Case no. 1
A 23-year-old, nonsmoking, Saudi male was referred to our hospital complaining of abdominal pain for 18 months. He was well until 14 months prior to presentation, when he started to complain of intermittent colicky, mostly right-sided abdominal pain, not related to meals and associated with occasional nausea, vomiting, and frequent bowel motion up to six or seven times daily but without blood or mucous. Four months after developing the pain, his appetite decreased and he lost 30 kg in weight. His family history was unremarkable except for a history of CD in third-degree relatives. On examination he was found to be thin built and not in distress, and his vital signs were normal. His abdomen was mildly tender in the right upper quadrant with no palpable masses or organomegaly. Laboratory investigations showed the following: white blood cell count (WBC) 5.34 × 109/L, hemoglobin (Hb) 7.4 g/dL, hematocrit 24.7%, platelets 701 × 109/L, erythrocyte sedimentation rate (ESR) 75 mm/h, total bilirubin 1.5 umol/L, alkaline phosphatase 100 unit/L, alanine aminotransferase 25 unit/L, aspartate aminotransferase 24 unit/L, γ-glutamyl transpeptidase 16 unit/L, total protein 56 g/L, albumin 15 g/L, phosphorus 1.29 mmol/L, calcium 1.93 mmol/L, antithrombin III 90.1 sec (normal 75–125), activated protein C resistance 88 (normal 70–140), and protein S 54.8 L (normal 50–120). Lower gastrointestinal endoscopy showed ileitis with nodular terminal ileal mucosa and aphthous ulcers with normal colon. Multiple biopsies were taken and the histopathology revealed CD. Computed tomography (CT) enterography showed segmental wall thickening of the ileal loops with creeping fat and enlarged mesenteric lymph nodes most likely representing CD as well as partial obstruction at the distal ileum with significant dilated proximal bowel loops. There was also inferior vena cava thrombosis with central intraluminal filling defect extending to the right internal iliac vein ( and ). Pulmonary CT revealed small subsegmental pulmonary emboli. This was treated with low-molecular-weight heparin. There was no family history of TE. He was started on Pentasa, gradually increasing the dose until it reached 1 g three times daily + Caltrate, iron, and vitamin D supplements. Azathioprine was introduced with a dose of 50 mg once daily. He quickly responded to treatment and his laboratory follow-up showed a rise in his hemoglobin level reaching 10 g/dL, normalization of his calcium level, and elevation of his albumin level reaching 28 g/L. His inflammatory markers, including ESR and CRP, gradually came down.
CT scan of the abdomen showing inferior vena cava thrombosis (single arrow). Note also partial obstruction at the distal ileum with significant dilated proximal bowel loops and bowel thickening (double arrow).
Abdominal CT scan showing inferior vena cava thrombosis (single arrow) with dilated and thickened small bowel loops (double arrow).
Case no. 2
A 39-year-old, nonsmoking, Saudi male with a known case of CD was diagnosed 5 years previously in a local hospital. He was started on azathioprine, prednisolone, and mesalazine, but he was not compliant to treatment. Two years later, he developed enterovesical fistula and was started on antitumor necrosis factor (TNF) (infliximab). He received four cycles with partial improvement and then was lost for follow-up. He presented to our hospital with a 20-day history of abdominal pain, distention, and fever with chills. He presented a history of intermittent fecaluria and pneumaturia for the last few months with a history of decreased appetite and weight loss of about 10 kg. During examination, his abdomen was distended with ascites and decreased bowel sounds. He underwent a CT scan, which revealed an enterocolic fistula, superior mesenteric vein thrombosis, and a few enlarged retroperitoneal and mesenteric lymph nodes (). He was scheduled for surgery and underwent ileocecal resection, sigmoid colectomy, terminal ileostomy, and colostomy. Pathology of the resected intestines showed changes consistent with CD with ulcerations, fissuring, and fistula formation. He did not receive thromboprophylaxis postsurgery. He was stable until the 12th postoperative day when he suddenly developed shortness of breath and was found to be tachypneic, tachycardic, and hypotensive. Spiral CT of the chest showed evidence of a small left upper lobe subsegmental pulmonary embolism (). There was no family history of TE. He was started on low-molecular-weight heparin, and his workup showed a low level of antithrombin III and factor V Leiden, suggestive of underlying thrombophilia, so he was started on warfarin with a stable hospital course and restarted on anti-TNF (infliximab). Currently, he is being followed up in the clinic, is doing well, and is waiting for closure of his stomas.
