Cecal Volvulus Associated with Incomplete Situs lnversus and Splenic Dysgenesis
J. William Futrell, MD,‘ Cleveland, Ohio Arnold M. Rosen, MD, Cleveland, Ohio William Annable, MD, Cleveland, Ohio Jerry S. Wolkoff, MD, Cleveland, Ohio
The functional malformations associated with situs inversus are primarily cardiovascular although significant visceral or structural deformities or both do occur. Indeed, more than half the patients born with situs inversus (approximately 1 in 11,000 persons, according to Gould [I]) have other major congenital anomalies. When an acute surgical emergency occurs in such a person, one should therefore be alerted to the possibility of other unsuspected intra-abdominal disease. The occurrence of acute cecal volvulus associated with several of the other lesser appreciated anomalies related to situs inversus, such as splenic dysgenesis and liver symmetry, has not previously been reported. This problem creates a number of heretofore unencountered operative decisions and is currently cited to stress the necessity for prompt surgical intervention and the need to expect the unexpected. Case Report A fifty-three year old Caucasian man presented to the Cleveland Veterans Administration Hospital with a one week history of diffuse abdominal pain which had grown precipitously worse over the preceding twelve hours. The pain was cramping, without localization or radiation, and was associated with nausea and vomiting. Physical examination revealed an acutely ill appearing From the Department of Surgery, Case Western Reserve University School of Medicine, Cleveland, Ohio. * Present address and address for reprint requests: Division of Plastic Surgery, The Johns Hopkins Hospital, Baltimore, Maryland 21205.
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man with a markedly distended, tympanitic abdomen. Bowel sounds were hyperactive with occasional highpitched rushes. Digital rectal examination showed nothing abnormal with no stool in the rectum and no palpable masses. The chest was clear bilaterally and there was a regular cardiac rhythm with the point of maximal impulse in the fifth left intercostal space. There were no murmurs or cardiomegaly and no abdominal scars. The temperature was 1Ol’F and the white blood cell count was 12,000 per mm3 with 78 per cent polymorphonuclear leukocytes and 22 per cent lymphocytes. The patient revealed that he had been one of twins, and at birth the infants had been joined physically and required operative separation. The other twin allegedly died at that time although the patient was unable to give further information regarding this disclosure. He had no physical evidence of a conjoined birth except for the presence of what he termed a “birthmark scar” measuring approximately 3 cm in diameter in the right deltoid area. He also stated that as an army inductee he had been told that his “testicles were reversed.” He had no further history regarding this fact and apparently had served his military tenure and lived his subsequent life without notable illness. The patient’s testicles were re-examined and considered to be normal with the left slightly lower than the right. Findings on chest x-ray film (Figure 1) were normal and flat and upright films of the abdomen (Figures 2 and 3) revealed a single large distended loop of bowel in the left upper quadrant and multiple air-fluid levels. There was no free air. The grossly distended bowel had haustral markings that extended incompletely across the lumen and this section was thought to represent colon. The x-ray findings were thought to be most consistent with cecal volvulus.
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Figure 1. A preoperative chest film showing the normal cardiac silhouette in the left hemithorax. Figure 2. An upright abdominal fiim demonstrating muftipie air-fluid /eve/s and a large loop of distended bowei in the left upper quadrant. Figure 3. A fiat plate of the abdomen showing the marked/y distended bowel loop in the left upper quadrant. The haustrai markings do not completely cross the lumen, suggesting that the segment represents colon.
