Hemangiomas and vascular malformations of the GI tract

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Hemangiomas and vascular Malformations of the GI tract Stephen Yoo M.D.

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Seminars in Colon and Rectal Surgery

Cite this article as: Stephen Yoo M.D., Hemangiomas and vascular Malformations of the GI tract, Seminars in Colon and Rectal Surgery, http://dx.doi.org/10.1053/ j.scrs.2015.01.010 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

Hemangiomas and Vascular Malformations of the GI Tract Stephen Yoo M.D. Division of Colon and Rectal Surgery, Department of Surgery Cedars-Sinai Medical Center, Los Angeles CA

Correspondence: Stephen Yoo M.D. 9400 Brighton Way, Suite 307 Beverly Hills, CA 90210 Phone: (310) 273-0511 Fax: (310) 273-0314 [email protected] Submitted for publication to Seminars in Colon and Rectal Surgery, October 2014. Abstract: Hemangiomas and vascular malformations of the GI tract are a rare entity and present as overt or occult bleeding. Presenting as a singular cavernous hemangioma or malformation, which is often located in the recto-sigmoid region, they can be distributed throughout the intestinal digestive system. Despite characteristic radiographic features such as radiolucent phleboliths on plain film imaging and a purplish nodule on endoscopy, misdiagnosis is common. There is potential for local invasion, therefore adjunctive imaging such as CT and MRI are part of the suggested workup. Endorectal ultrasound with Doppler has also been found to be useful in some instances. With an emphasis on sphincter preservation, surgical resection is the mainstay of treatment,

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though in instances where an extensive resection is not feasible, non-surgical endoscopic treatment with banding and sclerotherapy has been reported with success.

Key Words: Hemangioma, Cavernous hemangioma, Vascular Malformation, Angiodysplasia, Blue rubber bleb

Epidemiology Hemangiomas and vascular malformations of the GI tract, first documented in 1839, are an infrequently encountered entity1,2. Potentially occuring anywhere in the gastrointestinal tract, the small bowel is the most frequent site of hemangiomas and malformations, though they account for 10% of all small bowel tumors3. With only 200 cases documented from 1931 to 1974, colonic an anorectal hemangiomas and malformations are an even more rare entity4,5. A historical review in 1949 of GI hemangiomas and malformations identifed 38% of hemangiomas as colorectal in origin, whereas another review classified 50% of colorectal lesions located in the rectum2,6. To date, there have been over 130 reports of rectal hemangiomas/malformations. The age of a definitive diagnosis of GI hemangiomas and malformations range from 2 months to 79 years old, due to the fact that many are misdiagnosed2,7,8. There is a slight female preponderance with a male:female ratio of 1:2.5, though colonic hemangiomas and malformations have an equal male:female ratio2. Looking at the sub classification of vascular malformations known as blue rubber bleb nevus syndrome, the largest operative single institution series (n=10), identified a mean patient age of 16, with a range of 2-36 years old9. The intial bleeding symptoms were present early (mean 5 years).

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Histology, pathology and classification Complicated by the confusing nomenclature, the term “hemangioma” is often misused. Modifiers and adjectives such as “strawberry-“, “cavernous-“, and “capillary-” are added to the term “hemangioma”, but are not hemangiomas in the strictest definition11. In 1982, Mulliken and Glowacki classified vascular lesions in a consistent and meaningful scheme based on the histology and endothelial cell turnover12,13. Normal endothelial conditions are correctly termed “vascular malformations”, calssified by their dominant abnormality (venous malformation, arteriovenous malformation, lymphatic malformation, lymphatic-venous malformation, and capillary malformation)11,14. High endothelial cell turnover entities are accurately termed hemangioms (rapidly involuting congenital hemangioma, infantile hemangioma, non-involuting congenital hemangioma, Kaposiform hemangioendothelioma, and tufted angioma). Present at birth, the majority of these lesions undergo a spontaneous involution. Steroid administration can accelerate the involution process. The International Society for the Study of Vascular Anomalies approved this classification system in 1996 (Table 1). Until this point, this article used the term “hemangioma” due to the audience’s familiarity with the term. Though hemangiomas do occur in the GI tract, the predominant entities are vascular malformations, including those termed as “cavernous hemangiomas”. In precise terms, this is a misnomer and the term “cavernous vascular malformation” or “cavernous malformation” is more accurate and will be used. An embryologic error in morphogenesis is how vascular malformations result14. Lacking smooth muscle, mature endothelial channels allow expansion over time from hydrostatic means, and not

