Hyponatremic Hypertensive Syndrome Presenting as Malignant Hypertension in a Four-Year-Old Girl

The prevalence of renal and renovascular hypertension in the general population is not known precisely; in children it probably accounts for the majority of cases of secondary hypertension. The symptoms of renovascular hypertension vary, and sometimes it can be asymptomatic. In rare cases, clinical manifestations can include electrolyte disorders including hyponatremia. An uncommon etiology of true hyponatremia of renal origin is the hyponatremic hypertensive syndrome. It has been reported in adults with malignant hypertension, but was considered as an unusual form of presentation of renovascular disease in children. In this report, a four-year-old girl presented with hypertensive encephalopathy related to the hyponatremic hypertensive syndrome caused by right renal artery stenosis. Treatment with percutaneous angioplasty was successful, with total resolution of symptoms. This case emphasizes the importance of routine blood pressure evaluation as a major contribution to the prevention of morbidity and mortality associated with severe forms of hypertension in children.     

Reno-vascular hypertension in childhood: a nationwide survey

Renovascular disease accounts for 8–10% of all cases of paediatric hypertension, whereas, in adults, its incidence is approximately 1%. The Turkish Paediatric Hypertension Group aimed to create the first registry database for childhood renovascular hypertension in Turkey. Twenty of the 28 paediatric nephrology centres in Turkey responded to the survey and reported 45 patients (27 girls, 18 boys) with renovascular hypertension between 1990 and 2005. The age at presentation ranged from 20 days to 17 years. The mean blood pressure at the diagnosis was 169/110 mmHg. Chief complaints of symptomatic patients were headache (38%), seizure (18%), epistaxis (4%), growth retardation (4%), cognitive dysfunction (4%), polyuria (2%), palpitation (2%), and hemiplegia (2%). Renovascular hypertension was found incidentally in 11 children. The diagnosis of renovascular hypertension was established with conventional angiography in 39 patients, MR angiography in three, CT angiography in two, and captopril diethylene triamine penta-acetic acid (DTPA) scintigraphy in one patient. Twenty-one children had bilateral renal artery stenosis and 24 had unilateral renal artery stenosis. Of these, 14 (31%) had fibromuscular dysplasia; 12 (27%) Takayasu’s arteritis; six (13%) neurofibromatosis; two (5%) Williams syndrome; one (2%) Kawasaki disease; one (2%) mid-aortic syndrome; one (2%) extrinsic compression to the renal artery, and eight (18%) unspecified bilateral renal artery stenosis. Hypertension was controlled with antihypertensive drugs in 17 patients. Percutaneous transluminal angioplasty (PTRA) or surgery had to be performed in 28 patients: PTRA in 16 patients, PTRA + surgery in one patient and surgery in 11 patients (four nephrectomies). The importance of vasculitic disease, especially Takayasu’s arteritis, should not be underestimated in children with renovascular hypertension.     

Cause of primary vaginal hydrocele and ascites in advanced liver cirrhosis

The use of percutaneous transluminal angioplasty as the primary treatment of renovascular stenosis in adults has recently been described. Previously, only three children have been reported to have undergone transluminal angioplasty for stenosis of the renal artery and hypertension. At our hospital, transluminal angioplasty was attempted in four children with renal artery stenosis; one attempt was successful and three were unsuccessful. The three patients who required surgical repair of the renal artery after unsuccessful transluminal angioplasty have been described in detail. The histopathology of the stenotic vessels is also discussed. Based on the analysis of the three children, certain criteria have been derived to select pediatric patients with renovascular hypertension either for attempted transluminal angioplasty or for primary surgical revascularization.     

Fibromuscular dysplasia of the renal artery: Management and outcome

Renovascular hypertension may be caused by atherosclerotic disease or less commonly by fibromuscular dysplasia (FMD) of the renal arteries. Fibromuscular dysplasia is the commonest cause of renal artery stenosis in the younger age group and affects women predominantly. A review of our clinical database identified all patients with renovascular hypertension. All relevant clinical, biochemical and radiological findings on those with FMD were noted. The outcome of percutaneous transluminal renal angioplasty (PTRA) or reconstructive surgery was evaluated. Eight out of 62 (13%) patients with hypertension secondary to renovascular disease had FMD (all female; bilateral in four; mean age at diagnosis 37.6 years; age range 12–70 years). The mean duration of hypertension before the diagnosis of FMD was 3.3 years (range 3 months–10 years). A renal artery bruit was detected in five, hypertensive retinopathy in three and one had mild renal insufficiency. Twelve PTRAs were attempted on 10 stenotic lesions in six women. This cured the hypertension in three, while the other three have required less antihypertensive therapy. Percutaneous transluminal renal angioplasty was complicated by a trivial renal artery dissection in one, and a small upper pole infarction in another. One patient required a repeat PTRA. The other two women presented before the availability of PTRA and had successful reconstructive surgery. Fibromuscular dysplasia was the cause of hypertension in eight out of 62 (13%) patients with renovascular hypertension. Percutaneous transluminal renal angioplasty has shown encouraging results with a low complication rate. If technically feasible, PTRA should be attempted on all patients with FMD of the renal artery.     