CT scan of the abdomen showing thrombosis in the superior mesenteric vein (arrow). Also note the ascites.
CT scan of the chest showing pulmonary embolism (arrow).
Case no. 3
A 49-year-old, Saudi female was diagnosed as having ulcerative pancolitis and was referred to our hospital for further management. She was managed with mesalazine and prednisolone. Her disease was nonremitting. She developed left cerebrovascular accident. A Doppler ultrasound showed total occlusion of the left carotid artery and a 2-D transesophageal echo showed a normal heart with no intramural thrombus. She was not on thromboprophylaxis. During her stay at our hospital she also developed right popliteal deep venous thrombosis and pulmonary embolism. She was started on low-molecular-weight heparin but continued to have hematochezia and a drop in her hemoglobin, so it was decided that an inferior vena cava filter should be inserted. A repeat colonoscopy showed moderately severe pancolitis with multiple pseudo polyps. Histopathology was compatible with UC with no dysplasia or cytomegalovirus. Her investigations showed the following: Hb 3.8 g/dL, mean corpuscular volume 61.5, mean corpuscular hemoglobin 17.6, platelets 432,000/mm3, ESR 75, and albumin 27 g/L. Her liver function tests and renal profile were normal. All studies for thrombophilia were negative, including protein S, protein C, antithrombin III, and factor V Leiden. Lupus anticoagulant was negative and JAK2 mutation was ordered. She was started on prednisolone and azathioprine and was titrated up to 100 mg once daily in addition to aspirin 100 mg once daily. She went into remission after that and steroids were discontinued. She was maintained on mesalazine 1 g twice daily and azathioprine 100 mg orally once daily.
Case no. 4
A 17-year-old female presented to our hospital with easy fatigability, palpitation, dizziness, and pallor, and decreased appetite for 1 month. This was associated with mild abdominal pain and bleeding from the rectum. She was intentionally trying to lose weight and over a 3-month period she lost about 20 kg. Clinically, she was pale but not jaundiced and was not in respiratory distress. Her chest and abdominal examinations were normal. Her investigations showed the following: WBC 3.4 × 109/L, Hb 3.1 g/dL, platelets 106,000/mm3, calcium 8.2 mmol/L, albumin 3.4 g/L, and total bilirubin 1.55 umol/L. All other investigations were normal. She received a blood transfusion and underwent a colonoscopy. The colonoscopy showed markedly congested, erythematous mucosa of the left colon, which was oozing fresh blood with diffuse ulceration, granularity, and submucosal hemorrhage. Her upper endoscopy showed chronic gastroduodenitis. She was diagnosed as having UC of the left colon and was started on treatment with prednisolone, mesalamine, omeprazole, steroid enema, and mesalazine enema. She was discharged home in a good general condition but readmitted 2 months later with bilateral lower limb edema of 1-week duration. This was associated with pain on walking. Examination showed the following: prothrombin time (PT) 14.6 sec (control = 13.4 sec) and partial thromboplastin time (PTT) 35 sec (control = 30.2 sec); antithrombin III was slightly reduced, but protein C, protein S, plasminogen, and factor V Leiden were all within normal limits. Lupus anticoagulation, antinuclear antibodies (ANA), and anti-DNA were negative. Repeated factor III was normal; ANA, antimitochondrial antibodies, antiribosomal antibodies, antibasement membrane antibodies, and antineutrophil (ANCA) antibodies were all negative. Her Hb was 9 g/dL, WBC 5.7 × 109/L, and platelets 223,000/mm3. She was diagnosed as having bilateral deep vein thrombosis. She was not using oral contraceptive pills, and there was no family history of TE. While in hospital she developed chest pain associated with shortness of breath. Pulmonary angiography showed evidence of pulmonary embolism with a filling defect and a thrombus was seen within the right inferior pulmonary artery. She was started on anticoagulant therapy and was discharged 2 weeks later with an international normalized ratio of 3.3. She was subsequently followed up in clinic and was well.
