American Journal of Emergency Medicine xxx (2015) xxx–xxx
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Facial nerve palsy in a 3-year-old child with severe hypertension—a case report Abstract We report an interesting case of a child with new-onset malignant hypertension (HTN) associated with facial paralysis. A review of the medical literature on this association and discussion of diagnostic and management aspects are included. A 3-year-old Pakistani boy was transferred to the emergency department (ED) from his pediatrician's office due to concern for facial weakness. He had presented to the ED 2 days prior with a chief complaint relayed by his father of “swollen face; we see it more when he talks,” which occurred several days after rhinorrhea and congestion. At that time, he was afebrile and well appearing (there was no blood pressure [BP] measurement); there was noted mild right facial swelling without erythema, tenderness, fluctuance, or rash. Cranial nerve function was intact. He was diagnosed with right facial swelling (due to probable allergic reaction) and discharged home with pediatrician follow-up. Two days later, there was no improvement. He was evaluated by his pediatrician, who noticed unilateral facial muscle weakness and referred him to the ED. On second ED visit, parents denied patient had fever, rash, pain/headache, visual deficit, eye redness/ discharge, ear pain, or alteration in behavior/level of activity/mental status. Perinatal history was unremarkable. Family history was positive for consanguinity (parents were cousins) but negative for HTN. Medical history was significant for thalassemia, eczema, and food allergies. Parents denied any recent travel, rashes, insect/tick bites, or known sick contacts affecting the patient. Immunizations were up to date for age. The patient was not taking any current medications. On physical examination, he was well appearing, active, smiling/playful, in no apparent distress. He had mildly dysmorphic features with triangular facies, frontal prominence, low set ears, anteverted nostrils, and micrognathia. Developmentally, his body mass index was below the third percentile. He had right-sided upper and lower facial paralysis, specifically with weakness affecting the right corner of his mouth, right eyelid, and right forehead musculature. His vision was grossly normal, with intact extraocular movements and reactive pupils. With the exception of cranial nerve 7, cranial nerves 2 to 12 were intact. He had normal strength and sensation in all 4 extremities; deep tendon reflexes were 3+ and equal bilaterally. Head ears eyes nose throat examinations were within normal limits; specifically, ophthalmologic examination was without retinal abnormality or papilledema, and there was no evidence of acute otitis media. His neck was supple with no visualized or palpable masses. Cardiac examination was normal, lungs were clear to auscultation, and abdomen was soft and nontender with no masses or bruit. Blood pressure measured at triage was 224/112; heart rate, 99 beats per minute; respiratory rate, 24 breaths per minute; peripheral oxygen
saturation, 98% (in room air); rectal temperature, 97.6°F. His BP was measured 5 times over the next hour with readings of 194/110, 205/124, 195/ 124, 195/96, and 187/111. Blood pressure was measured in all 4 extremities, and there was no discrepancy in hypertensive readings. The initial differential diagnosis included hypertensive emergency; central nervous system (CNS) pathology (stroke, central nervous system tumor, and encephalitis); malignant HTN due to cardiac, endocrine, or renal disease; and Bell's palsy. The patient was placed on a cardiac monitor, and peripheral venous access was obtained. The initial priority was to therapeutically lower the BP by 10% to 20% while under ED supervision, before leaving the department to perform radiographic imaging. He initially received a dose of oral nifedipine 6 mg (0.5 mg/kg), with little change in BP. He next received intravenous (IV) labetalol 20 mg (1 mg/kg), with decrease in BP to 153/87. Upon BP reduction, he was transported to radiology for a cranial/facial computed tomography (CT). The CT scan was normal. Upon return to the ED, his BP was 