Fibromuscular dysplasia of the renal artery: Management and outcome

SUMMARY: Renovascular hypertension may be caused by atherosclerotic disease or less commonly by fibromuscular dysplasia (FMD) of the renal arteries. Fibromuscular dysplasia is the commonest cause of renal artery stenosis in the younger age group and affects women predominantly. A review of our clinical database identified all patients with renovascular hypertension. All relevant clinical, biochemical and radiological findings on those with FMD were noted. the outcome of percutaneous transluminal renal angioplasty (PTRA) or reconstructive surgery was evaluated. Eight out of 62 (13%) patients with hypertension secondary to renovascular disease had FMD (all female; bilateral in four; mean age at diagnosis 37.6 years; age range 12–70 years). the mean duration of hypertension before the diagnosis of FMD was 3.3 years (range 3 months-10 years). A renal artery bruit was detected in five, hypertensive retinopathy in three and one had mild renal insufficiency. Twelve PTRAs were attempted on 10 stenotic lesions in six women. This cured the hypertension in three, while the other three have required less antihypertensive therapy. Percutaneous transluminal renal angioplasty was complicated by a trivial renal artery dissection in one, and a small upper pole infarction in another. One patient required a repeat PTRA. the other two women presented before the availability of PTRA and had successful reconstructive surgery. Fibromuscular dysplasia was the cause of hypertension in eight out of 62 (13%) patients with renovascular hypertension. Percutaneous transluminal renal angioplasty has shown encouraging results with a low complication rate. If technically feasible, PTRA should be attempted on all patients with FMD of the renal artery.     

Superior mesenteric artery stenting to augment splenorenal arterial bypass for intractable complex renovascular hypertension

An alternative source of visceral arterial inflow is sometimes necessary for renal revascularization when aortorenal endarterectomy or bypass is inappropriate due to severely diseased aorta. We report the case of a 46-year-old male with recurrent intractable renovascular hypertension in renal failure secondary to occlusion of the celiac axis due to progression of aortoarteritis following splenorenal arterial bypass performed 5 years before. Aortogram visualized intestinal arterial arcade supporting patent bypass with critically stenosed superior mesenteric artery. Successful angioplasty with stenting of superior mesenteric artery restored adequate renal flow through the bypass leading to recovery and easy control of hypertension.     

Renovascular hypertension

Renal artery stenosis is one of the most important forms of secondary hypertension. For years, the only causative treatment was nephrectomy. With rapid advances in cardiovascular and transplantation surgery, operative procedures in renovascular hypertension become more and more sophisticated. Revascularization is superior to medical management of renovascular hypertension in terms of preserved renal function. In recent years, surgical result have been excellent, and even patients with rather complex forms of renovascular hypertension have been successfully operated upon. New classes of antihypertensive drugs, particularly beta-blockers and angiotensin I converting enzyme inhibitors, have enabled the control of blood pressure in most patients with renovascular hypertension but do not assure preservation of renal function. Finally, a fascinating technique, the percutaneous transluminal renal angioplasty, has rapidly advanced to become one of the most popular methods in the treatment of hypertension secondary to renal artery stenosis. However, percutaneous transluminal renal angioplasty is the treatment of choice for most nonostial, nonocclusive lesions.     

Captopril renal scintigraphy--an advance in the detection and treatment of renovascular hypertension

Despite the risks associated with renovascular hypertension and the durable benefits of revascularization, the detection of patients with renovascular hypertension and the selection of those who will benefit from interventional therapy remains a challenge. We have previously documented the reliability of captopril renal scintigraphy in predicting angiographically significant renal artery stenosis in patients suspected of having renovascular hypertension. In the present study we report our recent experience with this noninvasive technique in predicting outcome after revascularization. Captopril renal scintigraphy involves the administration of 50 mg of captopril 3 hours after a baseline technitium-99m diethylenetriaminepentaacetic acid renal scan and 1 hour before a repeat captopril renal scintigraphy scan. Nineteen of the last 70 patients with clinically suspected renovascular hypertension undergoing captopril renal scintigraphy had abnormal renal scan outcomes, and 17 had a decrease in flow or function after captopril (positive captopril renal scintigraphy). Eight of these 17 with abnormal findings on captopril renal scintigraphy underwent revascularization, and the hypertension was cured or improved in six of the eight: two of three after surgical bypass grafting and four of five after angioplasty. In the seven surviving patients with abnormal renal scan results but no change with captopril (negative captopril renal scintigraphy), improvement in hypertension after treatment occurred in only one: one of two after nephrectomy, zero of three after bypass surgery, and zero of two after angioplasty (p less than 0.05). We conclude that captopril renal scintigraphy is an accurate predictor of hypertension response to revascularization. Further evaluation of this new noninvasive technique for assessing patients with suspected renovascular hypertension appears warranted.     