Sigmoidoscopy to 30 cm revealed no abnormalities. An emergency barium enema revealed a redundant, nondistended sigmoid colon with an obstruction and spiral contour of mucosal folds at the level of the transverse colon. (Figure 4.) In addition, because of the patient’s statement regarding “reversed testicles,” a small amount of Hypaque’ was injected through a nasogastric tube to determine the placement of the stomach. This revealed that the stomach was positioned abnormally on the right side of the abdomen (Figure 5), consistent with situs inversus. In view of the normal placement of the sigmoid colon seen on barium enema, a pre-
operative diagnosis of cecal volvulus in a patient with incomplete situs inversus was made. At operation a 360 degree volvulus of the cecum and terminal ileum was found. There was massive cecal distention with three longitudinal serosal tears but no free perforation. The bowel was gently derotated in a counterclockwise direction, and good vascularity and viability were apparent. The normal position of the cecum seemed to be in the right lower quadrant, although palpation of the upper part of the abdomen confirmed the abnormal location of the stomach on the right side with a peculiarly symmetrical liver located approximately in
Figure 4. A preoperative barium enema revealing normal placement of a redundant sigmoid and descending colon with obstruction and nonfiiiing beyond the transverse colon. There appears to be a spiral contour of the mucosal folds, suggesting torsion at the site of obstruction. Figure 5. insertion of a smaii amount of contrast medium through a nasogastric tube reveals ectopic placement of the stomach in the right upper quadrant. There is resktuai barium from the barium enema in the sigmokt colon in the left lower quadrant.
The American Journal ol Surgery
the midline. The gallbladder was situated slightly toward the left side of the abdominal cavity as was the pylorus. There was no palpable normal spleen, although a small golf-ball-sized, whitish structure on a small vascular pedicle was removed from the right upper quadrant. Sectioning indicated that this was splenic tissue and the subsequent pathology report confirmed the “hyalinization” of. the capsule accounting for the whitish appearance. Because the preoperative barium enema examination demonstrated that the normal position of the descending colon and rectum was in the left lower quadrant, proper positioning of the cecum in the right lower quadrant was obtained and cecostomy performed. The serosal lacerations were repaired with interrupted 4-O silk sutures. The patient’s postoperative course was uneventful and the cecostomy tube was removed on the twelfth hospital day. He was discharged two days later and has remained well. Comments Situs inversus may be present in various stages or degrees of organ reversal. The term “situs inversus totalis” refers to the complete mirror-image transposition of all asymmetrical body structures. Dextrocardia (situs inversus cardia) refers to the mirror-image position of the heart in the right hemithorax, whereas situs inversus viscerum refers to the reversal of the normal asymmetry of the gut structures. Situs solitus is the correct terminology for the normal anatomic situation. The typical diagnostic physical findings associated with complete situs inversus include localization of the apical cardiac impulse in the right thorax, gastric tympany on the right, liver dullness on the left, and the location of the right testis lower in the scrotal sac than the left. This latter finding was confirmed by Cholst  who found that in 93 per cent of 6,000 normal army inductees the left testis assumed a lower position than did the right, whereas in six cases of situs inversus the opposite was true. A similar finding had been suggested earlier by others [3,4] and is thought to be due to a reversal in the pattern of the normal venous hydrostatic pressures. Embryologically the originally paired cardinal veins evolve into a system whereby normally the right testicular vein empties directly into the vena cava whereas the left empties into the left renal vein. The longer course traversed by the left testicular and internal spermatic vein is thought to produce an elevated venous pressure on that side, thereby exerting a greater hydrostatic force pushing the left testis to a lower position. The higher incidence of varicocele on the left is presumably due to the same phenomenon. In situs inversus all such situations are reversed.
