case was confined to the j e j u n u m (Table 1). T h e remainder with the longest duration o f symptoms was almost entire small bowel volvulus, but it was presumed that distal ileum where perforated was affected initially. M c K e c h n i e a n d Priestley 11 stated that "distention a n d increased peristalsis usually aid in the formation of the p r i m a r y loop and produce further twisting of the intestine once volvulus has been formed." I n general, it is likely that volvulus tends to occur in the ileum. Incidence: Primary small bowel volvulus has been reported sporadically and it is difficult to estimate the true incidence. But Santulli 16 reported 10 cases of this condition out of 207 consecutive cases of neonatal intestinal obstruction. T h e volvulus with unknown cause affects y o u n g e r infants, a n d those with known cause affects older infants a n d children. 7 This is not true of a m i d g u t volvulus with malrotation. Males predominate regardless o f the cause. 7 Etiology : T h e causative factors o f local small bowel volvulus, has been reported as follows: vitelline duct r e m n a n t , 1,15 t u m o r and tumor-like mass 18 (i.e. polyp, duplication, etc.), internal hernia, 1 m e c o n i u m ileus, 8 adhesions, 2 congenital a b n o r m a l band, a6 HenochSch6nlein p u r p u r a a n d ascariasis. Furthermore there are case reports of localized small bowel volvulus associated with congenital intestinal atresia or m e c o n i u m peritonitis but it is not clear which lesion is p r i m a r y or secondary. I n infants and children p r i m a r y volvulus of the small bowel is rare. These cases m a y be w h a t some authors m a y call "volvulus without malrotation." T h e majority of these reported cases are male neonates and have a widespreaded volvulus extending over most of the small bowel. We reviewed the reported cases of this category of "volvulus without malrotation," a n d discovered that this category includes various subdivisions those in which the descriptions of the rotation of the duodenojejunal loop or cecocolic loop or both were obscure, in which the volvulus was caused by malfixation of only ascending colon a n d cecum, a n d in which isolated incomplete rotation was noted. Since this category is not an established entity we should use the term "volvulus without malrotation" with great care, because (1) the term of " m a l r o t a t i o n " m a y also be applied to the realfixation of ascending colon and cecum with normal rotation of duodenojejunal and cecocolic loop, 6 (2) in addition to malrotation and/or malfixation o f the intestine, there are some other u n k n o w n causes of volvulus, such as p r i m a r y local volvulus as in our and Santulli's cases. Swenson 18 described that " m o r e localized forms of volvulus are the result of redundancy o f only a portion o f the mesentery." Chamberlain 3 reported three neonatal cases having normal rotation o f the duodenojejunal loop and a b n o r m a l kinkings in duodenal loop due to localized a b n o r m a l fixations to the posterior a b d o m i n a l wall. I f these abnormal kinking and fixations develop in the mesentery of the small bowel, they m a y cause localized small bowel volvulus. None of our cases h a d such a b n o r m a l fixations. An abnormality in the mesentery in case 5 was considered a result of impaired blood supply to the area. T h e pathological findings in case 5 drew our attention, because this might set a stage for congenital intestinal atresia or stenosis. Disturbance o f intestinal passage can be one of the causes ofvolvulus. But none of our cases demonstrated the findings suggesting this etiology such as Hirschsprung's disease, m e c o n i u m ileus and m e c o n i u m plug syndrome. The Time of Occurrence: As mechanical factors which p r o d u c e small bowel volvulus, Vaez-Zadeh 22 cites sudden changes of posture and i n t r a a b d o m i n a l pressure, rapid filling o f an e m p t y bowel and hyperperistalsis. All these factors are present during the course