Atherosclerotic renovascular disease: diagnosis and treatment

The technical expertise and tools required to treat renovascular obstruction have become commonplace, and many series of patients revascularized with surgery, balloon angioplasty or endovascular stenting have been reported. Nevertheless, although hypertension and renal failure are easy to diagnose, their cause often remains elusive. Evidence is developing that patients with hypertension and atherosclerotic renal artery stenosis may often have hypertension and renovascular disease but not hypertension because of renovascular disease. As a result, diagnosis and therapy are increasingly directed towards the preservation of renal function, and the future of renal revascularization will depend on how well potential therapies address this goal.     

Renovascular hypertension in children: current concepts in evaluation and treatment

Since 1981, we have evaluated and treated 22 children with renovascular hypertension (RVH). Seventeen patients had stenosis of their native renal arteries, and five had stenosis of the artery in a transplanted kidney. RVH was caused by fibromuscular dysplasia in 13 patients, by trauma in 2 patients, and by arteritis in 2 patients. Among the patients who had transplanted kidneys, three had technical causes for stenosis and two had stenosis due to rejection. The disease was unilateral in 10 patients, bilateral in 5, and present in a solitary kidney in 7, including the five renal transplants. Diagnostic studies that strongly suggested the presence of renovascular disease were an initial diastolic blood pressure greater than 100 mm Hg, an elevated peripheral vein renin activity level, and an abnormal renal scan if the patient's hypertension was being controlled with an angiotensin-converting enzyme inhibitor (ACEI). Only the renal arteriogram was 100% accurate in confirming the presence of RVH. Percutaneous angiographic correction was attempted in 13 patients and resulted in lasting improvement of the hypertension in five (38%). Surgical revascularization was attempted in 17 children, including the 8 with failed angioplasty, with improvement or cure of the hypertension in 15 patients (88%). Combining percutaneous transluminal angioplasty (PTA) and surgical results gave 20 of 22 patients (91%) with cure or improvement of their hypertension. Four of 27 affected kidneys (15%) could not be revascularized and were removed. We conclude from this series of patients that despite improvements in noninvasive studies, renal arteriogram remains the only study that is 100% accurate in evaluating children for RVH.(ABSTRACT TRUNCATED AT 250 WORDS)     

The role of unilateral nephrectomy in the treatment of nephrogenic hypertension in children

OBJECTIVES: To define the efficacy of unilateral nephrectomy in a large series of patients presenting with renal disease and hypertension, as the latter may be a prominent finding in children with nephrourological disease (renal parenchymal disease, renovascular disease, obstruction, renal dysplasia and cancer). PATIENTS AND METHODS: We retrospectively reviewed the hospital and outpatient records of 118 children who presented for evaluation with hypertension, and who had a nephrectomy between 1968 and 2003. Patients included in the study were those who had a unilateral nephrectomy for benign renal hypertension with a normal contralateral kidney; in all, 21 had complete records and follow-up were evaluated. The hypertension was associated with primary renal disease, obstruction and renovascular disease. Blood pressure and medication requirements were compared before and after surgery, the blood pressure values also being compared with published nomograms. RESULTS: Patients were diagnosed with hypertension at a median age of 5 years and had a nephrectomy at a median of 11 months after the diagnosis. The median follow-up after surgery was 39 months. Most patients responded well and became normotensive, or there was a reduction in the need for medication. The median time to normalization was 2, 10 and 11 days in patients with primary renal disease, obstruction and renovascular disease, respectively. CONCLUSION: Nephrectomy is successful in normalizing blood pressure in children with benign renal hypertension and with a normal contralateral kidney.     

