Preservation of small bowel with the selective use of heparin and second look laparotomy in acute...

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CASE REPORT OPEN ACCESS International Journal of Surgery Case Reports 3 (2012) 260–262 Contents lists available at SciVerse ScienceDirect International Journal of Surgery Case Reports j ourna l ho me pa ge: www.elsevier.com/locate/ijscr Preservation of small bowel with the selective use of heparin and second look laparotomy in acute mesenteric ischaemia: A case report Dina Fouad a,, Shayanthan Nanthakumaran a , Henry G. Watson b , Colin G. Millar c , Peter M. King a a Department of General Surgery, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZD, UK b Department of Haematology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZD, UK c Department of Nephrology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZD, UK a r t i c l e i n f o Article history: Received 1 March 2012 Accepted 13 March 2012 Available online 20 March 2012 Keywords: Janus Kinase 2 Essential thrombocythaemia Acute mesenteric ischaemia Heparin Second look surgery a b s t r a c t INTRODUCTION: Acute mesenteric ischaemia may occur due to mesenteric arterial embolus, thrombosis, non-occlusive mesenteric ischaemia or venous thrombosis resulting in ischaemia of the bowel wall. PRESENTATION OF CASE: A 41 year old woman presented with worsening abdominal pain, decreased appetite, nausea and vomiting. Examination revealed right lower quadrant tenderness. Investigations revealed elevation of her inflammatory markers. At laparotomy two separate segments of ischaemic but potentially viable small bowel were identified secondary to mesenteric venous thrombosis. Bowel salvage was attempted with the use of intravenous unfractionated heparin and this was confirmed following a second look laparotomy. DISCUSSION: Despite a normal platelet count at presentation a diagnosis of JAK-2 positive essential thrombocythaemia was made thus explaining the acquired prothrombotic state underlying the venous thrombosis. The selective use of intravenous unfractionated heparin and second look laparotomy may provide a means for bowel preservation in these cases. CONCLUSION: This case highlights the potential of bowel salvage can be achieved following an episode of acute mesenteric ischaemia with the use of intravenous unfractionated heparin and selective second look laparotomy and the importance of considering underlying myeloproliferative disease in such cases even in the absence of a thrombocytosis at presentation. © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. 1. Introduction Acute mesenteric ischaemia occurs due to inadequate blood flow through the mesenteric circulation causing ischaemia of the bowel wall. It occurs as a result of acute mesenteric arterial embolus (50%), acute mesenteric arterial thrombosis (25%), non-occlusive mesenteric ischaemia (15–20%) or mesenteric venous thrombosis (5–10%). 1 The damage to the bowel wall can vary from reversible ischaemia to full thickness infarction resulting in necrosis and perforation. Patients usually present with intense visceral pain dis- proportionate to physical signs. Acute mesenteric arterial emboli usually originate from a car- diac source. Patients experience severe pain due to the sudden nature of the occlusion. Mesenteric arterial thrombosis occurs at the site of pre-existing atherosclerosis involving the superior mesenteric artery. Patients usually have a preceding history of chronic mesenteric ischaemia before the acute thrombotic event, precipitated by hypotension following a myocardial infarction or arterial complications such as a dissection or trauma. Non- occlusive mesenteric ischaemia occurs due to arterial spasm due to Corresponding author at: Department of Urology, Western General Hospital, Crewe Road South, Edinburgh EH4 2XU, UK. Tel.: +44 7793954384. E-mail address: [email protected] (D. Fouad). cardiogenic shock, septic shock, or hypovolaemia. Mesenteric venous thrombosis often presents with acute abdominal pain in a younger cohort. Symptoms may be less dramatic, and the diagnosis difficult to establish. Most patients will have one or more risk fac- tors for a generalised pro-thrombotic state. These risk factors may be congenital or acquired and include hereditable thrombophilic abnormalities such as deficiency of the natural anticoagulants antithrombin and protein C and S or inheritance of the common gain of function mutations factor V leiden and the prothrombin gene G20210A mutation. Acquired prothrombotic states may arise locally from the abdominal cavity such as acute pancreatitis, active inflammatory bowel disease, malignancy or sepsis or may result from a systemic procoagulant state such as that resulting from the use of the combined oral contraceptive pill or the development of antiphospholipid syndrome or myeloproliferative disease. 2 We describe a case of acute ischaemia of the small bowel due to mesenteric venous thrombosis secondary to essential throm- bocythaemia, which was managed with intravenous heparin and a second look laparotomy resulting in preservation of the small bowel. 2. Presentation of case A 41 year old Caucasian woman presented to the General Surgi- cal Unit with a 5 day history of continuing central abdominal pain 2210-2612/$ see front matter © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.ijscr.2012.03.007

