Chief Resident General Surgery Department of Surgery North Shore Long Island Jewish Medical Center

								Chief of Trauma and Critical Care and Attending Surgeon Department of Surgery North Shore University Hospital North Shore Long Island Jewish Health System Manhasset, NY

	Rotational gut abnormalities are rare in adults, but the most common of such abnormalities in this population is midgut volvulus. Patients generally report significant abdominal pain, and prompt diagnosis is imperative for preventing potentially life-threatening complications. Ultrasonography and computed tomography (CT) scanning are two useful modalities for visualizing this abnormality. We report the case of a pa?tient whose mesenteric volvulus was di?agnosed on CT scanning and successfully treated with lysis of the adhesion and a prophylactic appendectomy.

	A 50-year-old white woman came to the emergency department several hours after dinner with severe right lower quadrant pain. The symptoms were much more severe before her arrival at the hospital. She reported no recent changes in her bowel movements, no nausea or vomiting, and she had passed flatus. On physical examination her abdomen was not distended, bowel sounds were present, and the abdomen was soft. Tenderness was elicited in the right lower quadrant without rebound or guarding on palpation. She had no fever and her laboratory re?sults were normal. Her medical history was significant for bilateral breast im?plants and a total abdominal hysterectomy due to painful fibroids.

?A CT scan of the abdomen and pelvis showed a normal appendix, but there was gastric distention with debris indicating evidence of gastroparesis. A whorled pattern of the superior mesenteric vein (SMV) around the axis of the superior mesenteric artery (SMA) was observed (Figure), consistent with a partial bowel malrotation with volvulus.

?The patient elected to have an exploratory laparotomy 48 hours later. She was found to have a viable loop of small bowel in the right lower quadrant twisted around an adhesion from the pelvis. Lysis of the adhesion and a prophylactic appendectomy were performed.

?The appendix contained a carcinoid tumor and there was fibrous obliteration of the lumen. Micro?path?ology showed a tumor measuring up to 0.4 cm invading the muscularis propria up to the outer layer.

 In Western countries, the annual oc?currence of small bowel volvulus ranges from 1.7 to 5.7 in 100,000 of the population compared with 24 to 60 in 100,000 of the population in Asia or Africa.

 Rotational gut abnormalities are generally found in pediatric populations, but may be encountered in symptomatic adults with serious outcomes. Although symptomatic bowel malrotation in adults is rare, midgut volvulus is the most common complication of malrotation in adults.

	Several etiologic factors have been proposed. An increased incidence of the disorder has been described among Muslims in Afghanistan during the Ramadan festival, possibly from ingesting large quantities of fiber after fasting.

 In Turkey, small bowel volvulus constitutes 8% of all cases of mechanical in?testinal obstruction and 13% of all small bowel obstructions.

	In Western countries, volvulus of the small bowel is more common in patients with acquired predisposing factors such as malrotation, congenital bands, postoperative adhesions, Meckel's diverticulum, internal hernia, and pregnancy.

 The proposed mechanism of volvulus is secondary to a small bowel loop fixed at two points that twists around its mesentery.

	The most common symptom is central abdominal pain. Laboratory tests are un?reliable; however, a white blood cell count above 18,000/mm

, high lactate and amylase levels, and metabolic acidosis are present in over 50% of patients with strangulated bowel.

	Ultrasonography can be the first mo?dality used to check for midgut volvulus because it will show if there is reversal of the normal relationship between the SMA and SMV, which can be seen as a clockwise whirl sign. When ultrasonography findings are inconclusive, a CT scan should be next in the diagnostic workup.

 The midgut is supplied by the SMA, and when the small bowel is wrapped around this artery, it creates a distinctive whirl sign on CT scanning (Figure, panel C).

 The SMV is normally anterior and to the right of the SMA; thus, the SMV positioned to the left of the SMA can suggest bowel malrotation.

	Since small bowel volvulus is uncommon, ischemia or infarction can result from a delay in diagnosis. The cardinal presenting symptom is abdominal pain. There is no specific diagnostic clinical sign or abnormality in laboratory or radiologic findings. The diagnosis can only be confirmed by laparotomy, and failure to perform exploratory laparotomy cannot be justified. Whereas some authors document successful eneteropexies in managing this disorder, others recommend resection and primary anastomosis for all small bowel volvulus cases, with an overall mortality rate of 10% to 35% following resection.

 Whichever intraoperative decision is made, it has been shown that early diagnosis and early ex?ploratory laparotomy are keys to successful management.

	Mesenteric volvulus is an uncommon but potentially life-threatening condition. Little information is available for the recommended treatment of viable bowel, and no information is available for suggested elective treatment of mesenteric volvulus. Because laboratory tests are unreliable, one must have a high index of suspicion when a patient presents with abdominal pain. Ultrasonography and CT scanning are useful modalities for diagnosing midgut volvulus. Once the diagnosis of mesenteric volvulus is made, prompt surgical intervention is required to either lyse adhesions, detorse the bo?w?el, or formally resect and anastomose the bowel if it is found to be ischemic.

	1. Gulati SM, Grover NK, Tagore NK, et al. Volvulus of the small intestine in India. Am J Surg. 1973;126(5):661-664.

	2. Shaw-Nan J, Hsein-Jar C, Yung-Fang C, et al. Midgut vol?vulus with acute abdomen in an adult patient: a case report. Chin J Radiol. 2003;23:37-41.

	3. Duke JH Jr, Yar MS. Primary small bowel volvulus: cause and management. Arch Surg. 1977;112(6):685-688.

	4. Gurleyik E, Gurleyik G. Small bowel volvulus: a common cause of mechanical intestinal obstruction in our region. Eur J Surg. 1998;164(1):51-55.

