Mesenteric and Retroperitoneal Cysts

ROBERT J. KURTZ, M.D. TOMAS M. HEIMANN, M.D. A. ROBERT BECK, M.D. JAMES HOLT, M.A.*

Mesenteric and retroperitoneal cysts are rare intra-abdominal From the Department of Surgery and the Department of tumors. Ten new patients are presented as well as 152 other Biomathematics, * Mount Sinai School of Medicine, cases reported in the English literature. These 162 cases were City University of New York, New York, New York then analyzed for significant trends. Patients under 10 years of age were significantly different from the older group with respect to a shorter duration of symptoms, a higher number of patients requiring an emergency operation, a lower number of recurrences to the present. This approach allows the analysis ofa large and the location of the cyst. Patients with retroperitoneal cysts number of cases for trends or other factors that might were more likely to have incomplete excision of the cyst and help the clinician in the diagnosis and treatment ofthese therefore had a higher incidence ofrecurrence. They also required conditions as well as leading to a better understanding of marsupialization more often. Retroperitoneal cysts should be the problems related to the management of these rare considered a different entity from mesenteric cysts even though they present clinically in a similar fashion. The outcome of sur- diseases. gical treatment is less satisfactory in patients with retroperitoneal cysts. Incidence The medical records of all patients ,M 'ESENTERIC CYSTS are rare admitted to the intra-abdominal Mount Sinai Hospital from 1950 to 1984 were reviewed. masses. This entity was first described in 1507 Ten by the Florentine anatomist patients with mesenteric or retroperitoneal cysts Benevieni' while were identified in this 35-year period. performing an autopsy on an During this time, 8-year-old boy. It was not there were approximately until 1842 that Rokitansky2 recorded the first 1,064,000 admissions to the description Mount Sinai Hospital, ofwhich approximately 10% were ofa chylous cyst and in 1852 Gairdner3 described the first omental cyst. pediatric admissions. The estimated incidence of 10 cases Finally, in 1880 Tillaux4 performed the first per 1,064,000 admissions successful on (1/106,400) at our institution operation a cystic mesenteric tumor. This is similar to the was followed by who in 1883 previously reported incidences, which Pean,5 described the first range from eight cases per 820,000 marsupialization of a mesenteric tumor. (1/102,500) admissions Because at the Mayo Clinic8 to three cases per 750,000 (1/250,000) mesenteric and retroperitoneal cysts are seen admissions9 at the so infrequently there is very little information available University of Mississippi Medical on them.6 Most Center. published studies consist of only a few The incidence cases. This has led to some in this study in the pediatric age group false impressions and conclu- (less than 10 years old) is five cases sions regarding these tumors of the abdomen. The rarity per 101,000 (1/20,200) of these conditions has admissions. This figure is also in agreement with reports also contributed to the fact that from Los Angeles Children's the correct preoperative diagnosis is infrequently made.7 Hospital of three cases per For these 12,425 (1/4,142) admissions5 and Children's Hospital of reasons, the authors have undertaken the review Akron with two cases of their own institution's records but have also reviewed per 68,750 (1/34,375) admissions.'0 all the cases in published the English literature from 1950 Clinical Material Ten patients with mesenteric or retroperitoneal cysts Reprint requests: Robert J. Kurtz, M.D., Department of Surgery, Mount Sinai School of Medicine, One Gustave Levy Plaza, New York, treated at our institution from 1950 until 1984 are re- NY 10029. ported. The diagnosis was proven in all cases at laparot- Submitted for publication: August 19, 1985. omy. Five patients were male and five were female. They

109 Ito KURTZ AND OTHERS Ann. Surg. * January 1986 itoneal cysts. One ofthese patients required an emergency operation for acute onset of abdominal pain. Operative treatment consisted of complete excision of the cyst in six cases (see Fig. 1), and in the remaining four the cyst was only partially excised. Three of these four patients had retroperitoneal cysts. Three patients required resection ofa portion ofthe small intestine and in all ofthese cases the reconstruction was performed by an end-to-end anas- tomosis. None of the cysts was malignant. They ranged in size in the eight patients in whom this information is known from four to 36 cm in diameter. The estimated volume of the cysts ranged in these patients from 11 to 5600 cc. Only one patient developed a recurrence of the cyst and required a second operation. This patient had a retroper- itoneal cyst, which was partially excised at the first op- eration (see Fig. 2). She is doing well 7 years after mar- supialization of the cyst, despite the persistent drainage FMG. 1. Appearance ofa large mesenteric cyst that was completely excised. of fluid.