177/121; he was given another dose of oral nifedipine 6 mg (0.5 mg/kg), after which his BP declined to 147/88. He was admitted to pediatric intensive care unit. Emergency department workup was remarkable for urinalysis showing trace protein and moderate hemoglobin. Basic serum metabolic panel showed sodium 139 mmol/L, potassium 3.9 mmol/L, chloride 101 mmol/L, bicarbonate 24 mmol/L, blood urea nitrogen 9 mg/dL, creatinine 0.2 mg/dL, and glucose 132 mg/dL. Complete blood count showed white blood cell 10000/mm3, hemoglobin 13.9 gm/dL, hematocrit 43%, and platelet count 251000/UL. Venous blood gas showed pH 7.42, PaCO2 38 mm Hg, PaO2 60 mm Hg, and lactate 2.2 mmol/dL. A chest x-ray showed clear lungs and normal heart size and contour. Upon admission to pediatric intensive care unit, the patient had persistent marked elevation of BP despite receiving multiple IV doses of labetalol. An arterial line placed for continuous BP monitoring, and a nicardipine drip (1 μg/kg per minute) was initiated. Abdominal sonogram showed a relatively small left kidney (5.7 cm) compared to the right kidney (8.2 cm) with normal renal echogenicity; there was no hydronephrosis, mass, or stone. A CT angiogram of the abdomen with IV contrast was performed, which showed asymmetric left renal atrophy (Fig. 1) and decreased caliber of the left renal artery compared to the right (Fig. 2), although no focal stenotic segment was identified. During hospitalization, he underwent cardiac, infectious, rheumatologic, nephrogenic, endocrinologic, and genetic workup. Echocardiogram showed moderate left ventricle hypertrophy but no evidence of coarctation of the aorta. Infectious workup showed no growth in blood, urine, and throat cultures; Lyme serology, liver function test, and coagulation studies were within normal limits. Serum, anti-streptolysin O titre, C3, and C4 were within normal limits as well as serum angiotensin-converting enzyme and aldosterone levels. Serum renin
0735-6757/© 2015 Elsevier Inc. All rights reserved.
Please cite this article as: Viteri B, et al, Facial nerve palsy in a 3-year-old child with severe hypertension—a case report, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.04.057
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B. Viteri et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx
Fig. 1. Computed tomographic angiogram of abdomen, coronal view, showing smaller left kidney (arrow).
Fig. 2. Computed tomographic angiogram of abdomen, sagittal view, showing narrowed caliber of left renal artery (arrow) and smaller left kidney.
level was significantly increased 33 ng/mL per hour (0.25-5.82). Urine homovanillic acid concentration was within normal limits; there was an upward trend in serial vanillylmandelic acid urine concentrations with time (8.7 to 9.3 to 15.4 mg/g creatinine). Serum thyroidstimulating hormone and T4 and lipid profile were within normal limits; serum cortisol level in the morning was 2.5 μg/dL. Genetic studies were unremarkable. Over the next 7 days, his HTN persisted; despite initiating multiple oral antihypertensive drugs, including oral enalapril, labetalol, and amlodipine, he could not be weaned from IV antihypertensive medication. At that point, it was decided to transfer the patient to another facility that had the capability for vascular intervention to treat pediatric renal artery stenosis. During hospitalization, the patient's facial paralysis gradually improved and was much less apparent at the time of transfer. At the transfer facility, a renal artery angiogram confirmed left renal artery narrowing without discrete focal stenosis. Angioplasty was performed but was unsuccessful in resolving his HTN. A renal artery stent was then placed; despite this, the patient required triple antihypertensive therapy of amlodipine, labetalol, and clonidine to control his HTN. Eventually, his facial palsy completely resolved approximately 1 month after presentation. Acquired facial nerve palsy (FNP) is a relatively uncommon pediatric condition, affecting approximately 5 to 10 per 100000 children per year [1]. Although sometimes associated with an underlying infectious, traumatic, malignant, or HTN etiology [2], the most common cause of acute unilateral facial