Departments of Pediatric Intensive Care and Pediatric Cardiology,
Royal Hospital, Muscat, Oman

In view of persistence of severe pain two days after admission, a pediatric medical consultation was requested. Abdominal examination was not contributory. However a detailed cardiovascular examination revealed feeble femoral pulses with systolic hypertension in the upper extremities (BP in left arm 173/105 mmHg, left leg 128/98 mmHg). Precordial examination showed normal heart sounds, systolic ejection click and grade 3/6 ejection systolic murmur in the upper left sternal border. The ocular fundi were normal. Electrocardiogram (ECG) revealed evidence of left ventricular hypertrophy. Chest X-ray showed cardiomegaly with the aortic indentation producing the reverse sign. Echocardiogram showed severe post-subclavian coarctation of aorta with pressure gradient of 50 mmHg by Doppler and bicuspid aortic valve. The abdominal pain was considered secondary to the severe hypertension with reduction in renal and enteric blood flow,¹ as no other cause of pain could be identified. The child was shifted to the pediatric intensive care unit and started on labetalol and morphine infusion for the control of severe hypertension and abdominal pain. Color Doppler study of the celiac axis and superior mesenteric artery were normal.  After 24 hours, his hypertension and abdominal pain resolved. He was started on oral propranolol and cardiac catheterization done later revealed classical post-subclavian coarctation (Fig. 1). He subsequently underwent elective transcatheter balloon dilation of coarctation of aorta in our hospital and remains well with normal blood pressure on follow-up.

Acute abdominal pain can be a distressing experience for a child and elucidation of its cause sometimes remains difficult for the treating doctor. Symptoms associated with surgically correctable causes include bile-stained vomitus, asymmetric pain, local tenderness and peritonism.²  Common causes of acute abdominal pain in children include appendicitis, mesenteric adenitis and non-specific abdominal pain. Uncommon causes include infective diarrhea, food poisoning, sickle cell crisis, gall stones, pancreatitis, peptic ulcer, acute intermittent porphyria, malrotation, intussusception, ureteric calculi, urinary tract infection, pneumonia, diabetic ketoacidosis and Henoch-Schonlein purpura. Coarctation of aorta is the most common correctable cardiovascular cause of hypertension. It occurs in 6% to 8% of all cases of congenital heart defects and twice as common in males. The diagnosis is often missed unless the patient develops congestive heart failure usually in early infancy or presents with a cardiac murmur and hypertension usually in older children. Symptoms of late presentation may also include headache, fatigue, chest pain, pain in the legs after exercise or rarely intracranial hemorrhage. The diagnosis of coarctation of aorta was obviously delayed in the surgical ward for the above child due to lack of attention to the admission BP of 143/87 mmHg while in pain, an incomplete clinical examination and absence of a chest X-ray in the initial work up. Acute abdominal pain which responded only to parenteral antihypertensives and narcotic analgesics is a rather unusual presenting symptom of coarctation of aorta with hypertension and poorly documented in the medical literature.^3,4 It has been reported after balloon dilation or surgical repair (post-coarctectomy syndrome) due to mesenteric arteritis.^1,5 Aortic dissection is extremely rare in childhood. Complications of coarctation of aorta include hypertension, left ventricular failure, aortic dissection, premature coronary artery disease, infective endocarditis, and cerebrovascular accidents.⁶ Surgical repair or balloon angioplasty is indicated in patients with a transcoarctation pressure gradient of >30 mmHg. Post-procedure complications include residual or recurrent hypertension, recurrent coarctation, development of aortic aneurysm and the possible sequelae of associated bicuspid aortic valve. In a pediatric review regarding early diagnosis of coarctation of aorta, the mean and median ages at referral were 8.4 and 5.8 years, respectively.⁷ Since early detection and treatment are essential to prevent the complications, routine measurement of upper and lower limb blood pressures during at least one physical examination after the neonatal period is advisable.

1. Hauser M, Kuehn A, Wilson N. Abnormal responses for blood pressure in children and adults with surgically corrected aortic coarctation. Cardiol Young 2000; 10: 353–357

2. Davenport M. Acute abdominal pain in children. BMJ 1996; 312: 498–501

3. Ivanov AM. Abdominal syndrome in aortic coarctation simulating acute appendicitis. Klin Med (Mosk)1991; 69: 90–91

4. Ingu A, Morikawa M, Fuse S, Abe T. Acute occlusion of a simple aortic coarctation presenting as abdominal angina. Pediatr Cardiol 2003; 24: 488–489

5. Sealy WC. Paradoxical hypertension after repair of coarctation of the aorta: a review of its causes. Ann Thorac Surg 1990; 50: 323–329

6. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults, First of two parts. N Engl J Med 2000; 342: 256–263

7. Ing FF, Starc TJ, Griffiths SP, Gersoni WM. Early diagnosis of coarctation of the aorta in children: a continuing dilemma. Pediatrics1996; 98: 378–382