	5. Matsuki M, Narabayashi I, Inoue Y, et al. Two adult cases of primary small bowel volvulus: usefulness of computed tomographic diagnosis. Radiat Med. 1997;15(3):181-183.

	6. Iwuagwu O, Deans GT. Small bowel volvulus: a review. J R Coll Surg Edinb. 1999;44(3):150-155.

	7. Bizer LS, Liebling RW, Delany HM, et al. Small bowel obstruction: the role of nonoperative treatment in simple intestinal obstruction and predictive criteria for strangulation obstruction. Surgery. 1981;89(4): 407-413.

	8. Mucha P Jr. Small intestinal obstruction. Surg Clin North Am. 1987;67(3): 597-620.

	9. Janssens F, Verswijvel G, Smits J, et al. Midgut volvulus in an adult patient. JBR-BTR. 2003;86(2):74-76.

	10. Kreisler-Marino E, Reid ME, Gordon PH. Novel operative management of primary mesenteric volvulus. Dig Surg. 2001;18(3): 243-245.

	11. Roggo A, Ottinger LW. Acute small bowel volvulus in adults. A sporadic form of strangulating intestinal obstruction. Ann Surg. 1992;216(2): 135-141. 

Articles

Surgical Rounds

Arie E. Pelta, Chief Resident General Surgery, Department of Surgery, North Shore Long Island Jewish Medical Center; Jay Yelon, Chief of Trauma and Critical Care and Attending Surgeon, Department of Surgery, North Shore University Hospital, North Shore Long Island Jewish Health System, Manhasset, NY

Mesenteric Volvulus Presenting as Rightlower Quadrant Pain

	Fund giant Fidelity recently slashed the annual expenses on its big index funds to 0.10% of assets. In comparison, the Vanguard 500 Index charged 0.18% of assets, according to an article in 

. During the first 8 months of 2004, the $97-billion Vanguard 500 Index took in $3 billion in new investments.

	Should we get ready for index mutual fund price wars? Not exactly, but the growing popularity of these investment vehicles has some competing fund companies taking serious steps. But before you invest your money in a fund that simply mirrors the S&P 500 Index, surgeon investors should be aware of a few things.

	Fees outweigh gains?Over the past 10 years, the S&P 500 has averaged ?annualized returns of 10.7%, compared with 8.6% for actively managed large-cap funds, and have averaged 12.8% and 11.1%, respectively, over the past 20 years. A reason for the difference is that index funds buy and hold stocks within the index they track, which makes them less expensive to run. Most blue-chip funds take an average of more than one percentage point a year out of returns before they make a trade.

	According to a study conducted by the Consumer Federation of America and Fund Democracy, not all index funds are created equal. The average S&P 500 Index fund charges 0.82% or more than some actively managed funds, which can make a big difference even in a short period of time. Suppose you invested $20,000 into the class A shares of the Morgan Stanley S&P 500 Index in 1997, which charges 0.70%?plus an up-front sales load. You would have about $1,100 less today ($24,620 versus $25,720) than if you had invested the money in the Vanguard 500 Index.

	Pay more for the same?Although you may be paying extra for an active fund manager, you might end up owning the same companies that are in the index. According to Money, when funds do well and attract more cash from investors, they have to buy more stocks. Invariably, investors end up owning many of the big stocks that are in the S&P 500 Index and end up pay-ing more for an actively managed fund that bears a strong resemblance to an index fund.

	Keep in mind, however, that the S&P 500 represents only 77% of the value of all US stocks. There are approximately 4,500 smaller companies to be considered. The Vanguard Total Stock Market Index tracks the Dow Jones Wilshire 5000, which includes both small- and large-cap companies. These funds offer maximum diversification from a single investment source.

	As risky as the sector?Because in?dex funds in many ways mirror the market segments they follow, they are also susceptible to risk. As the article explains, companies listed on the S&P 500 automatically assume a bigger portion of the index as their market value increases. The more popular a stock is, the more of it an index fund will own.

	For example, when the tech and telecom bubble burst in 2000, the Van?guard 500 Index had 39% of its as?sets in tech-type stocks, contributing to a 37% drop in value over the next 2 years. Similarly, by the time the market bottomed out in 2002, the fund held only 16% of its assets in those areas, proving that an index fund is generally no safer than the sector it follows.

	It's important to remember that while investing in index funds has its advantages, there are no guarantees. Doing your homework prior to making any investment decision is the best course of action.?Ed Rabinowitz

 Hoping to catch income and capital appreciation, many people in today's uncertain stock market chase after performance like a dog after its own tail. With interest rates still near 40-year lows, where can surgeon investors turn to fight off inflation, the bane of investing, and still garner outstanding returns? Authors Don Schreiber Jr. and Gary E. Stroik maintain that the only constant in investing is dividends, and their book 

All About Dividend Investing: The Easy Way to Get Started

 (McGraw-Hill; 2004) provides compelling evidence for this often forgotten investment medium. A distribution of a company's earnings, dividend-paying stocks have garnered superior performance for over a century?despite stock market highs and lows?while reducing short-term risk and maximizing long-term growth. With a combined investing experience of over 40 years, Schreiber and Stroik find that dividend stocks are the glue that holds a portfolio together and work for just about any investor at any stage of investing by lowering risk, providing growth, and steadily increasing over time. This step-by-step guide helps in?vestors identify underpriced dividend-paying companies, choose dividend ratios, and then assemble the best picks into a solid and successful dividend portfolio.

	Can you imagine throwing away over $7,000 per year over the next 4 years? An increasing number of parents with college-bound kids cannot either. With average 2004?2005 room and board costing $7,434 at private 4-year colleges and $6,222 at public 4-year colleges, according to the College Board, some parents are investing in the future of their children's education through real estate. Low interest rates and flexible financing have provided an opportunity for 6% of investment buyers to purchase a second home for their college student, according to the

. Buying a condo or townhouse allows your child to pick their own roommate, someone who potentially helps offset housing costs, and provides a tax write-off for parents. Although many wait until sophomore year of college, some parents start as early as grade school in the hope that their child will attend a college in a particular area. 