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proliferative expansion as hemangiomas do. A classificaton of intestinal malformations was devised based on the histological abnormality (Table 2). The capillary subtype of malformations are located in the small bowel, appendix, and perianal skin20. They lack a true capsule, ad usually singular, and are well circumscribed. With accompanied edema and inflammation, ½ can have associated mucosal ulceration. The proliferation of capillaries with thin-walled spaces lined by endothelial cells is the histological hallmark. The cavernous subtype composes 80% of rectosigmoid malformations16-18,21,22. Cavernous malformations are large spaces lined by single or multiple layers of endothelial cells, as opposed to capillary malformations (Figure 1). Often polypoid, the localized variety of cavernous malformations can be symptomatic. Reported up to 30cm in length, the diffuse variety of cavernous malformations can be multiple. With the rectum involved in 70% of instances, these entities have the potential for local invasion to adjacent structures and can be corcumferential2,23. Ranging form solitary lesions to clusters, the gross appearance of GI vascular malformations overwhelmingy present as intraluminal lesions, though diffuse cavernous malformations can infiltrate the submucosa and beyond and can extend to adjacent structures. GI vascular malformations can be an feature of associated syndromes with characteristc organ involvement. Simultaneous vascular malformations in the spinal cord, brain, and skin as well as other clincial traits such as bone and limb hypertrophy, arteriovenous fistulas and varicose veins have been documented (Table 3)8,16,17,20,24. Only 1.8% of cutaneous hemangiomas and vascular malformations have GI vascular malformations present, despite cutaneous involvement being a frequent association25. No evidence exists of predisposed inherited patterns when analyzing

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mono- and dyzygotic twins26. However, this counters a case report describing 6 families that demonstrated an autosomal morphology with incomplete penetrance27. Pathophysiology The key differntiation between hemangiomas and vasular malformations in the original classification by Mulliken and Glowacki are due to the smooth muscle proliferation12,13. Related to the defect intrinsic to the endothelial cells and secretion of growth factors, the abnormality arises in the embryological mesoderm. The 3:1 female to male ratio is thought due to hormonal influence2. The different histological subtypes are thought due to the the developmental stage of when the angiogenic abnormality occurred in the stem cell cycle, with capillary malformations developing from an earlier defect than cavernous malformations2,8,16. Alteration of blood flow from the abnormal endothelium can sequester platelet and clotting factors and initiate coagulation29. Kasabach-Merrit syndrome can develop in rapidly expanding malformations, where a DIC like picture can develop with consumption of fibrinogen and factors V and VIII, uncontrolled bleeding and a 35% mortality rate30. Calcification and phelbolith development from the constant sequestration of flow can be seen in 50% of cases31. Erosion of the malformation into the bowel lumen and subsequent bleeding and also fragmentaiton of reticulocytes by the thrombus can lead to anemia. Local or segmental bowel ischemia can result from the sequestration and coagulation16. Case reports describe invasion the sacrum, bladder and uterus of the hemangioma/malformation into surrounding structures, especially when the abnormality originates in the rectosigmoid region. Malignancy is rarely encountered, despite the ability for local invasion8,32,33. Clinical Presentation History

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Misdiagnosis of the hemangioma and malformation is a common theme. 80% of patients underwent one prior inappropriate surgical procedure34-36. A cases series identified that 4 out of 5 patients with an eventual diagnosis of a vascular malformations first underwent a hemorrhoidectomy37. GI bleeding was misdiagnosed as hemorrhoids and ulcerative colitis in another series evaluating rectosigmoid cavernous vascular malformations34,35. Evaluating 47 patients, another series estimated a delay in diagnosis of 16 years38. With similar conclusions, a separate review estiamted that the correct age of diagnosis was at 19 years with 51% of the patients undergoing either an inappropriate or ineffective operation36. Intraluminal bleeding is present in a majority of cases, and an estimated 80% of patients exhibit symptoms39-41. Recurrent painless bleeding is seen in up to 90% of cases, with half of pateints showing chronic iron deficient anemia. With episodes worsening over time, the first presentation is often in childhood6. There is potential for intraperitoneal or retroperitoneal bleeding when transmural malformations exist. Hemothorax or hemopericardium can present when there are non-GI malformations. Mucosal trauma causes erosion and eventual bleeding with the increased bleeding occuring in larger and more distal subtypes. Though infrequent, obstruction is possible42. Circumferential masses can cause luminal obstruction, and polypoid lesions can act as a lead point for intussception to result in obstruction43. Larger malformations can present with constipation secondary to tenesmus. Abdominal or pelvic pain can also be another presenting symptom. Physical Exam Hemangiomas and malformations usually present with few findings. Though not overt masses, distal lesions can be detected on digital examination. The tumors have a nodular sensation and