weakness is idiopathic Bell's palsy (75% of cases) [3]. Although the diagnosis of Bell's palsy depends on exclusion of other etiologies, acute-onset FNP is often readily diagnosed and treated as idiopathic without an extensive workup when a child presents with isolated unilateral facial weakness. There is a paucity of medical literature characterizing the interesting clinical association of FNP with HTN in children. Most are single case reports [4-20]. To our knowledge, only 1 comprehensive published series exists, which reviewed 26 published cases of FNP associated with malignant HTN reported over a 50-year span (23 cases involved children) [20]. In all, HTN was associated with underlying renal pathology in 10 pediatric cases (most commonly an associated congenital anomaly), a cardiovascular lesion in 4 cases, and endocrinopathy in 1 case. Interestingly, 3 cases occurred in conjunction with Guillain-Barré syndrome, presumably due to either autonomic instability or hyperreninemia [21]. Many of these cases did not have neuroimaging performed to assess for associated neurologic lesion. Of those with renal disease, only 2 cases involved children with renal artery stenosis (RAS). More than 90% of patients had complete or nearly complete resolution of the FNP within the range of a few days to up to 12 months. Our case involves a young child with new-onset FNP due to underlying RAS-induced HTN. The RAS was likely due to fibromuscular dysplasia. Renal artery stenosis causes reduced renal arteriolar perfusion pressure, with activation of the renin-angiotensin-aldosterone system. The HTN was discovered serendipitously via routine vital sign measurement at ED triage. Because of young patient age, BP had not been routinely measured either a few days earlier at initial ED visit or at follow-up with the pediatrician 2 days later. Blood pressure
Table 1 Common etiologies of pediatric hypertension by age Infants
Ages 1-6 y
Ages 7-12 y
Adolescents
Thrombosis of renal artery or vein Congenital renal anomaly Coarctation of the aorta Bronchopulmonary dysplasia
Renal artery stenosis Renal parenchymal diseasea Wilms tumor Neuroblastoma Coarctation of the aorta
Renal parenchymal diseasea Renovascular abnormalities Endocrinopathyb Essential HTN
Essential HTN Renal parenchymal diseasea Endocrinopathyb
a b
Considerations include glomerulonephritis, reflux nephropathy, and congenital anomalies. Considerations include hyperthyroidism, Cushing syndrome, pheochromocytoma, and hyperaldosteronism.
Please cite this article as: Viteri B, et al, Facial nerve palsy in a 3-year-old child with severe hypertension—a case report, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.04.057
B. Viteri et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx Table 2 Etiologies of pediatric facial nerve paralysis Idiopathic (Bell's palsy) Infectious (Lyme disease, herpes simplex virus, varicella zoster virus, and acute otitis media) Trauma Genetic syndromes Tumor/malignancy Malignant HTN
measurement is often omitted in young children unless specifically indicated. Based on this case, we recommend BP measurement in all patients with FNP; when elevated, it is imperative to also measure BP in all 4 limbs to assess for discrepancies possibly indicating coarctation of the aorta. The etiologic considerations of malignant (secondary) pediatric HTN is extensive and involves multiple organ systems, especially renal, cardiac, and endocrine (Table 1) [22,23]. In children with newly diagnosed malignant HTN, an underlying renal parenchymal lesion is most likely [23]; other age-specific causes include RAS, coarctation of the aorta, neuroblastoma, pheochromocytoma, and a variety of other conditions [22,23]. The etiology of FNP (Table 2) most commonly includes idiopathic Bell's palsy and a variety of infectious causes; HTN, although rare, is a consideration. Our case demonstrates etiologic overlap between the 2 conditions. The causal association between HTN and FNP is unclear. A proposed mechanism for facial weakness with malignant HTN involves vascular compromise of cranial nerve 7. Possibilities include facial nerve swelling associated with vascular engorgement; hemorrhage into the facial canal with nerve compression, an