of ranged in age from 6 months to 68 years with a mean of Analysis Published Cases 22. Five ofthe patients were 10 years old or younger. The All cases of mesenteric and retroperitoneal cysts re- average age of the patients with the retroperitoneal cysts ported in the English literature from 1950 until the present was 33 years, while the average age of the patients with were reviewed. The data obtained were analyzed using mesenteric cysts was 15 years. the computer services ofthe City University ofNew York. The average duration of symptoms before hospitaliza- Student's t-test was used to determine statistical signifi- tion was 2.6 months with a range of from 12 hours to 12 cance for the numerical data and a chi square or Fisher's months. Eight patients presented with abdominal disten- exact test analysis was employed to compare data grouped tion, and nine had abdominal pain. Their temperature into categories. Only cases described with sufficiently de- on admission ranged from 37.0 to 39.3 C with a mean of tailed information were included in this study. One 37.5 C. hundred sixty-two patients were found, including the 10 The correct preoperative diagnosis was made in only patients being presented. Forty-eight per cent of the pa- three cases, and two ofthese were patients with retroper- tients were females. The mean age was 25 years, with a range from 1 day to 83 years. The duration of symptoms ranged from 3 hours to 6 years, with a mean of nearly 6.6 months. Abdominal pain was present in 58% of the cases and abdominal distention in 50%. The correct pre- operative diagnosis was made in 30 of the 122 patients (25%) in whom this information is known. Ofthe 60 cases for whom this information is known, 45% were operated on as emergencies. The cyst was located in the small bowel mesentery in 91 patients (60%), in the large bowel mesentery in 37 (24%), and in the retroperitoneum in 23 (14.5%). In 11 patients the location was not given more specifically than in the mesentery, and in 11 cases the location was not described. A simultaneous intestinal resection was per- formed in 56 ofthe 134 patients in whom this information is known. In 37 cases a resection of the small intestine was performed. In three cases a right hemicolectomy was necessary with an ileotransverse anastomosis. There was FIG. 2. Computerized tomograph showing a large retroperitoneal cyst at the level of the kidneys. The cyst has been marsupialized and contains one case each requiring resection of the transverse and air and fluid. the sigmoid colon. Vol. 203 * No. I ABDOMINAL CYSTS III The average estimated volume ofthe cyst was 2114 cc TABLE 1. Statistically Significant Differences between the Patients with Retroperitoneal and Mesenteric Cysts with a range of from 4 to 24840 cc. The cysts ranged in diameter from 2 to 36 cm, with a mean of 13.2 cm. The Retroperitoneal Mesentery p cyst was completely excised in 128 patients and partially Total number of patients 23 139 excised in 15. In 14 patients the cyst was marsupialized. Average age (years) 40 (23)* 23 (139) <0.001 Five patients were found to have malignant cysts. Ten Number of emergency cysts recurred, requiring a second operation. Five ofthese operations 0(5) 27 (54) <0.05 Number of second recurrences occurred in the 23 patients (22%) whose cysts operations 9 (23) 6 (133) <0.001 were retroperitoneal in location (p < 0.001). There were Number of recurrences 5 (21) 5 (119) <0.001 three deaths (2%). Number cysts completely excised 12 (22) 116 (121) <0.001 The 162 patients were divided into two groups. Patients Number of cysts below the age of 10 years numbered 64 and those in the marsupialized 10 (22) 4 (116) <0.001 older group numbered 98. The mean ages were 3.9 and Number of bowel resections 0 (20) 56 (114) <0.001 39.1 years, respectively. Sixty-three per cent ofthe patients in the younger group were males, while only 38% were * Total number of patients in whom this observation is known. male in the older group (p < 0.01). The duration ofsymp- toms was 2.2 months and 9.8 