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of delivery. Our two cases (cases 2 and 3) were considered to have developed the volvulus shortly after delivery. In other two cases (cases 1 and 5) it was considered by the macroscopic and microscopic findings that the volvulus had alreadly occurred in utero. The occurrence of volvulus of the remainder (case 4) may be a day before admission. Symptoms: In-all of our cases the main symptoms were vomiting and abdominal distention, and preoperatively in three out of five cases there was a history of bloody stool and hematemesis. On the other hand, in the cases of midgut volvulus with malrotation, abdominal distention, hematemesis and/or bloody stool are not so frequently seen, 17 but vomiting is always present. The different symptoms found between localized small bowel volvulus and midgut volvulus may be attributed to the level of obstruction and the degree of twisting. 2~ For example, in the latter abdominal distention may be confined to the upper abdomen even if it is recognized, because its stalk involving the colon which may act as a role of cushion so that the damage from the volvulus may be less severe than in the former. 20 In the former the twisting is more intense than in the latter, and the involved loop more easily becomes gangrenous. Therefore resection of the involved loop is more frequently required in the former. Diagnosis: In many of the reports 10-1e it is generally agreed that a diagnosis of small bowel volvulus is very difficult to make because there is no specific findings in the clinical picture to distinguish from simple strangulating bowel obstruction. The preoperative diagnosis of our cases are strangulated obstruction in three, intestinal perforation and congenital ileal atresia in one, respectively. In Juler's series 10 of adult small bowel volvulus, an interesting finding was the occurrence of albuminuria in a high percentage (65 per cent). This finding was not elucidated in our cases. A plain film of the abdomen merely shows a picture of simple or strangulated obstruction which is so inconclusive that radiographic findings preclude from correct diagnosis. The described x-ray findings, most helpful in making the diagnosis are, "arcade pattern," "long fluid levels," etc.5, a0 Long fluid levels were noticed in three of our five cases and thought to be helplhl findings to some extent. Contrast enema should be performed with great care, because the affected portion of intestine tends to fall into necrosis rather rapidly. A film of case 1 (Fig. 1) was interpreted as contrast material flowing into the ileum and directly delineated a part of twisted ileum. An abnormal kinking of the ileum on the film of case 4 (Fig. 3), although it resembles "beak sign ''9 or "snake head sign ''9 in sigmoid volvulus, was interpreted as formed by the extrinsic pressure of dilated loops surrounding it. Prognosis: Localized small bowel volvulus rapidly progresses to gangrene and perforation of the involved bowel. Therefore the ultimate survival of the neonates depends upon the severity of the concomitant peritonitis. I f laparotomy is carried out rapidly, we may say the result is more satisfactory. In our series, four cases were found to have a perforation of intestine and peritonitis at the time of surgery and resection was necessary in all cases. However, all of them recovered except for one treated nine years ago. O f our survived four patients, three were complicated with postoperative small bowel obstruction due to adhesions necessitating careful follow-up. CONCLUSION
The characteristic clinical features of localized small bowel volvulus with unknown etiology were as follows; (1) Male predominance.