ischemic stroke affecting the postnuclear nerve fibers, or bleeding in the facial nerve nucleus. Our patient received a full range of advanced contrast-enhanced neuroimaging, including head and facial magnetic resonance imaging; none of which identified any such abnormality. Our patient presented with malignant HTN and focal neurologic deficit. This initially caused concern for possible HTN-induced intracranial pathology, which led to a preliminary classification of “HTN emergency.” The first step in evaluating patients with acute facial paralysis is to determine whether the lesion is central or peripheral. Physical examination revealed paresis of the forehead musculature on the affected side, which helped localize the lesion to peripheral cranial nerve 7. Despite this, in addition to instituting immediate therapeutic measures to lower the BP, a priority in initial diagnostic maneuvering included performing cranial CT imaging to rule out intracranial pathology. The recommended ED management of pediatric malignant HTN includes prompt administration of antihypertensive medication to effect peripheral vasodilation (Table 3) [24]. Intravenous antihypertensive agents are usually recommended as first-line management of HTN Table 3 Drugs commonly used in ED treatment of malignant HTN • Nifedipine, SL or PO, 0.25-0.5 mg/kg (maximum, 10 mg), which reduces blood pressure within 5-20 min, with maximum effects at 60-90 min. Because it is only available in oral form, its absorption/effects can be erratic, thus making it most commonly recommended for children with hypertensive urgency. • Labetalol, IV, 0.2-1 mg/kg per dose (maximum, 20 mg), which can lower blood pressure within 5-10 min after IV administration by blocking α- and β-receptors peripherally at a ratio of 1:3 to effect vasodilation. It can be given as a IV bolus infusion every 10 min or by continuous IV infusion and is not associated with reflex tachycardia or increased cardiac output. • Nicardipine, IV infusion, 1-3 μg/kg per minute, controls BP rapidly by producing arterial vasodilation, thus reducing peripheral vascular resistance. It can be administered as an IV infusion. Advantages include lack of decreased cardiac output and limited effect on chronotropic and dromotropic heart function. • Sodium nitroprusside, IV infusion, 0.5-10 μg/kg per minute, which has potent, rapidly achieved vasodilating effects on arterioles and venules within minutes of administration. It is IV infused and can be precisely titrated in cases of acute hypertensive crises.
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emergency to rapidly and reliably effect lowering of BP. Although our patient was initially classified as such due to malignant HTN with focal neurologic deficit, he appeared to be well and was otherwise asymptomatic, and neurologic examination was otherwise normal; thus, we considered the relationship between HTN and FNP to be an association, rather than causal per se. As such, our initial therapeutic maneuver involved administration of the oral antihypertensive nifedipine, after which the parenteral medication labetalol was used. The goal was to initially decrease the BP by approximately 20%, avoiding an excessively rapid or steep fall in BP, which can result in underperfusion of vital organs. Ancillary management aspects for patients with malignant HTN include arterial line placement to promote continuous BP monitoring and accurately assess progress with therapeutic intervention. As with most patients with HTN-induced FNP, nerve dysfunction was transient and returned to normal after controlling BP elevation. The ultimate treatment goal for patients with RAS-associated HTN is relief of increased renal artery intravascular resistance. Definitive measures include percutaneous transluminal angioplasty, with or without stent placement, and surgical bypass. Angioplasty with stent placement was unsuccessful in resolving our patient's HTN; he had to continue taking antihypertensive medication. There is an association between FNP and severe HTN in children. All children presenting with FNP require accurate BP measurement. Those with malignant HTN should receive prompt pharmacologic intervention to partially lower the BP and a