: If you purchase a property before your child is in college, there is no guarantee that they will want or will be able to attend the college you choose for them. Also, many colleges require first-year students to live on campus. Your best bet may be to wait until your child is a student, and then decide if purchasing a second home is the right investment for you.

Strategies to lower homeowner's insurance 

	Although you may not have much control over skyrocketing malpractice premiums, you can lower your homeowner's insurance premiums by as much as 10% to 20%, according to the AAII (Ameri?can Association of Individual Investors) Journal. Consider the below strategies:

	Ask for a discount?Factors such as proximity to a fire station, type of building materials used, and retiree status may qualify you for a discount. Many insurers also reward customer loyalty of 5 or more years with lower rates.

	Look for a package deal?Many com?panies offer a discount if you purchase multiple policies, such as homeowner's, auto, and life. Shop around first: separate policies from different in?surers may be a better deal.

	Don't insure your land?Unless your home is on a cliff and the ground falls out from underneath it, your land's value will remain constant?it's the building that you need to worry about. Insure your house and its contents only against theft, fire, hurricanes, and other natural disasters.

	Install safety devices?Smoke detectors, burglar alarms, fire and police alarms, and deadbolt locks function as double-duty safety and discount devices, according to many insurance carriers.

	Up the ante?Reduce your overall insurance premium by assuming more risk. Keep in mind that raising your de?ductible means that you cannot make claims for smaller items and will have to pay for repairs out of pocket.

Wedding day frocks adorned with credit score

 Before taking a walk down the aisle and into wedded bliss, soon-to-be brides and grooms should first sit down and settle their financial issues together. According to 

, Jim Trippon of Trippon Wealth Management LLC states that couples need to do the following before making a commitment: understand each other's financial goals; see each other's credit score and report; and create a manageable written budget. Although such topics may seem unromantic to couples on the verge of exchanging vows, the Illinois Certified Public Accountants Society in Chicago urges engaged pairs to complete a 10-point financial compatibility quiz, which can help foster awareness of what finances and financial habits individuals will be brining into a marriage. InvestmentNews also states that young couples need to understand that dividing assets in a divorce is more difficult than discussing money before marriage. Although a prenuptial agreement may seem like a good idea, Trippon says they create friction. Instead, he advises his wealthy clients to set up separate trusts for individual assets, which is a simpler and cleaner method of managing premarital finances. When discussing a sensitive topic such as money, it's always best for couples to seek out the guidance of a neutral and objective third-party professional, such as an accountant or financial planner.

 Save on college with reciprocity agreements 

	If your college-bound child has their heart set on studying entomology, but your state college does not offer the program, don't be bugged that you may have to pay the high costs of out-of-state tuition in order to fulfill your child's dream. Instead, see if your state has reciprocity agreements with interstate institutions of higher learning. The Acade?mic Common Market, a reciprocity agree?ment among the 16 states that comprise the Southern Regional Academic Board (

), offers students the op?portunity to study one of 1,400 un?dergraduate or graduate programs that their state may not offer. Institutions grant an out-of-state waiver annually for students who qualify and are accepted in?to a school's program. The Midwest Higher Exchange Compact (

) of?fers a Midwest Student Exchange Pro?gram for six of its 11 participating states. Public colleges and universities charge out-of-state students no more than 150% of the in-state resident tuition rate for specific programs. For example, if a particular public college offers instate students a tuition of $10,000 and out-of-state students a tuition of $30,000, this program would ensure that out-of-state students eligible for the program would pay a tuition no higher than $15,000. For private institutions a 10% reduction is offered. Similar programs exist for other states, including the New England Regional Student Program offered by the New England Board of Higher Ed?ucation (

) and the West?ern Interstate Commission for Higher Education (

), which of?fers three programs: the Western Un?dergraduate Exchange, the Western Regional Grad?uate Program, and the Professional Student Exchange Pro?gram. The only states that do not offer reciprocity agreements are South Dakota, Rhode Island, Delaware, New Jersey, New York, and Pennsylvania.

	Every bargain-hunting surgeon has heard retail stores touting the amazing deal of no-interest payments for a whole year and similar incentives. According to BankRate.com, while this may sound like a great buy, there are some serious caveats. If you are able to pay off your purchase within a year without paying any interest, then it is a great deal. But if you take your time to make the payments and the interest kicks in, you may be in a bind. At that point, the finance company applies the interest retroactively effective to your purchase date, which can get very expensive. The buy now, pay later campaign is also problematic. If you're taking advantage of such an opportunity because you can't afford the item right now, how can you be so sure you can afford it when the payments start? It's best to hold off the purchase until you can save the money to buy it outright. If you can pay for the item at once, you avoid the risk of high-interest payments, which will cost you much more in the long run.

 Beware of banks bearing gifts and other offers 

	You could make good use of a new computer, and $50 cash back is a very tempting offer. Many financial institutions use tactics such as these to attract new customers, but are the gifts worth it? Before allowing temptation to sway you into switching financial institutions or applying for another credit card, think twice.

 of?fers some suggestions on how you can decide if the carrot dangled in front of you is indeed a golden opportunity.

	Let a gift become a tiebreaker. If you are torn between two identical programs from two or more competitors, it's okay for the gift to become a deciding factor. But it's never a good idea to allow the gift to become the sole reason for opening a new account.

	Don't forget the other gift tax. Did you know that the bank must report the fair market value for any gift worth more than $10 to the IRS as income? This rule also applies to multiple gifts totalling more than $10 as well.