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(2) S c o u t film d e m o n s t r a t i o n o f e i t h e r p a t t e r n s o f d i s t a l s m a l l b o w e l o b s t r u c t i o n i n t h e m a j o r i t y o f cases o r l o n g f l u i d levels. (3) V o l v u l u s t e n d i n g to a f f e c t i:he i l e u m . (4) R a p i d p r o g r e s s i o n to i n t e s t i n a l i n f a r c t i o n a n d p e r f o r a t i o n r e q u i r i n g s u r g i c a l intervention. (5) S a t i s f a c t o r y r e s u l t s f o l l o w i n g e m e r g e n c y s u r g e r y e x c e p t for f r e q u e n t p o s t o p e r a t i v e ileus d u e to a d h e s i o n s . (Received for p u b l i c a t i o n on J a n u a r y 12, 1977) References 1. Benson, C.D. and Linkner, L.M. : The surgical complications of Meckel's diverticulum in infants and children, Arch. Surg. 73 : 393-398, 1956. 2. Benson, C.D., Lloyd, J.R. and Krabbenhoft, K.L.: The surgical and metabolic aspects of massive small bowel resection in the newborn, J. Pediatr. Surg. 2: 227-240, 1967. 3. Chamberlain, J.W.: Partial intestinal obstruction in the newborn due to kinking of the proximal small bowel, N. Engl. J. Med. 275: 1241-1242, 1966. 4. Firor, H.V. and Harris, V.J.: Rotational abnormalities of the gut, re-emphasis of a neglected facet, isolated incomplete rotation of the duodenum, Am. J. Roentgenol. 120: 315-321, 1974. 5. Frimann-Dahl, J.: Roentgen examinations in acute abdominal disease, 3rd ed., pp. 264278, C.C. Thomas Publisher, Springfield, 1974. 6. Gross, R.E. : The surgery of infancy and childhood, pp. 192-203, W.B. Saunders Company, Philadelphia, 1953. 7. Hamanaka, Y., Narita, N., Tsuboi, K., Onoda, K. and Murakami, Y. : A survived neonatal case of intestinal volvulus without malrotation, Geka Shinryo (Surgical Diagnosis & Treatment) 18" 703-706, 1976 (in Japanese). 8. Holsclaw, D.S., Eckstein, H.B. and Nixon, H.H.: Meconium ileus, a 20-year review of 109 cases, Am. J. Dis. Child. I09: 101-113, 1965. 9. Hunter, J.G. and Keats, T.E.: Sigmoid volvulus in children, a case report, Am. J. Roentgenol. 108: 621-623, 1970. 10. Juler, G.L., Stemmer, E.A. and Connolly, J.E. : Preoperative diagnosis of small bowel volvulus in adults, Am. J. Gastroenterol. 56: 235247, 1971. 11. McKechnie, R.E. and Priestley, J.T.: Volvulus of small intestine, a report of thirtyseven cases, Am. J. Surg. 34: 286-291, 1936.
12. Moretz, W.H. and Morton, J.J.: Acute volvulus of small intestine, analysis of 36 cases, Ann. Surg. 132: 899-912, 1950. 13. Nixon, H.H. and Tawes, R." Etiology and treatment of small intestinal atresia: analysis of a series of 127 jejunoileal and comparison with 62 duodenal atresias, Surgery 69 : 41-51, 1971. 14. Ohkawa, H., Takahashi, H., Maie, M., Ohnuma, N., Saito, K., Sato, H. and Kazuma, K. : Small bowel volvulus in newborn period, Geka (Surgery) 34: 688-694, 1972 (in Japanese). 15. Rutherford, R.B. and Akers, D.R.: Meckel's diverticulum: a review of 148 pediatric patients, with special reference to the pattern of bleeding and to mesodiverticular vascular bands, Surgery 59: 618-626, 1966. 16. Santulli, T.V.: Intestinal obstruction in the newborn, Bull. N.Y. Acad. Med. 33: 175-194, 1957.
17. Stewart, D.R., Colodny, A.L. and Dagget, W.C.: Malrotation of the bowel in infants and children, Surgery 79: 716-720, 1976. 18. Swenson, O.: Pediatric surgery, 3rd ed., pp. 660-664, Appleton-Century-Crofts Inc, New York, 1969. 19. Tsuchida, Y., Makino, S., Honna, T. and Saito, S.: Three cases of volvulus without malrotation, Nippon Shinseo'i Gakkai Zasshi (Acta NeonatologicaJaponica) 13: 79-80, 1977 (in Japanese). 20. Tsunoda, A.: Malrotation and volvulus of small intestine, Geka Shinryo (Surgical Diagnosis & Treatment) 1 8 : 6 0 7 612, 1976 (in Japanese). 21. Wilkinson, T.S. and Stone, H.H." Intestinal volvulus without malrotation in a fourmonth-old infant, Am. Surg. 3 3 : 3 6 5 366, 1967. 22. Vaez-Zadeh, K., Dutz, W. and NowroozZadeh, M. : Volvulus of the small ~ntestine in adults: a study of predisposing factors, Ann. Surg. 169:265 271, 1969.
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