Captopril-enhanced renal scintigraphy in the diagnosis of pediatric hypertension

Hypertension in childhood is no longer a rare condition mainly secondary to renal, or renovascular diseases, as a growing proportion of children are obese and hypertensive, with the phenotype of metabolic syndrome. Thus, we need to reconsider our practice in the examination of the hypertensive child and redefine the place of non-invasive methods for screening of renovascular hypertension, and specifically, to evaluate the value of captopril-enhanced renal scintigraphy at the two ends of the palette: the obese child with hypertension and the severely hypertensive prepubertal child. Renal artery stenosis in children is mainly due to fibromuscular dysplasia and stenoses associated with syndromes involving single or multiple smaller branch vessels. This explains the low specificity and sensitivity of the color-Doppler ultrasound method and captopril renal scintigraphy. Even the more sophisticated computed tomography (CT) and magnetic resonance imaging (MRI) angiographic techniques are, at present, not sensitive enough to exclude stenoses of the small branches definitely. Thus, children in whom there is a strong suggestion of renovascular hypertension should undergo angiography with a view to endovascular treatment, as non-invasive imaging has no significant benefit and might lead to a delay in treatment. In the cases when the probability of renovascular disease is moderate a basic assessment of renal function and structure is sufficient. In the neonate, catheter-associated thromboembolic disease is among the most common causes hypertension. It should be controlled medically until the patient is old enough to undergo angiography and angioplasty successfully. Thus, in this age group, there is a place for functional imaging with renal sonography and angiotensin-converting enzyme inhibitor (ACEI) renography to detect hemodynamically significant renovascular disease, with the limitations mentioned above. However, the rapid technical evolution of non-invasive methods requires periodic re-consideration of the actual standpoints.     

Yield of renal arteriography in the evaluation of pediatric hypertension

The prevalence of renovascular disease is estimated to be 3%–5% in pediatric patients with hypertension. The utility of non-invasive imaging tests has not been evaluated in children, and renal arteriography remains the diagnostic test of choice. However, there are no established guidelines for the application of this test and information is not available about the likelihood of detecting an abnormality if an arteriogram is performed in children with hypertension. Therefore, we reviewed the yield of renal arteriography in pediatric patients if the test was performed based on the following two criteria: (1) severe hypertension exceeding the 99th percentile for age and sex or (2) failure to control high blood pressure with one antihypertensive drug. During the period 1983–1998, 28 children (mean age 11.7 years) who satisfied one of the above criteria underwent renal arteriography to investigate hypertension. None of the patients were renal transplant recipients. The average duration of hypertension was 11 months and the peak blood pressure was 168/107 mmHg. The renal arteriogram was abnormal in 12 patients (43%). Unilateral renal artery stenosis was the most-common abnormality. When the patients were divided into two groups – those with an abnormal or normal test result – they did not differ in age, sex, or racial distribution. The peak systolic blood pressure was higher in children with an abnormal renal arteriogram (P<0.05). Among those undergoing the arteriogram on the basis of the first criterion, i.e., severe hypertension, 11 of 23 (48%) studies were abnormal. Five children had an arteriogram based on the second criterion – failure to control the blood pressure with one medication – and in 1 patient (20%) the test was abnormal. We conclude that the prevalence of renovascular disease in a population of hypertensive children subjected to renal arteriography is around 40%. Two clinical criteria – namely severe hypertension or failure to control hypertension effectively with one drug – are useful to guide the application of renal arteriography in children with hypertension. Received: 12 August 1999 / Revised: 24 November 1999 / Accepted: 28 November 1999     

Renovascular hypertension in children.

OBJECTIVE: To study the etiology, clinical spectrum. image findings, management and outcome of children with renovascular hypertension (RVH). MATERIAL AND METHODS: Twenty children (aged 5 days to 15 years) were studied and treated for RVH during 1977-1998. In 14 cases hypertension was found during a routine examination. Six cases had heart failure and/or hypertensive encephalopathy. Diagnosis was made with aortography. Post-captopril renography and Doppler ultrasonography were obtained in 8 patients and spiral computed tomography angiography in 2. Treatment consisted of surgery (8 patients), percutaneous transluminal angioplasty (PTA) (5) or antihypertensive drugs only (8). RESULTS: Initial blood pressure was 62 +/- 31 mmHg > 95th percentile for systolic and 44 +/- 22 mmHg for diastolic blood pressure. Twelve children had unilateral and 8 had bilateral arterial stenosis. In 3 cases lesions were intrarenal. RVH was due to fibromuscular dysplasia (7 patients) and associated to middle aortic syndrome (5). neurofibromatosis (3), William's syndrome (2). Takayasu's arteritis (1) and pheochromocytoma (1). Treatment of choice was decided depending on the size of the child and location and severity of the stenosis. At the end of the follow-up (78 +/- 49 months), 9 patients are normotensive without medication and 7 are normotensive with drugs. Three patients have died, 2 for unrelated causes and I for cardiac failure; 1 child was lost to the follow-up. CONCLUSIONS: Although symptoms are relatively uncommon. renovascular disease is a frequent cause of severe hypertension in childhood. Non-invasive diagnostic techniques appear useful as screening methods. Treatment by surgery or PTA is successful if patients are carefully selected.     