	Read the fine print. That $1,000 computer may be a tempting reason to buy a $20,000 certificate of deposit (CD), but make sure you look at the terms of the deal. If you need to withdraw any money within 10 years (before the end of the CD's term), the bank can deduct the computer's cost from the balance in addition to adding a penalty for early withdrawal.

Special offers and plans for overseas calls 

	Finally, making an overseas phone call will cost less than your actual cell phone. Although international landline, domestic landline, and cell-phone rates have dropped over the years?the average international landline call cost $0.52 in 2000 compared with only $0.20 in 2003?international rates for cell phones remained high, despite the demand. According to the 

, Americans in 2003 chatted for over 2 billion minutes to international callers, up 36% from 2002. Now providers such as Verizon Wireless and T-Mobile offer discounted international calling packages in addition to their standard plans, which charge extra for expensive international calls without an international subscription. For only $3.99 per month, Verizon Wireless offers rates that start at $0.09 per minute to over 100 countries within their network through their In?ter?national Long Distance Value Plan, saving cost-conscious consumers over 55% per minute plus standard airtime. T-Mobile offers call plans north and south of the US border for an additional $4.99 per month plus $0.05 per minute to Mexico and $0.09 per minute to Canada. With such substantial discounts for international rates, you now have no excuse not to call your madre or mere.

Move over BizRate. Several up-and-coming Web sites are positioning themselves to outperform the average online deal purveyor, according to the 

. Combing the Internet for the cheapest prices around on anything from apparel to electronics and more, these newcomers offer new features that give veterans like Yahoo Shopping and Froogle, Google's answer to the bargain hunt, a run for their money. Become.com links consumers to the Web sites of more than 7 million products from 500 merchants. Although it only encompasses 2.5 million, Smarter.com provides pictures of the actual products and direct links to the Web page on the merchant's Web site, and you can narrow your search by price range and brand. In reaction to growing concerns over Internet fraud, BuySafeShopping.com provides up to $25,000 of insurance from an independent third party with its Bonded Shopping?.

	Deal?ers are carefully screened by a comprehensive BuySafe inspection to verify the seller's identity and capabilities. It's not just consumers who benefit from comparison-shopping Web sites, of course. Mer?chants gain greater visibility by listing their products, and bargain shoppers get the best prices around.

Surgeon's Bulletin Board

								Clinical Associate Professor of Urology Department of Urology University of Illinois College of Medicine Chicago, IL

Professor of Pathology Department of Pathology Northwestern University School of Medicine Chicago, IL

	Desmoid soft tissue tumors are rare. The authors reviewed the literature and found 648 reported cases. Of these cases, only two presented as an abscess and none as gram-negative sepsis. The au?thors report the first case of a desmoid tumor presenting as septicemia (

) and an abscess. This case highlights the challenges in diagnosing this rare pathological entity.

	Desmoid is derived from the Greek word desmos, meaning band or tendon.

 The medical meaning of desmoid is a rare type of soft tissue tumor. Soft tissue tumors are derived from mesoderm and originate in nonepithelial extraskeletal tissue. Soft tissue tumors are uncommon; only 9,420 cases were estimated to occur in 2005.

Ac?cording to Weiss and Goldblum, there are 14 major subdivisions of soft tissue tumors, one of which includes fibromatoses.

	Fibromatoses are benign fibrous tissue proliferations characterized by (1) proliferation of well-differentiated fibroblasts; (2) an infiltrative pattern of growth; (3) the presence of collagen; and (4) the lack of mitotic activity. Weiss and Gold?blum caution that "the term fibromatosis should not be applied to nonspecific fibrous proliferations that are part of an inflammatory process."

 Fibromatoses are di?vided into superficial and deep tumors (Table 1). The deep fibromatoses, also referred to as des?moid tumors,

 can be intra-ab?dominal or extra-abdominal. Intra-ab?do?minal desmoids can be pelvic or me?senteric. A review of the literature re???vealed most cases (80%) were extra-abdominal; the remaining 20% were intra-abdominal, whether pelvic or mesenteric. Fewer than 10% of all des?moids are of the mesenteric variety.

	Desmoid tumors have been described as being "quite rare"

 They usually present as painless, slowly enlarging masses. These tumors are usually benign but can infiltrate contiguous structures.

 Desmoids can also recur, particularly those associated with Gardner's syndrome; however, with wide excision, the recurrence rate is low.

	Because of their rarity and protean manifestations, desmoid tumors present a diagnostic challenge and are seldom diagnosed preoperatively. We present a case of presumed mesenteric fibromatosis that exemplifies this dilemma.

	A previously healthy 67-year-old white man awoke at 5: 00 am with violent shaking chills and fever. His only significant medical history was a bleeding duodenal ulcer 5 years earlier. He had experienced no recent fevers or abdominal pain. The patient was taken to the emergency de?partment where sepsis, initially attributed to pneumonia, was diagnosed. The gram-negative (Pseudomonas and Candida) sepsis was treated and controlled emergently with antibiotics and fluids.

	A contrast-enhanced computed tomo?g?raphy (CT) scan performed 3 days after admission to the hospital revealed a large, complex, heterogenous, left ab?dominal mass measuring 10 x 12 cm and extending into the pelvis (Figure 1). The mass was contiguous su?periorly with the transverse colon and ileum and was contiguous inferiorly with the urinary bladder and sigmoid colon, which contained multiple diverticula. The mass contained enhancing soft tissue components, gas, fluid, and contrast.

	The workup continued with colono?s?copy, which was negative for familial adenomatous polyposis (FAP), but re?vealed diverticular disease. Five days after ad?mission, while the workup continued, the mass spontaneously ruptured. This re?sulted in acute abdomen, which required an emergency exploratory la?parotomy.