thorough evaluation to identify the organ system responsible for BP elevation. In most instances, the FNP resolves completely after BP normalizes. Bernarda Viteri MD Nicholas Koch MD Heda Dapul MD William Bonadio MD ⁎ Department of Pediatrics, Maimonides Medical Center, Brooklyn, NY ⁎ Corresponding author E-mail address: [email protected] http://dx.doi.org/10.1016/j.ajem.2015.04.057 References [1] Rownlands S, Hooper R, Hughes R, Burney P. The epidemiology and treatment of Bell's palsy in the UK. Eur J Neurol 2002;9:63–7. [2] Lorch M, Teach SJ. Facial nerve palsy: etiology and approach to diagnosis and treatment. Pediatr Emerg Care 2010;26(10):763–9. [3] Gilden DH. Clinical practice. Bell's palsy. N Engl J Med 2004;351(13):1323–31. [4] Bag O, Karaarslan U, Acar S, Unalp A. Alternating facial paralysis in a girl with hypertension. Case report. Arch Argent Pediatr 2013;111:133–6. [5] Agarwal R, Manandhar L, Saluja P, Grandi B. Pontine stroke presenting as isolated facial nerve palsy mimicking Bell's palsy: a case report. J Med Case Rep 2011;5:287–9. [6] Aynaci FM, Sen Y. Peripheral facial paralysis as initial manifestation of hypertension in a child. Turk J Pediatr 2002;44:73–5. [7] Bialestock D. Hyperplasia of the adrenal medulla in hypertension of children. Arch Dis Child 1961;36:465–73. [8] Harms M, Rotteveel J, Kar N, Gabreels F. Recurrent alternating facial paralysis and malignant hypertension. Neuropediatrics 2000;31:318–20. [9] Laufer J, Passwell J, Keren G, Cohen B. Raised plasma renin activity in the hypertension of the Guillain-Barre syndrome. Br Med J 1981;282:1272–3. [10] Lewis VE, Peat DS, Tizard EJ. Hypertension and facial palsy in middle aortic syndrome. Arch Dis Child 2001;85:240–1. [11] Lloyd AV, Jewitt DE, Still JD. Facial paralysis in children with hypertension. Arch Dis Child 1966;41:292–4. [12] Margabanthu G, Brooks J, Barron D, Miller P. Facial palsy as a presenting feature of coarctation of aorta. Interact Cardiovasc Thorac Surg 2003;2:91–3. [13] Moore P, Fiddler GI. Facial palsy in an infant with coarctation of the aorta and hypertension. Arch Dis Child 1980;55:315–6. [14] Siegler RL, Brewer ED, Corneli HM, Thompson J. Hypertension first seen as facial paralysis: case reports and review of the literature. Pediatrics 1991;87:387–9. [15] Smilari P, Incorpora C, Polizzi A, Sciacca P. Recurrent facial paralysis in a child with renovascular hypertension. Pediatr Med Chir 1995;17:461–3. [16] Smith N, Grattan-Smith P, Andrews IP, Kainer G. Acquired facial palsy with hypertension secondary to Guillain-Barre syndrome. J Paediatr Child Health 2010;46:125–7. [17] Tirodker UH, Dabbagh S. Facial paralysis in childhood hypertension. J Paediatr Child Health 2001;37:193–4. [18] Voorhees RL, Zeitzer LD, Ross M. Hypertension and associated peripheral facial paralysis. Laryngoscope 1972;82:899–902.
Please cite this article as: Viteri B, et al, Facial nerve palsy in a 3-year-old child with severe hypertension—a case report, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.04.057
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[19] Zeis P, Rao S, John E, Aschinberg L. “Stress” polycythaemia and peripheral facial palsy complications of severe hypertension. Acta Paediatr Scand 1979;68:287–9. [20] Jorg R, Milani G, Simonetti G, Bianchetti M, Simonetti B. Peripheral facial nerve palsy in severe systemic hypertension: a systematic review. Am J Hypertens 2013;26:561–5. [21] Stapleton F, Skoglund R, Daggett R. Hypertension associated with the Guillain-Barré syndrome. Pediatrics 1978;62:588–90. [22] Gavrilovici C, Boiculese L, Brumariu O, Dimitriou A. Etiology and blood pressure patterns in secondary hypertension in children. Rev Med Chir Soc Med Nat Iasi 2007; 111:70–81.
[23] Kapur G, Ahmed M, Pan C, Mitsnefis M, Chiang M, Matoo T. Secondary hypertension in overweight and stage 1 hypertensive children: a Midwest Pediatric Nephrology Consortium report. J Clin Hypertens 2010;12:34–9. [24] National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents. The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 2004; 114:555–76. Adour KK, Wingerd J. Idiopathic facial paralysis (Bell's palsy): factors affecting severity and outcome in 446 patients. Neurology 2004;24:1112–6.