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CASE REPORT – OPEN ACCESSInternational Journal of Surgery Case Reports 3 (2012) 260– 262

Contents lists available at SciVerse ScienceDirect

International Journal of Surgery Case Reports

j ourna l ho me pa ge: www.elsev ier .com/ locate / i j scr

reservation of small bowel with the selective use of heparin and second lookaparotomy in acute mesenteric ischaemia: A case report

ina Fouada,∗, Shayanthan Nanthakumarana, Henry G. Watsonb, Colin G. Millarc, Peter M. Kinga

Department of General Surgery, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZD, UKDepartment of Haematology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZD, UKDepartment of Nephrology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZD, UK

r t i c l e i n f o

rticle history:eceived 1 March 2012ccepted 13 March 2012vailable online 20 March 2012

eywords:anus Kinase 2ssential thrombocythaemiacute mesenteric ischaemiaeparinecond look surgery

a b s t r a c t

INTRODUCTION: Acute mesenteric ischaemia may occur due to mesenteric arterial embolus, thrombosis,non-occlusive mesenteric ischaemia or venous thrombosis resulting in ischaemia of the bowel wall.PRESENTATION OF CASE: A 41 year old woman presented with worsening abdominal pain, decreasedappetite, nausea and vomiting. Examination revealed right lower quadrant tenderness. Investigationsrevealed elevation of her inflammatory markers. At laparotomy two separate segments of ischaemic butpotentially viable small bowel were identified secondary to mesenteric venous thrombosis. Bowel salvagewas attempted with the use of intravenous unfractionated heparin and this was confirmed following asecond look laparotomy.DISCUSSION: Despite a normal platelet count at presentation a diagnosis of JAK-2 positive essentialthrombocythaemia was made thus explaining the acquired prothrombotic state underlying the venous

thrombosis. The selective use of intravenous unfractionated heparin and second look laparotomy mayprovide a means for bowel preservation in these cases.CONCLUSION: This case highlights the potential of bowel salvage can be achieved following an episodeof acute mesenteric ischaemia with the use of intravenous unfractionated heparin and selective secondlook laparotomy and the importance of considering underlying myeloproliferative disease in such cases

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. Introduction

Acute mesenteric ischaemia occurs due to inadequate bloodow through the mesenteric circulation causing ischaemia of theowel wall. It occurs as a result of acute mesenteric arterial embolus50%), acute mesenteric arterial thrombosis (25%), non-occlusive

esenteric ischaemia (15–20%) or mesenteric venous thrombosis5–10%).1 The damage to the bowel wall can vary from reversibleschaemia to full thickness infarction resulting in necrosis anderforation. Patients usually present with intense visceral pain dis-roportionate to physical signs.

Acute mesenteric arterial emboli usually originate from a car-iac source. Patients experience severe pain due to the suddenature of the occlusion. Mesenteric arterial thrombosis occurst the site of pre-existing atherosclerosis involving the superioresenteric artery. Patients usually have a preceding history of

hronic mesenteric ischaemia before the acute thrombotic event,

recipitated by hypotension following a myocardial infarctionr arterial complications such as a dissection or trauma. Non-cclusive mesenteric ischaemia occurs due to arterial spasm due to

∗ Corresponding author at: Department of Urology, Western General Hospital,rewe Road South, Edinburgh EH4 2XU, UK. Tel.: +44 7793954384.

E-mail address: [email protected] (D. Fouad).

210-2612/$ – see front matter © 2012 Surgical Associates Ltd. Published by Elsevier Ltdoi:10.1016/j.ijscr.2012.03.007

bocytosis at presentation.012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.

cardiogenic shock, septic shock, or hypovolaemia. Mesentericvenous thrombosis often presents with acute abdominal pain in ayounger cohort. Symptoms may be less dramatic, and the diagnosisdifficult to establish. Most patients will have one or more risk fac-tors for a generalised pro-thrombotic state. These risk factors maybe congenital or acquired and include hereditable thrombophilicabnormalities such as deficiency of the natural anticoagulantsantithrombin and protein C and S or inheritance of the commongain of function mutations factor V leiden and the prothrombingene G20210A mutation. Acquired prothrombotic states may ariselocally from the abdominal cavity such as acute pancreatitis, activeinflammatory bowel disease, malignancy or sepsis or may resultfrom a systemic procoagulant state such as that resulting from theuse of the combined oral contraceptive pill or the development ofantiphospholipid syndrome or myeloproliferative disease.2

We describe a case of acute ischaemia of the small bowel dueto mesenteric venous thrombosis secondary to essential throm-bocythaemia, which was managed with intravenous heparin anda second look laparotomy resulting in preservation of the smallbowel.