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are soft and compressible. When large, a mass on abdominal examiation can sometimes be palpated. Associated cutaneous manifestations syndromes should be remembered20. Workup Laboratory Evidence of chronic or acute blood loss will be present. In large consumptive masses, a decrease in clotting factors such as fibrinogen, platelets, and factors V and VIII is seen. Imaging Plain film 50% of cases can demonstrate calcified phleboliths due to the sequestration of blood flow31. The calcifications occur in the colonic wall and does not extend to the soft issue, and this therefore underestimates the presence of the hemangiomas and malformations. When lateral and outside the pelvic venous plexus, the presence of these phleboliths are specific signs (Figure 2)33. These findings are rare in the normal population, occuring less than 5% of the time in individuals under 30. The appearance of phleboliths in younger patients should raise the suspicion of malformations6,33,44. Contrast studies Anterior displacement of the rectum and widening of the presacral space can infer the mass effect and soft-tissue component of large cavernous rectal malformations. These masses may collapse with air insufflation. The primary manifestation of hemangiomas and malformations that are identified with contrast studies occur in obstructing or polypoid lesions (Figure 3)33. CT Transmural enhancing bowel-wall theckening with or without phleboliths is pathognomonic (Figure 4)33. The extent of extramural extension and surrounding invasion can be accurately

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evaluated. CT colonography has been advocated in that it aids in the diagnostic yield by helping identify mucosal lesions and intraluminal characteristics, as well as distribution33. MRI Especially in cases of rectal malformations, MR can enhance the diagnosis. In addition to wall thickening, the slow flow can produce high T2 signal intensity. The perirectal fat also demonstrates increased signal intensity with serpiginous structures correlating to the small vessels supplying the hemangioma (Figure 5). These MR features are highly specific and are not found in other clinical entities. Though hemorrhoids can present with similar T2 findings, the difference in the two entities can be seen by location and lack of perirectal fat extension or abnormalities in hemorrhoids. Phleboliths and calcifications are less easily detected on MR vs. CT or plain film. Ultrasound One instance of massive hemorrhage in pregnancy was diagnosed with the use of endorectal ultrasound (Figure 6). The diagnosis was aided by the utilization of Doppler studies demonstrating pulsitile flow45. Angiography A delayed venous phase is a commonly seen pattern in mesenteric angiography. It also demonstrates a characteristic pooling, most often seen in the rectosigmoid. This can be identified in the absence of active bleeding, and it is helpful for identifying synchronous lesions6,7. However, the presence of thrombosis can lower the sensitivity of angiography as a diagnostic modality, as lesions can have a hypovascular or avascular appearance.

Endoscopy

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Essential to the workup and evaluation of hemangiomas and malformations, colonoscpy is crucial40,46. The polypoid lesions can collapse with insufflation, as noted with air-contrast barium enemas. The intraluminal characteristics have submucosal projections that range from blue to red (Figure 7). Overt ulceration is rarely seen and pinpoint areas of bleeding are possible. Inflammatory bowel disease can be mistakingly diagnosed due to mucosal edema, nodularity, and vascular congestion2,17,31. Hemorrhoids are also a frequent misdiagnosis. Evaluation of the upper GI to aid in the identification of synchronous lesions is suggested, as well as a complete colonoscopy to assess the proximal extension. Biposy of the lesion is not recommended6,17,18,31,44,47, due to the obvious potential for bleeding, though some have suggested its use when obtained with caution to enhance the correct diagnosis48. Management Medical Critical care and resuscitative principles take first priorty, as with all GI bleeding, insuring that hemodynamic stability is achieved. In true hemangiomas by histological classification, corticosteroids have achieved success in the plastics/reconstructive literature49-51. However, being that most of the GI associated hemangiomas are actually vascular malformations, pharmacological options are rarely effective. Endoscopic Snare polypectomy and cauterization have successfully treated ideal lesions of polypoid tumors with a narrow base52-54. Argon beam has also been reported to have success, even in instances of severe hematochezia55. A moderate rectosigmoid malformation was successfully treated over a course of 13 sessions endoscopically with injections of n-butyl-2-cyanoacrylate56. The same authors report a case of similar lesion on a different patient whom required 15 sessions, though