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A reversal of the normal “physiologic scoliosis” may also occur with situs inversus. Farkas  observed that in normal persons various degrees of scoliosis occur, with slight convexity to the right in the area of the thoracic spine caused by an increased gait force on the right due to the greater weight of the viscera on this side of the body. Such a relation is usually reversed in persons with situs reversus. In addition, the association between several anomalies of the respiratory tract and situs inversus has been suggested [6-8]. Kartagener’s syndrome  consisting of a triad of situs inversus, bronchiectasis, and sinusitis has been well documented. Olsen  has pointed out that the occurrence of bronchiectasis alone in patients with some degree of situs inversus far exceeds the predicted occurrence in the normal population. This finding tends to support the theory of a congenital origin in most cases of bronchiectasis. The explanation is that when bronchiectasis occurs, the pulmonary lobes and segments immediately adjacent to the heart are invariably involved, suggesting that mechanical obstruction from the surrounding tissue during the course of maturation of the lung may promote the development of immature bronchial pouches devoid of elastic or muscular tissue. With dextrocardia the factor of mechanical obstruction of the pulmonary parenchyma is greatly increased since the lungs remain in their normal anatomic position. A relationship between twinning, which in itself is a complex problem, and the development of situs inversus has been speculated. Relative to the current case, Newman [II] and Aird  have reported that situs inversus frequently appears in laterally conjoined human twins, the right hand member being the one affected as an apparent mirror-image effect. It is suggested that situs inversus may result from developmental arrest when fission takes place after bilateral symmetry has been established with the production of monozygotic twins, one of whom is the mirror image of the other. Situs inversus has also been observed in both members of dizygotic twins . In patients with situs inversus viscerum presenting with an acute illness, localization of the abdominal pain can be confusing. Organ reversal can occur without concomitant reversal of the sensory nerve pathway [I]. Nonreversal of the pain pathways would explain the occasional case in which symptoms of cholecystitis become manifest in the right upper quadrant or those of appendicitis in the right lower quadrant even though the involved organ is located on the opposite side. More
Futrell et al
frequently, however, the neural afferent sensory patterns are reversed in accord with the organ reversal. Although severe cardiac malformations are well known to be associated with situs inversus [14,15], rarely have the abdominal manifestations and other less recognized venous and visceral malformations been emphasized. These latter findings can include bilateral superior venae cavae, the absence of a segment of the inferior vena cava, agenesis of the spleen, and symmetry of the liver [10,16,17]. Wood and Blalock  reported that the incidence of biliary tract disease in patients with situs inversus is no higher than that in the general population. Of thirty-seven infants with situs inversus, Fonkalsrud, Tompkins, and Clatworthy  reported that fifteen had major intraabdominal anomalies requiring operative intervention. More than 80 per cent of these lesions were surgically correctable and two thirds of them were associated with congenital obstruction of the gastrointestinal tract. Eight of the fifteen patients had stenosis or atresia of the duodenum or jejunum, and seven had incomplete fixation of the bowel mesentery. Two of these patients required operation for correction of midgut volvulus. Because of this incidence of incomplete fixation of the bowel mesentery, intestinal volvulus is prone to occur. Although volvulus of the cecum is an unusual event in any population, it is an entity with which clinicians should be thoroughly familiar because of the acute need for emergency surgery. The frequent delay in diagnosis accounts for the high mortality which is often greater than 50 per cent when nonviable bowel is encountered [20,21]. Although the absence of a normal posterior mesenteric attachment may lead one to suspect that patients with some degree of situs inversus may be subject to a higher incidence