months, respectively (p < was in 61% ofthe 0.01). Abdominal distention present The 162 patients were also divided in two groups based younger group and 47% of the older group (p = NS). on the location of the cyst. There were 23 patients with Abdominal pain was present in approximately 62% of retroperitoneal cysts and 139 with mesenteric cysts. The in each group. The correct preoperative diagnosis patients statistically significant differences are listed in Table 1. was made in 11 of48 patients (23%) in the younger group Two ofthe five malignant cysts were in the retroperitoneal and in 19 of 74 (26%) in the older group (p = NS). group. The volume and diameter of the retroperitoneal cyst was completely excised in 95% ofthe patients The cysts were 2331 cc and 13.5 cm, while the mesenteric in younger group and 86% in the older group (p = NS). the cysts were 2080 and 13.2, respectively. The correct di- In younger group 18 ofthe 29 patients (62%) for whom the agnosis was made before operation in about 22% of each this information was known were treated as surgical group. Abdominal pain was present in 43% ofthe patients while nine of the 30 (30%) in emergencies, only patients with retroperitoneal cysts and in 51% ofthe patients with < were so treated. the older group (p 0.01) mesenteric cysts. Similarly, the incidences of abdominal The locations of the cysts in the younger group were pain were 49 and 60%, respectively (p = NS). different from those in the adults. In 45 ofthe 58 patients (78%) who were under the age of 10 for whom this infor- Discussion mation is known, the cyst was located in the small bowel mesentery, in nine (15%) in the large bowel mesentery, There are no pathonomonic signs or symptoms for ret- and in four (7%) it was retroperitoneal. Similarly, for the roperitoneal or mesenteric cysts."I'3 The clinical history older group, in 47 (51%) ofthe 92 patients for whom this and findings on the physical examination may suggest the information is known, the cyst was located in the small possibility of a cyst, but usually the diagnosis is made by bowel mesentery, in 28 (30%) in the large bowel mesen- the exclusion of other diagnoses.'3 Abdominal pain and tery, and in 18 (19%) it was retroperitoneal. This difference abdominal distention were each present in more than 50% in distribution was statistically significant (p < 0.01). A ofthe cases. Although these findings are nonspecific, when bowel resection was done in 29 of the 51 patients (57%) the patient also presents with a long duration ofsymptoms in the younger group and in 27 of the 83 patients (33%) (6.6 months), the clinical diagnosis of a cyst should be in the older group (p < 0.01). The cyst was marsupialized considered. The correct preoperative diagnosis was made in 5% ofthe patients in the younger age group and in 15% in only 25% of the reported cases. With a high index of of the older patients (p = NS). There was only one re- suspicion and the use ofultrasonic imaging and computed currence in 55 patients (2%) in the younger group, while tomography, the correct diagnosis should be made in there were nine recurrences out of 84 patients (11%) in nearly every case. the older group (p < 0.05). The cysts in the younger group There were approximately equal numbers ofmales and had a mean diameter and volume of 13.0 cm and 1732 females in the study when all the cases were considered. cc, while the results for the older group were 13.3 and When the patients were divided into the two groups based 2300, respectively (p = NS). There were three surgical on age, there was a statistically significant increase in the mortalities in the older group of patients (p = NS). All number of male patients in the younger group. There five of the patients with the malignant cysts were in the seems to be no obvious reason for this difference. older age group (p = 0.06). The surgical treatment of choice for retroperitoneal, 112 KURTZ AND OTHERS Ann. Surg. .January 1986 and mesenteric cysts is complete enucleation.'5",6 If this data whether malignant cysts originate from