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this patient died 4 months for recurrent bleeding after treatment was completed. This technique should only be used if surgery is not a feasible option. Operative The treatment of choice is surgical resection, despite more conservative options. Prior to 1971, the recommended treatment of rectosigmoid malformations was an abdominal perineal resection6,16,17,57-59. The current standard is a low anterior resection and a mucosal resection with sphincter preservation as the goal60. Other surgical options include segmental resection, a low anterior resection without a mucosectomy, or the modified Parks coloanal pull-through61,62. The proximal margin is well delineated by the presence of subserosal serpentine vessels in the colon with the malformation, as well as a more rigid bowel and thickened mesentery63. A doublestapling approach is feasible if normal distal bowel exists. Regarding mucosectomy, a plane exists between the muscularis and the mucosa, allowing a sleeve to be resected64. A removal of the mucosa 0.5cm proximal to the dentate line is made, with the aid of a submucosal epinephrine infiltration. Some approaches advocate a dissection down to the levators, and the preservation of a 3-4cm anal/distal cuff, and a hand-sewn anastomosis47. As with most anal anastomosis a proximal diverting ileostomy is created. In more proximal lesions, segmental resection, as well as full-thickness wedge resection, suture ligation and operative polypectomy is advocated9. The largest single institution analyzing bluerubber bleb malformations had advocated an aggressive surgical approach, and eradication of all identified lesions. This is combined with interoperative push-enteroscopy to aid in a thorough evaluation of all malformations. This series had analyzed 10 patients, and only had rebleeding in one instance, however that patient had over 557 lesions. Their experience advocates for complete

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resection, as lesions that were incomplete by banding or suture ligation techniques have expansion of residual malformations, rather than recurrence. If fortunate to have an isolated proximal malformation, laparoscopic approaches have been successful65. References: 1.

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Figures: Figure 1: Cavernous vascular malformation. Composed of sinus-like blood-filled spaces with prominent submucosal vascular channels. (Left: H&E 4x; right: H&E 10x). Reprinted with permission3.

Figure 2: CT reconstruction simulating plain film imaging. Characteristic phleboliths and their typical distribution is depicted. Reprinted with permission33.

Figure 3: Barium enema. Multiple polypoid submucosal masses throughout, especially in the rectosigmoid colon. Reprinted with permission33.

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Figure 4: CT. Rectosigmoid wall thickening with phleboliths. Reprinted with permission33.

Figure 5: MR. T2 weighted imaging demonstrating rectal wall thickening (arrow), and perirectal serpiginous vascularity (star). Reprinted with permission8.

Figure 6: ERUS. Demonstrates sponge-like nature of malformation. Not well represented is the duplex flow that is specific for identifying the vascular component. Reprinted with permission45.

Figure 7 : Endoscopic view. Reprinted with permission3.

Tables: Table 1 : Vascular Malformations versus Hemangiomas11 Vascular Malformation Hemangiomas Histology Normal endothelial cell High endothelial cell turnover turnover Presence at Birth Present (not always apparent) Usually absent Clincal Grows in proportion with Apparent 6-8 weeks after birth. person. Proliferative phase for 1-2 years then spontaneous involution. Diagnosis Imaging (MRI, CT, US, Clinical history, appearance Angiography) Treatement Depending on site, size Observation symptoms, etc. From If not fully involuted, minor conservative only to laser for surgical correction. If large or in capillary malformations, anatomically sensitive area, sclerotherapy with or without steroids or interferon gamma or excision, or surgery alone. surgical correction. Table 2 : Intestinal Hemangioma Classification15-19 Capillary Cavernous Localized (polypoid or non-polypoid) Diffuse infiltrating (expansive) Mixed Hemangiomatosis

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Table 3 : Associated syndromes20 Syndrome Inheritance Blue rubber bleb nevus Most sporadic syndrome Klippel-Trenaunay-Weber syndrome

Sporadic

Osler-Rendu-Weber syndrome

Autosomal dominant

Fig 1

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Characteristics Cavernous hemangiomas of the skin, GI tract and other viscera. Lesions are blue, tender, and blanche. Triad of cutaneous hemangiomas, bone and soft-tissue, hypertrophy of lower extremities, and congenital varicosities. Mucocutaneous telangectasias, especially oral and nasal. Hemangiomatous lesions in stomach, small intestine and rectum.

Fig 2

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Fig 3

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Fig 4

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Fig 5

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Fig 6

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Fig 7

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