of cecal vo1vu1us, this has not been reported. The two requirements for volvulus of any organ, namely, excessive mobility and a fixed point around which to rotate, may be exaggerated in a situation of partial situs inversus with incomplete rotation of the intestine. The cecal mobility usually occurs as a consequence of the cecum sharing a common ileocecal mesentery. Postmortem studies have shown that this condition by itself is present in 11 to 22 per cent of the normal population [22,23]. Other forms of complete malrotation or abnormal fixation of the ascending colon by transverse bands, the so-called Jackson’s membranes, may occur in association with cecal vol~ulus . In addition, a number of
nonrelated factors may be involved leading to some degree of colonic distention as the acute event precipitating the volvulus. These can include: (1) distal colonic obstruction, such as that due to cancer, diverticulitis, hernia, or impaction; (2) adhesive bands associated with a previous operation or, occasionally, previous appendicitis; (3) paralytic ileus; (4) aerophagia associated with advanced obstructive pulmonary disease or, occasionally, with excessive use of intermittent positive pressure ventilation; (5) excessive peristalsis associated with diarrhea or purgation; (6) intra-abdominal displacement or violent muscular contractions such as those occurring with pregnancy and parturition; (7) dietary factors, such as a high. roughage diet, possibly explaining to some extent the geographic distribution of the condition, it being more common in Central and Eastern European countries, Russia, Finland, and India . A careful history and physical examination will often suggest the presence of cecal VO~VU~~S,but roentgenograms of the abdomen are essential and often diagnostic. McGraw, Kreman, and Rigler  list the most characteristic features as follows: (1) gross cecal distention, usually in an ectopic position, particularly in the left upper quadrant (With axial rotation of the bowel, however, marked dilatation with no apparent displacement may occur.); (2) distended small bowel loops, most often located to the right of the cecum; (3) possible visualization of a gas-outlined ileocecal valve located to the right of the cecum; (4) spiral contour of the intestinal mucosal folds at the area of obstruction; and (5) a single air-fluid level most often in the left upper quadrant. This is in contrast to the double air-fluid level in the right upper quadrant found with sigmoid volvulus. In addition, the concavity of the “coffee-bean” deformity in cecal volvulus is most often directed toward the right lower quadrant as opposed to the left lower quadrant as in sigmoid volvulus. Although it is unusual for all of the classic signs to be present on routine radiographs of the abdomen, the suspicion of volvulus should alert the physician to the need for further studies. If the patient’s condition is such that it can be tolerated, a low pressure barium enema is the most satisfactory way to establish the correct preoperative diagnosis. This should always be preceded by sigmoidoscopy, however, to rule out the presence of a lowlying obstructive lesion. The reported case represents a variation of situs inversus with reversal of the normal position of the stomach, duodenum, and jejunum together with
The Amerlcan Journal of Surgery
of the normal positioning of the lower bowel. The presence of only a rudimentary spleen and a symmetrical liver with reversed biliary structures has previously been associated with situs inversus viscerum (161. Because of the acute surgical nature of the problem, assessment of the exact anatomic relationship at the time of surgery presented several confusing problems. In the typical case of cecal vo1vulus, initial assessment to determine the presence or absence of gangrenous bowel will dictate subsequent therapy. If the cecum is obviously gangrenous, right hemicolectomy is usually the most satisfactory treatment (without detorsion to prevent the toxic substances in the necrotic bowel from being absorbed) [20,21,24]. If the patient’s condition is too poor to tolerate colectomy, exteriorization has occasionally been recommended although most agree that this is not optimal therapy . If there is extensive distention of the proximal small bowel, a third alternative is resection with construction of a double-barrelled ileocolostomy to provide decompression. If the situation is less critical and the bowel is obviously