previously cannot be accomplished, then the next best alternative benign cysts or arose de novo. would be excision of the cyst with, if necessary, the re- In conclusion, retroperitoneal cysts and mesenteric cysts section of a portion of the adherent bowel. The last ac- should be regarded as separate clinical entities. They are ceptable choice would be marsupialization of the cyst. significantly different with respect to complications and Partial excision or simple drainage ofthe cyst usually re- recurrences. Retroperitoneal cysts are often incompletely sults in a recurrence. excised and require multiple operations. These cysts are There is a statistically significant correlation between more likely to require marsupialization. This treatment the recurrence of the cyst and location. Five of the 10 is less than ideal and results in a continuously draining recurrences occurred in the patients with retroperitoneal fistula. Whenever possible, a complete excision should be cysts. There is no correlation between age, duration of performed. symptoms, diameter, or volume of the cyst, and recur- rence. There were, however, no emergency operations References done in the group of statistically older patients with the 1. Braquehage J. Des kystes du mesentery. Arch Gen 1892; 170:291. retroperitoneal cysts. Retroperitoneal cysts are technically 2. von Rokitansky CF. Handbuch der Pathologischen anatomie. bd 2 more difficult to excise completely because oftheir prox- Handbuch der Speciellen Pathologischen Anatomie. Wein: imity to major blood vessels and other organs, and, there- Braumuller and Seidel, 1842. 3. Gairdner WT. A remarkable cyst in the omentum. Transactions of fore, a recurrence is much more likely. Furthermore, the the Pathological Society of London 1851; 3:185 1. borders of these retroperitoneal cysts are not as well de- 4. Tillaux PJ. Cyste du mesentere un homme: ablation par la gastromie: fined. These technical difficulties are the probable expla- quersion. Revue de Therapeutiques Medico-Chirurgicale Paris 1880: 47:479. nation for the statistical difference between these two 5. Slocum MA. Chylous mesenteric cyst. Am J Surg 1938; 41:463-73. groups with respect to the number of second operations, 6. Barr WB, Yamashita T. Mesenteric cysts. Review of the literature recurrences, rate of marsupialization, and the number of and report of a case. Am J Gastroenterol 1964; 41:53-57. 7. Hardin WJ, Hardy JD. Mesenteric cysts. Am J Surg 1970; 119:640- bowel resections performed. There was only one death 645. reported in patients with retroperitoneal cysts. 8. Judd ES, Crisp NW. Proc Staff Meet Mayo Clinic 1932; 7:555. of the younger patients in this series shows 9. Vaughn AM, Lees WM, Henry JW. Mesenteric cysts. Surgery 1948; Analysis 23:13-19. that they have a shorter duration of symptoms and are 10. Steinreich OS. The diagnosis of mesenteric cysts. Ann Surg 1955; more likely to present as surgical emergencies. Although 142:889-894. the average size and volume of the cysts are identical for 11. Parsons EO. True proliferating cystic lymphangomioma ofthe mes- entery. Ann Surg 1936; 103:595-604. both groups of patients, there were fewer retroperitoneal 12. Warfield JO. A study of mesenteric cysts. Ann Surg 1932; 96:329- cysts in the younger group. This resulted in a higher re- 339. sectability rate and a significantly lower recurrence. 13. Beahrs OH, Judd ES, Docherty MD. Chylous cysts ofthe abdomen. Surg Clin North Am 1950; 30:1081-96. The incidence of malignant cysts was 3%. All cancers 14. Razkowski HJ, Rehbock DJ, Cooper FG. Mesenteric and retroper- were found in adults. There was no difference in the du- itoneal lymphangioma. Am J Surg 1958; 97:363-67. or ofthese patients. Also, 15. Vanek VW, Phillips AK. Retroperitoneal, mesenteric and omental ration ofsymptoms presentation cysts. Arch Surg 1984; 119:838-842. size and location of the malignant cysts was identical to 16. Capresso PR. Mesenteric cysts: A review. Arch Surg 1974; 108:242- the benign cysts. It is not possible to tell from the reported 246.