	The laparotomy revealed a pus-filled left lower quadrant, which contained an opened 10- to 12-cm abscess cavity that adhered to the ileum and sigmoid colon. The abscess cavity contained a 5- to 7-cm solid mass (Figure 2). Because of the possibility of malignancy, the entire abscess cavity and mass were widely resected and a Hartmann's pouch and di?verting ileostomy were created. The patient's postoperative recovery was un?eventful except for an episode of me?t?abolic acidosis, which required rehospitalization. The Hartmann's diversion and ileostomy were closed 4 months later.

	The pathologist's report confirmed the gross surgical findings. The microscopic examination revealed sheets of spindle cells interspersed with inflammatory cells and few (if any) mitotic figures (Figure 3). Higher-power magnifications revealed an inflammatory infiltrate (Figure 4). Immunohistochemical stains were negative for c-Kit, ruling out a gastrointestinal stromal tumor. The final pathology diagnosed the lesion as mesenteric fibromatosis. A separately resected liver nodule was negative for malignancy.

	Desmoid tumors are deep fibroma?toses, and mesenteric desmoids are the least common variety (Table 1). A review of the literature revealed approximately 648 cases of desmoid tumors, of which 115 (23%) were mesenteric (Table 2).

	A unique feature of desmoid tumors is their association with Gardner's syndrome or FAP. There is an autosomal dominant genetic component to FAP. The incidence of desmoid tumors among patients with this condition has been reported to be 852 times higher14 than that of the general population. Desmoids associated with FAP are more common because there are often multiple tumors and the tumors tend to recur. Colonoscopy ruled out FAP in our patient.

	The differential diagnosis in this case, given the presentation and CT findings, included diverticulitis with abscess, adenocarcinoma of the colon, lymphoma, gastrointestinal stromal tumor, urachal remnant, and a small bowel lymphoma. Mesenteric fibromatosis would have been a statistically remote consideration.

	Our case was unusual for several reasons. First, desmoid tumors are rare. The annual incidence is estimated to be be?tween two and five cases per million.

 Second, our patient's presentation was unusual, because he had neither pain nor a mass but had an abscess and gram-negative sepsis. Abscesses are uncommon, and the literature contained no previously re?ported cases presenting as gram-negative sepsis. Unlike our case, most patients present with a gradually enlarging ab?dominal mass that becomes clinically relevant only when it encroaches on contiguous vital structures such as the bowel, nerves, or major vessels. There were four cases of desmoid tumors presenting as fever, but no further clinical details were given to explain the fevers.

 A preoperative diagnosis of desmoid tumor is unusual unless a diagnosis of FAP has been made previously. Third, desmoids are not usually associated with inflammatory processes or abscesses.

	The most interesting finding in this case of mesenteric fibromatosis was the presence of inflammatory cells and an abscess. Weiss and Goldblum specifically state that "the term fibromatosis should not be applied to nonspecific reactive fibrous proliferations that are part of an inflammatory process."3 Burke and colleagues also note that "lack of inflammation within the tumor is a key feature of fibromatosis."

 Our patient's 10 x 12-cm lesion was predominantly a fibroproliferative process with an associated abscess cavity. Although abscesses have been previously reported with desmoid tumors, these cases have not been described in detail. In this case, while the radiologist found numerous diverticula in the sigmoid colon, the pathologist noted no communication between the diverticula and the abscess. Based on these findings, a diagnosis of mesenteric fibromatosis was made, which was confirmed by a second opinion from the Armed Forces Institute of Pathology.

	Our case is unusual because the clinical presentation did not conform to that expected of a desmoid tumor. Our patient did not present with a slowly enlarging painless mass, but instead had sudden onset of fever. The gross surgical and pathology findings also showed a large inflammatory process and Pseudomonas gram-negative sepsis, which has not been reported before this case. According to Weiss and Goldblum, these findings are grounds for excluding the diagnosis of fibromatosis; however, our case indicates that an inflammatory process should not necessarily rule out the possibility of a desmoid tumor. The pathologists, including those at the Armed Forces Institute of Pathology who gave a second opinion, agreed that this was a case of mesenteric desmoid tumor. This report suggests that appearances can be deceiving when it comes to diagnosing desmoid tumors.

 1. Allen PW. The fibromatoses: a clinicopathologic classification based on 140 cases. Am J Surg Pathol. 1977;1(3):255-270.

	2. Jemal A, Murray T, Ward E, et al. Cancer statistics, 2005. CA Cancer J Clin. 2005;55(4):10-30.

	3. Weiss S, Goldblum J. Enzinger and Weiss's Soft Tissue Tumors. St. Louis, Mo: Mosby; 2001:309.

	4. Kumar V, Cotran R, Robbins S. Robbins Basic Pathology. Philadelphia, Pa: WB Saunders Company; 2003:784.

	5. Reitamo JJ, Hayry P, Nykyri E, et al. The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population. Am J Clin Pathol. 1982;77(6):665-673.

	6. Anthony T, Rodriguez-Bigas MA, Weber TK, et al. Desmoid tumors. J Am Coll Surg. 1996;182(4):369-377.

	7. Stout A, Lattes R. Tumors of the soft tissues. Washington, DC: Armed Forces Institute of Pathology; 1967:19.

	8. Dong-Heup K, Kim DH, Goldsmith HS, et al. Intra-abdominal desmoid tumor. Cancer. 1971;27(5):1041-1045.

	9. Yannopoulos K, Stout AP. Primary solid tumors of the mes?entery. Cancer. 1963;16:914-927.

	10. Easter DW, Halasz NA. Recent trends in the management of desmoid tumors. Summary of 19 cases and review of the literature. Ann Surg. 1989;210(6):765-769.

	11. Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor. Not a benign disease. Arch Surg. 1989;124(2):191-196.