2. Presentation of case

A 41 year old Caucasian woman presented to the General Surgi-cal Unit with a 5 day history of continuing central abdominal pain

. All rights reserved.

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nd gastrointestinal upset characterised by decreased appetite,ausea and vomiting which had initially been treated as a gastritisy her General Practitioner She had no significant past medical his-ory, in particular she had no personal or family history of venoushromboembolism despite previous use of the combined contra-eptive pill and a successful pregnancy. She had no features ofre-existing inflammatory bowel disease or overt liver disease. Shemoked 20 cigarettes a day.

On examination she had a sinus tachycardia, a blood pressuref 120/90 mmHg and apyrexial. Abdominal examination confirmedight lower quadrant tenderness. Blood results revealed elevationf her inflammatory markers (white cell count: 24.8 and a C-eactive protein: 87) and full blood count showed a haemoglobinf 148 g/l and platelets of 175 × 109/l. Her chest, abdominal X-raynd ultrasound scan of abdomen were unremarkable.

With a presumptive diagnosis of appendicitis, a laparoscopyas performed, however this was converted to a laparotomy in

ight of the findings. Two separate segments of small bowel 30 cmn length in her distal and mid small bowel were noted to beschaemic but potentially viable. The mesenteric vessels supplyinghese two segments of small bowel appeared thrombosed but shead a good pulse in the origin of her superior mesenteric artery.

t was decided not to resect these segments at this stage but toommence therapeutic dose unfractionated heparin and perform aecond look laparotomy 24 h later to assess bowel viability. At theecond laparotomy, both previously noted ischaemic small bowelegments appeared viable and resection was not required. Theatient was maintained on therapeutic heparin infusion. The use ofnfractionated heparin in this case was favoured because it offeredhe option for rapid discontinuation and reversal in the event ofleeding.

In light of the surgical findings and the lack of an iden-ifiable intra-abdominal precipitant for the mesenteric veinhrombosis the patient underwent investigations for a varietyf systemic prothrombotic disorders including; anti-phospholipidyndrome, myeloproliferative disease and paroxysmal nocturnalaemoglobinuria. The patient was found to have a mutation in JAK2V617F positive) leading to a diagnosis of JAK2 positive myelopro-iferative disease with a phenotype of essential thrombocythaemia

as established.Prior to discharge she was commenced on cytoreductive ther-

py to reduce her platelet count to a normal range in the form ofydroxycarbamide and formally anticoagulated with warfarin with

view to maintaining an INR target of 2.5 long-term. These strate-ies are designed to reduce the risk of future occlusive arterial andenous events.

. Discussion

Essential thrombocythaemia is a chronic myeloproliferative dis-rder (MPD) characterised by an increase in the total number ofirculating platelets due to persistent megakaryocyte proliferation.

mutation due to the substitution of valine to phenylalanine inosition 617 of the JAK2, an important signalling protein, rendersematopoietic cells more sensitive to growth factors such as ery-hropoietin and thrombopoietin.3 Mutations in JAK2 are found inpproximately 50–60% of cases of essential thrombocythaemia.4

he clinical manifestations of essential thrombocythaemia resultrom platelet activation,5 hyperviscosity, tissue hypoxia and vascu-ar occlusion6 with arterial thrombosis occurring more commonlyhan venous thrombosis.7 Mesenteric and portal vein thrombosis

re recognised rare complications of myeloproliferative disease.tudies have reported that JAK2 V617F positivity can range from2 to 74% in patients with splanchnic vein thrombosis.8,9 The diag-osis of MPD should be considered in these cases where there is no

PEN ACCESSrgery Case Reports 3 (2012) 260– 262 261

other obvious local intra-abdominal precipitant of thrombosis evenwhen the FBC is normal as cases have been described where theinitial thrombotic event predated the development of the typicalperipheral blood count abnormalities which characterise MPD.10