Please cite this article as: Viteri B, et al, Facial nerve palsy in a 3-year-old child with severe hypertension—a case report, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.04.057
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Facial nerve palsy in a 3-year-old child with severe hypertension—a case report Abstract We report an interesting case of a child with new-onset malignant hypertension (HTN) associated with facial paralysis. A review of the medical literature on this association and discussion of diagnostic and management aspects are included. A 3-year-old Pakistani boy was transferred to the emergency department (ED) from his pediatrician's office due to concern for facial weakness. He had presented to the ED 2 days prior with a chief complaint relayed by his father of “swollen face; we see it more when he talks,” which occurred several days after rhinorrhea and congestion. At that time, he was afebrile and well appearing (there was no blood pressure [BP] measurement); there was noted mild right facial swelling without erythema, tenderness, fluctuance, or rash. Cranial nerve function was intact. He was diagnosed with right facial swelling (due to probable allergic reaction) and discharged home with pediatrician follow-up. Two days later, there was no improvement. He was evaluated by his pediatrician, who noticed unilateral facial muscle weakness and referred him to the ED. On second ED visit, parents denied patient had fever, rash, pain/headache, visual deficit, eye redness/ discharge, ear pain, or alteration in behavior/level of activity/mental status. Perinatal history was unremarkable. Family history was positive for consanguinity (parents were cousins) but negative for HTN. Medical history was significant for thalassemia, eczema, and food allergies. Parents denied any recent travel, rashes, insect/tick bites, or known sick contacts affecting the patient. Immunizations were up to date for age. The patient was not taking any current medications. On physical examination, he was well appearing, active, smiling/playful, in no apparent distress. He had mildly dysmorphic features with triangular facies, frontal prominence, low set ears, anteverted nostrils, and micrognathia. Developmentally, his body mass index was below the third percentile. He had right-sided upper and lower facial paralysis, specifically with weakness affecting the right corner of his mouth, right eyelid, and right forehead musculature. His vision was grossly normal, with intact extraocular movements and reactive pupils. With the exception of cranial nerve 7, cranial nerves 2 to 12 were intact. He had normal strength and sensation in all 4 extremities; deep tendon reflexes were 3+ and equal bilaterally. Head ears eyes nose throat examinations were within normal limits; specifically, ophthalmologic examination was without retinal abnormality or papilledema, and there was no evidence of acute otitis media. His neck was supple with no visualized or palpable masses. Cardiac examination was normal, lungs were clear to auscultation, and abdomen was soft and nontender with no masses or bruit. Blood pressure measured at triage was 224/112; heart rate, 99 beats per minute; respiratory rate, 24 breaths per minute; peripheral oxygen
saturation, 98% (in room air); rectal temperature, 97.6°F. His BP was measured 5 times over the next hour with readings of 194/110, 205/124, 195/ 124, 195/96, and 187/111. Blood pressure was measured in all 4 extremities, and there was no discrepancy in hypertensive readings. The initial differential diagnosis included hypertensive emergency; central nervous system (CNS) pathology (stroke, central nervous system tumor, and encephalitis); malignant HTN due to cardiac, endocrine, or renal disease; and Bell's palsy. The patient was placed on a cardiac monitor, and peripheral venous access was obtained. The initial priority was to therapeutically lower the BP by 10% to 20% while under ED supervision, before leaving the department to perform radiographic imaging. He initially received a dose of oral nifedipine 6 mg (0.5 mg/kg), with little change in BP. He next received intravenous (IV) labetalol 20 mg (1 mg/kg), with decrease in BP to 153/87. Upon BP reduction, he was transported to radiology for a cranial/facial computed tomography (CT). The CT scan was normal. Upon return to the ED, his BP was 177/121; he was given another dose of oral nifedipine 6 mg (0.5 mg/kg), after which his BP declined to 147/88. He was admitted to pediatric intensive care unit. Emergency department workup was remarkable for urinalysis showing trace protein and moderate hemoglobin. Basic serum metabolic panel showed sodium 139 mmol/L, potassium 3.9 mmol/L, chloride 101 mmol/L, bicarbonate 24 mmol/L, blood urea nitrogen 9 mg/dL, creatinine 0.2 mg/dL, and glucose 132 mg/dL. Complete blood count showed white blood cell 10000/mm3, hemoglobin 13.9 gm/dL, hematocrit 43%, and platelet count 251000/UL. Venous blood gas showed pH 7.42, PaCO2 38 mm Hg, PaO2 60 mm Hg, and lactate 2.2 mmol/dL. A chest x-ray showed clear lungs and normal heart size and contour. Upon admission to pediatric intensive care unit, the patient had persistent marked elevation of BP despite receiving multiple IV doses of labetalol. An arterial line placed for continuous BP monitoring, and a nicardipine drip (1 μg/kg per minute) was initiated. Abdominal sonogram showed a relatively small left kidney (5.7 cm) compared to the right kidney (8.2 cm) with normal renal echogenicity; there was no hydronephrosis, mass, or stone. A CT angiogram of the abdomen with IV contrast was performed, which showed asymmetric left renal atrophy (Fig. 1) and decreased caliber of the left renal artery compared to the right (Fig. 2), although no focal stenotic segment was identified. During hospitalization, he underwent cardiac, infectious, rheumatologic, nephrogenic, endocrinologic, and genetic workup. Echocardiogram showed moderate left ventricle hypertrophy but no evidence of coarctation of the aorta. Infectious workup showed no growth in blood, urine, and throat cultures; Lyme serology, liver function test, and coagulation studies were within normal limits. Serum, anti-streptolysin O titre, C3, and C4 were within normal limits as well as serum angiotensin-converting enzyme and aldosterone levels. Serum renin
0735-6757/© 2015 Elsevier Inc. All rights reserved.