	12. Burke AP, Sobin LH, Shekitka KM, et al. Intra-abdominal fibromatosis. A pathologic analysis of 130 tumors with comparison of clinical subgroups. Am J Surg Pathol. 1990;14(4):335-341.

	13. Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17(1):158-167.

	14. Gurbuz AK, Giardiello FM, Petersen GM, et al. Desmoid tumors in familial adenomatous polyposis. Gut. 1994;35(3):377-381.

Patrick Guinan, Clinical Associate Professor of Urology, Department of Urology, University of Illinois College of Medicine, Chicago, IL; Pauline Chou, Professor of Pathology, Department of Pathology, Northwestern University School of Medicine, Chicago, IL

Sepsis is an Unusual Presentation of Desmoid Tumor

Esophageal rupture is a potentially life-threatening condition that is difficult to diagnose and manage. The incidence continues to rise because of the in?creased use of esophageal instrumentation during invasive diagnostic procedures. Precise diagnosis is essential for effective treatment and depends on early recognition of clinical features and accurate interpretation of diagnostic imaging. The authors report the case of an elderly woman who had trans?esophageal echocardiography monitoring during coronary artery bypass graft surgery. The patient had a complicated postoperative recovery, and a swallow study 10 days later showed a large noncontained esophageal perforation. Because of her multiple comorbidities and the risks of surgery, she was successfully treated conservatively with a continuous antibiotic irrigation-drainage system through a nasogastric tube above the perforation and a chest tube for drainage. This case suggests that conservative management of esophageal perforation is a valid option for patients with multiple comorbidities who are diagnosed late, even after the onset of sepsis. 

Surgical Resident Department of Surgery Brookdale University Hospital and Medical Center

								Resident Division of Cardiothoracic Surgery SUNY Downstate Medical Center

Cardiothoracic Fellow Division of Cardiothoracic Surgery SUNY Downstate Medical enter

								Professor and Chief Division of Cardiothoracic Surgery SUNY Downstate Medical Center

Attending Cardiothoracic Surgeon Division of Cardiothoracic Surgery SUNY Downstate Medical Center

Chairman of Surgical Services Department of SurgeryBrookdale Univeristy Hospital and Medical Center Brooklyn, NY

Associate Professor Division of Cardiothoracic Surgery SUNY Downstate Medical Center Brooklyn, NY

	Since described by Barrett in 1946, esophageal perforation continues to present a diagnostic and therapeutic challenge to the surgeon.

 It still carries a high rate of morbidity and mortality because of delays in diagnosis, ex?tensive mediastinal contamination, and inadequate surgical repair. The incidence of esophageal perforation has increased with the expanding use of invasive diagnostic technology. The etiology has changed from mostly spontaneous or traumatic to mostly iatrogenic.

	We report the case of esophageal perforation in a woman who had transesophageal echocardiography (TEE) during coronary artery bypass graft (CABG) surgery. Despite sepsis, multiple comorbidities, and the character of the eso?ph?ageal extravagation, we successfully treat?ed the patient's iatrogenic esophageal perforation using a conservative approach.

	A 72-year-old woman came to the hospital reporting chest pain and shortness of breath. Her medical history was significant for end-stage renal disease, diabetes mellitus, hypercholesterolemia, hypertension, gastroesophageal reflux disease, sarcoidosis, and breast cancer treated with lumpectomy and radiation. An ab?normal electrocardiogram (EKG) and elevated cardiac enzymes were consistent with an inferior-wall myocardial infarction.

	Cardiac catheterization showed that the patient had four-vessel disease, re?quiring CABG surgery. Prior to the skin incision, TEE was performed to evaluate cardiac function. The patient underwent a one-vessel coronary bypass. A mediastinal and left and right pleural chest tubes were placed intraoperatively. The mediastinal and left pleural chest tubes were removed on postoperative day 1, and the right chest tube was removed on postoperative day 2. On postoperative day 4, the patient developed tachypnea, shortness of breath, and hypoxia. A chest radiograph showed a right hydropneumo-thorax and bilateral basal lung consolidation. A right chest tube was placed, which drained about 1 L of milky fluid per day. The fluid had high amylase levels. The patient's fever rose from 37?C to 39?C, and her white blood cell count increased to 21,000/?L. Ten days after surgery, a Gastrografin and barium swallow study showed disruption of the midesophagus with communication into the right pleural space, which was confirmed by a chest radiograph (Figure 1).

	On postoperative day 11, the patient underwent an upper endoscopy and placement of an esophageal irrigation catheter, left chest tube, and feeding jejunostomy. The patient had two nasogastric tubes placed intraoperatively: a drainage tube in the stomach and an irrigation tube proximal to the perforation. The patient received 10,000 units of bacitracin in 1 L of normal saline through the irrigation tube at 50 mL to 75 mL per hour for 14 days. Bacitracin irrigation was followed by 20 g of cefazolin in 1 L of normal saline administered at 50 mL per hour for 12 days. The patient also received gatifloxacin and clindamycin intravenously during this time.

	While undergoing this conservative treatment, the patient received enteral feeds via a jejunostomy tube. She re?quired a tracheostomy and continuous ventilation after prolonged intubation and failure to wean from the ventilator. She also developed several episodes of sepsis, which were treated effectively with antibiotics.

	During the course of treatment, barium studies were repeated and showed interval improvement of the esophageal perforation. The patient was able to tolerate tracheostomy collar trials after 24 days of treatment. The chest tube drainage cleared and irrigation was discontinued after 26 days of continuous esophageal irrigation. The chest tube output decreased to less than 10 mL per day, and 33 days after the irrigation was stopped, the chest tube was removed. After 56 days of conservative treatment, the patient was started on a liquid diet, which was slowly advanced to a regular diet. She was discharged to a nursing facility 3 months after surgery.