viable after detorsion, several surgical alternatives are available including cecostomy, cecopexy, cecostomy and cecopexy, or right hemicolectomy. Although some suggest that cecostomy alone is usually inadequate to prevent recurrence [20,26], this has not always been true in every case. The use of two cecostomies to obtain two-point fixation of the cecum has also been suggested . Cecopexy is designed to prevent recurrence by total fixation of the cecum to the lateral abdominal wall. It may be performed by multiple sutures or by the more elaborate technic of burying the cecum under a peritoneal flap. Definitive right hemicolectomy may at times be the treatment of choice even when the bowel is obviously viable [20,24]. Unfortunately, there is no clear evidence in the literature to recommend one form of treatment over the other. It is clear, however, that unless the volvulus is caused by simple adhesions or bands, detorsion alone is probably inadequate to prevent recurrence [20,21,24,28]. retention
Summary An unusual case of cecal volvulus in a patient with partial situs inversus viscerum is presented. The associated conditions of liver symmetry and splenic dysgenesis were also present. The patient claimed to be a conjoined twin at birth. The association of twinning and situs inversus is discussed as are some of the lesser appreciated physical find-
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ings associated with the phenomenon. Treatment of the cecal volvulus in this case required consideration of a number of operative alternatives. References 1. Gould DM: Non-tubercutous lesions found in mass x-ray surveys. JAMA 127: 753, 1945. 2. Chokt MR: Discrepancies in pain and symptom distribution: position of the testicles as a diagnostic sign in situs inversus totali. Am J Surg73: 104. 1947. 3. Ebstein: Cited by Mandelstamm M, Reinberg SA [ 41. 4. Mandelstamm M. Reinberg, SA: Rontgenologische and electrokardiographische Untersuchungen uber ihre verschiedenen Typen. Ergeb inn Med Kinderheilkd 34: 154, 1928. 5. Farkas A: Physiologic scoliosis. J Bone Joint Surg [Am] 23: 607.1941. 6. Adams R. Churchill E: Siius inversus, sinusitis, bronchiectasis. J Thorac Cardiovasc Surg 7: 206, 1937. 7. Overholt E, Bouman D: Variants of Kartagener’s syndrome in the same family. Ann Intern Med48: 574, 1958. 8. Olsen A: Bronchiectasis and dextrocardia. Am Rev Tuberc 47: 435, 7943. 9. Kartagener M: Zur Pathogenese der Bronchiektasien; Bronchiektasien bei Situs viscerum inversus. Beitr K/in Tuberk 83: 489, 1933. 10. Varano N, Merklin R: Siius inversus. J lnt Co// Surg 33: 131, 1969. 11. Newman HH: The question of mirror-imaging in human oneegg twins. Hum Bioll2: 21, 1940. 12. Aird I: The conjoined twins of Kano. Br A&d J 1: 831, 1954. 13. Torgensen J: Concordant situs inversus in dizygotic twins. J Hered39: 293, 1948. 14. lvemark BI: Implications of agenesis of the spleen on the pathogenesis of cone-truncus anomalies in chikfhood. Acta Paedtatr Stand 44 (Suppl 104): 1, 1955. 15. Campbell M, Deuchar DC: The left sided superior vena cava. Br Heart J 16: 423, 1954. 16. Campbell M, Deuchar DC: Absent inferior vena cava, symmetrical liver, splenic agenesis and situs inversus, and their embryology. Br Heart J 29: 268, 1967. 17. Kilcoyne RF: Siius inversus: a review. Marquette Med Rev 29: 90, 1963. 18. Wood GA, Blalock A: Situs inversus totalis and disease of the biliary tract: survey of the literature and report of a case. Arch Surg 40: 885, 1940. 19. Fonkakrud EN, Tompkins R, Clatworthy HW Jr: Abdominal manifestations of situs inversus in infants and children. Arch Surg 92: 79 1, 1966. 20. Villet DD: Volvulus of the proximal colon. Ann Surg 150: 1075, 1959. 21. Powell JH, Bowers RF: Volvulus of the cecum. Ann Surg 143: 126, 1956. 22. Wolfer JA, Beaton LE, Anson BJ: Volvulus of the cecum: anatomical factors in its etiology. Surg Gynecol Obstet 74: 882, 1942. 23. Donhauser JL, Atwell S: Volvulus of the cecum with review of 100 cases in the literature and report of six new cases. Arch Surg58: 129, 1949. 24. Dowling BL, Gunning AJ: Caecal volvulus. Br J Surg 56: 124, 1969. 25. McGraw JP, Kremen AJ, Rigler LG: The roentgen diagnosis of volvulus of the cecum. Surgery24: 793, 1948. 26. Nelson TG, Bowers WF: Volvulus of the cecum and sigmoid colon. Arch Surg72: 469, 1956. 27. Barry F: Personal communication, 1973. 28. Krippaehne WW, Vetto M, Jenkins LL: Volvulus of the ascending colon: a report of twenty-two cases. Am J Surg 114: 323, 1967.