CLINICAL QUIZ

An unusual cause of acute abdomen in an adolescentwith concurrent disease: Questions

Yasar Kandur & Sevcan A. Bakkaloglu & Ipek Isik Gonul &Idil Yenicesu & Koray Akkan & Buket Dalgic

Received: 17 September 2013 /Revised: 19 September 2013 /Accepted: 25 September 2013# IPNA 2013

Keywords Acute abdomen . Diabetes . Celiac disease

Case summary

A 13-year-old girl was referred to our clinic with complaintsof severe abdominal pain and vomiting. Her past medicalhistory was remarkable: she had been diagnosed with type 1diabetes mellitus (DM) 10 years previously and with celiacdisease (CD) 1.5 years previously, and she had had an episodeof left vertebral arterial occlusion resulting in transient lefthemiparesis 1 year prior to presentation. A prothrombin20210A heterozygous mutation and a high level oflipoprotein(a) (117 mg/dl; normal range 4–30 mg/dl) wereidentified, and coumadin treatment was started. Six monthslater, anticoagulation was stopped due to hemoptysis, which

spontaneously resolved. She was the offspring of consanguin-eous parents.

On physical examination, the patient was pale, miserableand severely ill. Her blood pressure was 100/60 mmHg, bodyweight was 33 kg (<5 percentile), and height was 143 cm (5–10th percentile). There was extensive abdominal tendernessand defence as signs of peritoneal irritation and an acuteabdominal emergency. The laboratory data revealed a hemo-globin level of 8.7 g/dl; white blood cells, 14,000/mm3; neu-trophils, 87 %; platelets, 1,039,000/mm3; blood urea nitrogen,10 mg/dl; creatinine, 0.35 mg/dl; aspartate aminotransferase,20 U/l; alanine aminotransferase, 11 U/l; lactate dehydroge-nase, 466 U/l; erythrocyte sedimentation rate, 112 mm/h; C-reactive protein, 145 mg/l (normal range 0–6 mg/l). Urinalysisshowed a specific gravity of 1,008, pH of 7.0 and two eryth-rocytes and one leukocyte/HPF. She had no proteinuria.Prothrombin time was 13 s, and partial thromboplastin timewas 22 s. The lipoprotein(a) level had remained high(50.2 mg/dl). Serum complement levels were normal, andviral serology, autoantibodies (ANA, Anti DNA, c-ANCA,p-ANCA) were all negative. Abdominal Doppler ultrasonog-raphy revealed no pathological findings. Due to a previousthrombotic event, abdominal angiograpy was performed forthe evaluation of acute abdominal emergency, revealing oc-clusion of the jejunal and ileal branches of the superior mes-enteric artery (Fig. 1c). Other visceral arteries of the aorta wereintact. An embolic protection device was used for recanaliza-tion so that minimal re-canalization was achieved (Fig. 1b).Since she had a toxic appearance and due to the rapid wors-ening of her clinical condition, she underwent abdominalsurgery. Peroperatively, adhesions between the ileal and colon-ic segments and focal ischemic lesions progressing to bowelwall necrosis were observed. During bridectomy of the seg-ments with adhesions, closed perforations in the colon andileum segments were observed. The ischemic segments ofthe ileum, caecum, ascending colon and proximal part of thetransverse colon were resected and ilecolic anastomosis was

The answers to these questions can be found at http://dx.doi.org/10.1007/s00467-013-2649-7.

Y. Kandur (*) : S. A. BakkalogluDepartment of Pediatric Nephrology, School of Medicine, GaziUniversity, Ankara, Turkeye-mail: yaskan30@yahoo.com

I. I. GonulDepartment of Pathology, School of Medicine, Gazi University,Ankara, Turkey

I. YenicesuDepartment of Pediatric Hematology, School of Medicine, GaziUniversity, Ankara, Turkey

K. AkkanDepartment of Radiology, School of Medicine, Gazi University,Ankara, Turkey

B. DalgicDepartment of Pediatric Gastroenterology, School of Medicine, GaziUniversity, Ankara, Turkey

Pediatr NephrolDOI 10.1007/s00467-013-2648-8

performed. The resection material revealed disseminated mu-cosal ulceration of the small intestinal wall and segmentaladhesions with the colon, with necrotizing non-granulamatousfindings affecting one of the main arterial branches in themesentery (Fig. 1c).

Questions

1. Which diagnosis is compatible with the pathologicfindings?

2. How should the condition be treated?

Fig. 1 a Selective angiography showing occlusion of the proximal partof the jejunal artery (arrow). b Proximal embolic protection device(arrow) employed an occlusion balloon advanced over a guidewire distalto the thrombus to trap and aspirate thrombotic debris released duringangioplasty and stenting procedures. c Biopsy findings of the patient:

small bowel wall (on the left side) showing diffuse mucosal ulcerationand submucosal granulation tissue formation resulting in adhesion withthe colon on the right side of the image. Hematoxlyin and eosin (H&E)staining, magnification ×12.5. Inset Occluded artery with transmuralinflammation and foci of fibrinoid necrosis in the mesentery. H&E, ×40

Pediatr Nephrol