	A follow-up barium swallow study 5 months postoperatively showed no evidence of esophageal perforation (Figure 2). A chest radiograph and a computed tomography (CT) scan 7 months after sur?gery showed resolution of the right pleural effusion with no extravasations (Figure 3).

	The cause and location of an eso?phageal perforation are significant in predicting the success of treatment. Other key factors include whether the patient has any underlying esophageal disease and how much time elapses between the injury and commencement of medical treatment. Delaying medical attention 24 hours or more after injury can double the 20% mortality rate normally ascribed to esophageal perforation.

	Iatrogenic injury to the esophagus is the most common cause of esophageal perforation, with instrumentation during diagnostic procedures accounting for 59% of all cases and 15% occurring spon?taneously.

 Other causes include for??eign body ingestion (12%), trauma (9%), opera?tive injury (2%), and tumor (1%). Proce?dures that commonly cause esophageal perforation include TEE, eso?ph?agoscopy, sclerotherapy, variceal liga?tion, pneumatic dilation, bougienage, and laser the?r?apy. Esophageal manipulation should be minimized in patients with risk factors for esophageal perforation, such as exposure to radiation or those with scleroderma.

	To diagnose and treat an esophageal perforation effectively, it is important to recognize the clinical signs and symptoms of the injury and accurately analyze diagnostic images. Symptoms include pain, fever, chills, dysphagia, and he?ma?te?mesis. Signs include chest or cervical ten?der?ness on palpation, mediastinal crunch heard on cardiac auscultation (Hamman's sign), and crepitation. The presence of undigested food, a pH of less than 6.0,8 or an elevated salivary amylase level in pleural fluid collected by thoracentesis all indicate esophageal perforation. Plain chest radiographs for 90% of patients show pleural effusion, pneumomediastinum, subcutaneous emphysema, hydrothorax, hydropneumothorax, or subdiaphragmatic air, but can appear normal if taken early.

Any left-sided pleural effusion strengthens suspicion of perforation.

	Contrast esophagography remains the standard for diagnosing esophageal perforation. Water-soluble contrast agents, such as Gastrografin, are advocated for first-line screening of suspected perforation. If perforation is not detected with a water-soluble agent, serial barium contrast esophagography should follow.

 Because a negative result cannot rule out the possibility of perforation, an accurate diagnosis may require a CT scan or flexible esophagoscopy, or both.

	l Conservative management?Conser?vative management has been a controversial treatment method since the first successful series was reported in 1965 by Mengold and Klassen.

 Over the years, thoracic surgeons have postulated the best scenario to use this treatment, taking into consideration timing of diagnosis, location of the perforation, extent of in?jury, and the overall clinical picture. Several studies show high survival rates when instrumental perforations are treated within 24 hours of diagnosis.

 In contrast, a study by Junginger and colleagues showed that conservative treatment of early diagnosed esophageal rupture was associated with a high mortality rate.

 Michel and colleagues found that pa?tients with cervical lesions had a higher risk of mortality with conservative management than with surgical repair.

 In a study by Altorjay and colleagues, two of 20 patients died, revealing a mortality rate of 10% using a conservative ap?proach.

 The rate of comp?lications in the conservatively treated group was 20% (4 of 20 patients) compared with 50% in the operative group (33 of 66 patients). Other studies advocate use of conservative man?agement in patients with cervical perforations.

 To date, there is no strict criterion regarding the use of conservative management for esophageal perforations.

	Traditionally, patients with esophageal perforations are treated surgically; however, treatment of these patients with esophagectomy or primary repair is associated with a high mortality rate.

 Conservative management is made possible due to improved antibiotics and advances in nutritional support.

 It is thought to be appropriate for patients who fulfill Cameron's criteria, which include minimal signs of clinical sepsis, disruption contained in the mediastinum, drainage of the cavity back into the esophagus, minimal symptoms, delayed diagnosis, and clinically stable pa?tients.

 Conservative management is considered to be the first therapeutic choice in children.

 One retrospective study on 57 patients concluded that esophageal perforation requires aggressive treatment.

 We find that treatment depends on whether there is perforation of a healthy esophagus or perforation with a preexisting underlying intrinsic esophageal disease causing distal ob?struction. Esophageal perforation associated with stenotic lesions (benign or malignant) requires esophageal extirpation. Perforation in a healthy esophagus should be treated by primary closure if encountered early. Conservative management is appropriate when there is delay in diagnosing esophageal perforation.

	Conservative management includes taking nothing by mouth for at least 48 to 72 hours, at which time clear liquids can be initiated if the patient shows improvement. Because mediastinal sepsis is primarily responsible for deaths associated with delayed diagnosis of esophageal perforation, proper drainage and antibiotics are the keystones in management. Conservative management should in?clude administering broad-spectrum antibiotics for at least 7 to 14 days and total parenteral nutrition. Irrigating with an antibiotic solution and providing intravenous antibiotics are beneficial and may help control sepsis and local inflammatory reaction, allowing subsequent healing and closure of the esophageal opening.

 This should be carried out through esophagogastroduodenoscopy (EGD)-directed placement of two nasogastric tubes: one above and one below the esophageal perforation. The proximal tube is used for irrigation, and the distal tube should be used for gastric decompression and as an underwater seal chest tube for drainage. Because EGD allows direct visualization, it is a precise way to insert the irrigation-drainage system.

	Several factors are responsible for causing esophageal perforation, thus treatment for this life-threatening event has become more individualized. En?suring adequate pleuromediastinal drain?age with sufficient irrigation is imperative for all patients. Spontaneous eso?phageal perforations may require early surgical exploration with drainage and irrigation of the mediastinum and pleural cavity, whereas most iatrogenic lesions can be managed conservatively.