The incidence of acute mesenteric ischaemia is difficult to estab-lish due to the lack of population based data, however, Acostareported an incidence of 12.9 per 100,000 person-years in aSwedish cohort.11 It is likely that with an ageing population theincidence will increase.12

The diagnosis of acute mesenteric ischaemia can at times bedifficult to establish and often presents late with peritonitis. Themajority of patients will have a leucocytosis and an elevatedlactate level, however these findings are not specific to acutemesenteric ischaemia.13 Computer tomography (CT) accuratelydiagnosed mesenteric arterial occlusion or ischaemic bowel in 89%of the patients while mesenteric angiography confirmed mesen-teric thrombosis or embolism in 94% of cases according to a studyby Park et al.14 We would advocate the use of CT angiography toevaluate patients thought to have acute mesenteric ischaemia.

Immediate revascularisation and/or removal of necrotic bowelare the aims of therapy, with several options available dependingon the clinical scenario. These include: surgical management suchas arterial reconstruction or bypass, endovascular managementsuch as angioplasty and/or stenting or intraluminal thromboly-sis, or medical management with intravenous heparin. In additionbowel resection with immediate or delayed anastomosis may berequired.15 Despite advances in diagnostic modalities and treat-ments, the morbidity and mortality of acute mesenteric ischaemiaremains high, with studies reporting mortality rates between 53%and 93%.9

During laparotomy, assessment of bowel viability is critical.Simple measures such as intestinal colour, visible peristalsis andmesenteric pulsations or more complex methods such as the useof mesenteric colour duplex ultrasonography and the fluoresceinuptake test can be used.16,17 Either way preservation of bowel iscritical as resection of potentially viable bowel can lead to compli-cations at a later stage such as short bowel syndrome requiring longterm total parenteral nutrition and its associated complications.18

Second look laparotomy, carried out 24–48 h later, was firstintroduced by Shaw in 1965 to allow reassessment at an early stageon bowel viability. In addition it provides the opportunity to reviewthe effects of medical treatment such as intravenous heparin toallow for potentially compromised bowel to become viable.19 Arecent review of studies evaluating second look operations for acutemesenteric ischaemia found that although less than 50% of thepatients underwent a second look procedure, more than 40% ben-efited from this due to timely identification of on-going pathologyand appropriate management at the time of the procedure.15

It can be argued that these patients should be managedaggressively with the liberal use of second look laparotomies toimprove morbidity and mortality in this cohort. However, sec-ond look laparotomies carry the disadvantages of a further generalanaesthetic, increased risk of wound complications and post-operative infections. A study comparing planned re-laparotomywith laparotomy on demand found planned re-laparotomy to havean increased mortality rate.20 Therefore, second look laparotomiesshould be carried out according to the clinical scenario.

4. Conclusion

The occurrence of intra-abdominal venous and arterial throm-bosis in association with a myeloproliferative disease prior to thedevelopment of the typical peripheral blood phenotype is well doc-umented. However, this case highlights the potential for bowel

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alvage following acute mesenteric ischaemia with the use of hep-rin and selective second look laparotomy.

onflicts of interest statement

All authors declare they have no conflicts of interest.

unding

No funding has been received for the publication of thisanuscript.

thical approval

Written informed consent was obtained from the patient forublication of this case report. A copy of the written consent is avail-ble for review by the Editor-in-Chief of this journal on request.

uthor contributions

Dina Fouad contributed towards case analysis and collection,iterature review, writing and editing. Shayanthan Nanthakumaranontributed case analysis, literature review, writing and editing.eter King has done editing. Henry Watson and Colin Millar haveone writing and editing.

eferences

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2. LeFrancois C, Derlon A, Le Querrec A, Justum AM, Gautier P, Maurel J, et al. Mesen-teric venous thrombosis. Risk factors, treatment and outcome. An analysis of 18cases. Ann Fr Anes Reanim 1994;13(2):182–94.

3. McLornan D, Percy M, McMullin MF. JAK2 V617F: a single mutation in the myelo-proliferative group of disorders. Ulster Med J 2006;75(May (2)):112–9.

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4. Sokołowska B, Nowaczynska A, Bykowska K, Chocholska S, Wejksza K, Walter-Croneck A, et al. JAK2 mutation status, hemostatic risk factors and thrombophilicfactors in essential thrombocythaemia (ET) patients. Folia Histochem Cytobiol2011;49(2):267–71.

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