Renovascular hypertension

Renovascular hypertension is the most common cause of secondary hypertension. Interest in identifying patients with renal artery stenosis has been stimulated recently by advances in three areas. First, is the realization that not only can renal artery stenosis cause renovascular hypertension, but it can also lead to progressive renal failure (ischemic nephropathy) caused by progression of disease, usually atherosclerotic in nature. Second, advances in percutaneous transluminal renal angioplasty and, especially, the recent use of renal stents has led to a less invasive management of these patients as compared with traditional renal revascularization. Finally, the development of newer less invasive diagnostic techniques, both for the identification of patients with renal artery stenosis and to follow patients with known renal artery stenosis, has simplified the diagnostic aspect of the disease.     

Treatment of renal hypertension.

There are different types of renal hypertension: hypertension due to parenchymal renal disease, renovascular hypertension, hypertension due to urological disease, hypertension of endstage renal disease. Treatment has to consider-above all-the possibility of specific, medical or surgical procedures that may cause the underlying condition. If the underlying disease is not amenable to specific therapy, symptomatic medical treatment to lower blood pressure is indicated: besides control of sodium-intake and body weight antihypertensive drugs are generally indicated. We use them, alone or in combination, in the following line of order: diuretics, beta-adrenergic blockers, dihydralazine, reserpine, clonidine, alpha-methyldopa, guanethidine.     

肾血管性高血压的介入治疗

目的：研究经皮腔内血管成形术加内支架植入术治疗肾动脉狭窄致致肾血管性高山压的临床效果。方法：选用Palmaz支架,对7例大动脉炎、2例动脉弱样硬化,3例纤维肌性发育不良所致肾动脉病变先行经皮腔内血管成形术,然后放置支架,术后患者行常规抗凝治疗,结果：12例患者,经皮腔内血管成形术加支架植入术技术成功率为100％,血压下降60－120／35－100mmHg近期随访－32个月未见复发,结论：经皮腔内血管成形术加支架植入术治疗肾血管性管高血压效果满意,创伤小,患者痛苦少,为肾动脉狭窄所致肾血管性高血压较理想的治疗方法。     

Renal artery revascularisation can restore kidney function with absent radiotracer uptake

Background

Children with renovascular hypertension often present with severe hypertension. Some children have severe obstruction of their renal arteries resulting in <10% relative function on [^99mTc]dimercaptosuccinic acid (DMSA) scan. Conventional treatment of these children has been nephrectomy of the poorly functioning kidney to normalise their blood pressure (BP).

Case-Diagnosis/treatment

We describe three children aged 20 months to 9 years with severe renal artery stenosis and severe hypertension who had radionucleotide uptake of 0% in one kidney. In one case, no renal perfusion was demonstrated by duplex ultrasound scan. Significant recovery of relative renal function of 18 to 52% was achieved after revascularisation by percutaneous angioplasty or open surgery of the obstructed renal artery.

Conclusion

These cases illustrate that scintigraphy alone cannot be used to predict salvageable function in children with renovascular disease.     

Evaluation and preparation for surgical treatment of renal artery disease

The diagnosis of renovascular disease and renovascular hypertension is outlined. A comparison and analysis of the advantages of three forms of treatment are made. These include medical management, percutaneous transluminal coronary angioplasty, and surgical intervention. Selection of patients for revascularization to preserve renal function is discussed, and guidelines for determining renal salvageability are presented. Surgical revascularization is the treatment of choice for patients with ostial atherosclerotic renal artery disease, branch renal artery disease, or a renal artery aneurysm.     

Surgical treatment of complete renal artery occlusion in pediatric patients

Two children, 8 and 11 years old, presented with severe hypertension secondary to unilateral and bilateral total occlusion of the renal arteries, respectively. The 11-year-old developed sudden anuria requiring hemodialysis. Successful surgical reconstruction allowed recovery of renal function and normal blood pressure in both patients. Routine blood pressure control in the pediatric patient population, high clinical awareness, and judicious use of arteriography, provide the best chance for early diagnosis of renovascular disease. Surgical revascularization or transluminal angioplasty are the treatment modalities of choice in appropriately selected cases of renal artery stenosis. When total occlusion occurs, retrieval or preservation of renal function can be successfully achieved by direct surgical intervention.