Case no. 5
A 50-year-old, Saudi female presented to the emergency department with a 2-month history of bloody diarrhea and lower abdominal pain. Clinically, she was found to be pale with stable vital signs and a mild generalized abdominal tenderness. Initial investigations showed the following: WBC 9.4 × 109/L, Hb 8 g/dL, ESR 79, and albumin 2.6 g/dL. Her liver and renal function tests were normal. Flexible sigmoidoscopy up to the distal transverse colon revealed uniform involvement with moderately severe colitis. Histopathology was compatible with UC. She was started on mesalazine tablets in addition to prednisolone 40 mg daily. She was discharged to be followed up in the clinic. During prednisolone tapering she relapsed after 5 weeks. She presented to us again unwell with severe abdominal pain, vomiting, and fever. Clinically, she was found to have severe abdominal tenderness, guarding, and positive rebound tenderness. Her investigations showed the following: WBC 3.1 × 109/L, Hb 10.1 g/dL, PLT 152,000/mm3, INR 1.2, PTT 44, total bilirubin 2.13 umol/dL, direct bilirubin 2 umol/dL, and alkaline phosphatase 41 IU/L. Abdomen X-rays showed air under the diaphragm. She was admitted to the intensive care unit (ICU), was resuscitated, and underwent an emergency laparotomy. There was purulent fluid in the peritoneal cavity and a perforation at splenic flexure. Peritoneal lavage and subtotal colectomy were carried out, leaving the cecum and 10 cm from proximal ascending colon, which was brought out as a colostomy. The rectal stump was left behind. Her postoperative course was complicated by wound infection, which was treated with antibiotics, and intrahepatic cholestasis related to total parenteral nutrition, which resolved gradually. She was maintained on mesalazine, as she had residual colon left behind. During her stay in the ICU she developed weakness in all her limbs and was difficult to be extubated. Neurological examination revealed high mental function with intact cranial nerves but weakness in all limbs 2/5 with absent reflexes. Electromyography and nerve conduction studies revealed motor polyneuropathy that was more pronounced in the lower limbs, compatible with critical illness polyneuropathy. Her electrolytes, calcium, phosphorus, magnesium, trace elements, and vitamins were corrected and optimized. Physical therapy was intensified and, because of her prolonged stay in ICU, unfractionated subcutaneous heparin 5000 IU subcutaneously was given every 8 h. She had tracheostomy, but it was closed after improvement in her strength. The muscle strength in her limbs improved gradually, reaching 3/5 over 8 weeks after the operation, when she was found to have swelling and discoloration in her left lower limb. A Doppler ultrasound confirmed the presence of deep venous thrombosis in the left popliteal and femoral veins. Thrombophilia screening showed the following: protein S and C, antithrombin III, lupus anticoagulation, ANA, and anti-DNA were all negative. She was fully anticoagulated initially with heparin and subsequently with warfarin. Warfarin was continued for 6 months. She was maintained during this period on mesalazine tablets, in addition to suppositories. Proctoscopy and endoscopy through stoma revealed evidence of colitis. She underwent resection of remaining colon (rectum, proximal ascending colon) and had ileoanal pouch anastomosis. During follow-up over 5 years, no recurrence of thrombosis was observed.
Case no. 6
A 17-year-old, Saudi female was diagnosed as UC 9 months prior to her presentation to our hospital. Colonoscopy revealed pancolitis. She was maintained on mesalazine 1000 mg three times daily but was not compliant with her medication. She was admitted with moderate exacerbation of UC and severe iron deficiency anemia. Her investigations showed the following: WBC 6.7 × 109/L, Hb 5.3 g/dL, ESR 60, and albumin 2 g/dL. Her liver and renal function tests were normal. She received a blood transfusion and was started on prednisone 40 mg once daily and iron tablets. During her hospital stay she developed left leg swelling and calf pain. A Doppler ultrasound of the left lower limb revealed complete obstruction of the upper half of the femoral vein and popliteal vein by a thrombus. Thrombophilia study, including protein S and C, antithrombin III, and plasminogen functional chromogenic assay, was normal. Lupus anticoagulant and ANA were negative. She was started on unfractionated heparin to maintain her PTT at twice the normal limit and subsequently was put on warfarin 5 mg daily. She tolerated the treatment well but was readmitted with hematochezia 2 weeks after her discharge. Her INR was 1.8. As heparin was reported to have an anti-inflammatory effect in patients with UC, warfarin was held. It was decided to keep her on subcutaneous unfractionated heparin at a dose of 7500 IU subcutaneously twice daily. She was kept on subcutaneous heparin for 6 months, and the signs of DVT disappeared. No recurrence of DVT was noticed during her follow-up over 10 years.