	We used a conservative approach for managing esophageal perforation in a septic patient with a noncontained per-foration because surgery had an especially high mortality risk. The irrigation-drainage system we used resulted in a satisfactory outcome. Enteral nutrition maintains gut functioning while giving superior alimentation to parenteral nutrition. Close monitoring in the intensive care unit was necessary for quickly diagnosing and treating the patient's septic episodes.

	How best to treat esophageal perforation depends on the extent and location of the injury, the etiology, whether there is underlying esophageal disease, the pa?tient's general condition, and, most im?portantly, the time interval between perforation and diagnosis. A high index of suspicion and diligent follow-up are essential in diagnosing and treating esophageal perforation. Whether or not to use a conservative approach must be determined on a case-by-case basis. Our experience indicates that conservative man?agement might be safely extended to high-risk patients even in the setting of non?contained ruptures and sepsis.

	1. Jones WG 2nd, Ginsberg RJ. Esophageal perforation: a continuing challenge. Ann Thorac Surg. 1992;53(3):534-543.

	2. Bufkin BL, Miller JI Jr, Mansour KA. Esophageal perforation: emphasis on management. Ann Thorac Surg. 1996;61(5): 1447-1452.

	3. Brinster CJ, Singhal S, Lee L, et al. Evolving options in the management of esophageal perforation. Ann Thorac Surg. 2004;77(4):1475-1483.

	4. Iannettoni MD, Vlessis AA, Whyte RI, et al. Functional outcome after surgical treatment of esophageal perforation. Ann Thorac Surg. 1997;64(6):1606-1610.

	5. Whyte RI, Iannettoni MD, Orringer MB. Intrathoracic esophageal perforation. The merit of primary repair. J Thorac Cardiovasc Surg. 1995;109(1):140-146.

	6. Wang N, Razzouk AJ, Safavi A, et al. Delayed primary repair of intrathoracic esophageal perforation: is it safe? 

	7. Shaffer HA Jr, Valenzuela G, Mittal RK. Esophageal perforation. A reassessment of the criteria for choosing medical or surgical therapy. Arch Intern Med. 1992;152(4):757-761.

	8. Backer CL, LoCicero J 3rd, Hartz RS, et al. Computed tomo?g?raphy in patients with esophageal perforation. 

	9. Han SY, McElvein RB, Aldrete JS, et al. Perforation of the esophagus: correlation of site and cause with plain film findings. AJR Am J Roentgenol. 1985;145(3):537-540.

	10. Gollub MJ, Bains MS. Barium sulfate: a new (old) contrast agent for diagnosis of postoperative esophageal leaks. 

	11. Mengold L, Klassen KP. Conservative management of esophageal perforation. Arch Surg. 1965;91:232-240.

	12. Larrieu AJ, Kieffer R. Boerhaave syndrome: report of a case treated non-operatively. Ann Surg. 1975;181(4):452-454.

	13. Cameron JL, Kieffer RF, Hendrix TR, et al. Selective nonoperative management of contained intrathoracic esophageal disruptions. Ann Thorac Surg. 1979;27(5):404-408.

	14. Junginger T, Schafer W, Bottger T. Esophageal perforation?indications for surgical therapy [in German]. 

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	17. Gupta NM, Kaman L. Personal management of 57 consecutive patients with esophageal perforation. Am J Surg. 2004;187(1): 58-63.

	18. Platel JP, Thomas P, Giudicelli R, et al. Esophageal per-forations and ruptures: a plea for conservative treatment [in French]. Ann Chir. 1997;51(6):611-616.

	19. Salo JA, Isolauri JO, Heikkila LJ, et al. Management of de?layed esophageal perforation with mediastinal sepsis. Esoph?agectomy or primary repair? 

	20. Bladergroen MR, Lowe JE, Postlethwait RW. Diagnosis and recommended management of esophageal perforation and rupture. Ann Thorac Surg. 1986;42(3):235-239.

	21. Martinez L, Rivas S, Hernandez F, et al. Aggressive conservative treatment of esophageal perforations in children. J Pediatr Surg. 2003;38(5):685-689.

	22. Gupta NM, Kaman L. Personal management of 57 consecutive patients with esophageal perforation. Am J Surg. 2004;187(1): 58-63.

	23. Port JL, Kent MS, Korst RJ, et al. Thoracic esophageal perforations: a decade of experience. Ann Thorac Surg. 2003;75(4): 1071-1074.

	24. Santos GH. Difficulties encountered in the treatment of patients who develop sepsis after esophageal perforation. Ann Surg. 1990;212(5): 652.

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Albeir Mousa, Surgical Resident, Department of Surgery, 

Conservative Management of an Esophageal Disruption in a Septic Patient

					Gastrointestinal stromal tumors (GISTs) are uncommon and are preferentially treated by excision with good results. Morse and Evans report two cases of gastric GISTs that were successfully excised laparoscopically. Wide resections are not necessary because many GISTs are localized to areas of the stomach where a wedge resection can be performed. Suture closure of the area where the two staple lines meet is critical. Methylene blue is instilled into the distended stomach to check for leaks. If these tumors are in close proximity to the pyloric area, a distal resection may be required. The authors provide a good review of GISTs and thoroughly describe the laparoscopic technique.

Series Guest Editor, Professor and Chairman, Department of Surgery, Medical College of Georgia, Augusta, GA

Associate Professor of Surgery Department of Surgery Memorial Hospital York, PA

	Gastrointestinal stromal tumors (GISTs) are relatively rare tumors of the gastrointestinal (GI) tract, usually occurring in the stomach (60% to 70%).

 They arise from mesenchymal cells of the GI tract. Although surgical resection offers the best chance for a cure, there is local and distant recurrence in 40% to 90% of cases.

Andrew J. Morse, Chief Surgery Resident; Christopher Evans, Associate Professor of Surgery, Department of Surgery, Memorial Hospital, York, PA

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