Please cite this article as: Viteri B, et al, Facial nerve palsy in a 3-year-old child with severe hypertension—a case report, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.04.057
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B. Viteri et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx
Fig. 1. Computed tomographic angiogram of abdomen, coronal view, showing smaller left kidney (arrow).
Fig. 2. Computed tomographic angiogram of abdomen, sagittal view, showing narrowed caliber of left renal artery (arrow) and smaller left kidney.
level was significantly increased 33 ng/mL per hour (0.25-5.82). Urine homovanillic acid concentration was within normal limits; there was an upward trend in serial vanillylmandelic acid urine concentrations with time (8.7 to 9.3 to 15.4 mg/g creatinine). Serum thyroidstimulating hormone and T4 and lipid profile were within normal limits; serum cortisol level in the morning was 2.5 μg/dL. Genetic studies were unremarkable. Over the next 7 days, his HTN persisted; despite initiating multiple oral antihypertensive drugs, including oral enalapril, labetalol, and amlodipine, he could not be weaned from IV antihypertensive medication. At that point, it was decided to transfer the patient to another facility that had the capability for vascular intervention to treat pediatric renal artery stenosis. During hospitalization, the patient's facial paralysis gradually improved and was much less apparent at the time of transfer. At the transfer facility, a renal artery angiogram confirmed left renal artery narrowing without discrete focal stenosis. Angioplasty was performed but was unsuccessful in resolving his HTN. A renal artery stent was then placed; despite this, the patient required triple antihypertensive therapy of amlodipine, labetalol, and clonidine to control his HTN. Eventually, his facial palsy completely resolved approximately 1 month after presentation. Acquired facial nerve palsy (FNP) is a relatively uncommon pediatric condition, affecting approximately 5 to 10 per 100000 children per year [1]. Although sometimes associated with an underlying infectious, traumatic, malignant, or HTN etiology [2], the most common cause of acute unilateral facial weakness is idiopathic Bell's palsy (75% of cases) [3]. Although the diagnosis of Bell's palsy depends on exclusion of other etiologies, acute-onset FNP is often readily diagnosed and treated as idiopathic without an extensive workup when a child presents with isolated unilateral facial weakness. There is a paucity of medical literature characterizing the interesting clinical association of FNP with HTN in children. Most are single case reports [4-20]. To our knowledge, only 1 comprehensive published series exists, which reviewed 26 published cases of FNP associated with malignant HTN reported over a 50-year span (23 cases involved children) [20]. In all, HTN was associated with underlying renal pathology in 10 pediatric cases (most commonly an associated congenital anomaly), a cardiovascular lesion in 4 cases, and endocrinopathy in 1 case. Interestingly, 3 cases occurred in conjunction with Guillain-Barré syndrome, presumably due to either autonomic instability or hyperreninemia [21]. Many of these cases did not have neuroimaging performed to assess for associated neurologic lesion. Of those with renal disease, only 2 cases involved children with renal artery stenosis (RAS). More than 90% of patients had complete or nearly complete resolution of the FNP within the range of a few days to up to 12 months. Our case involves a young child with new-onset FNP due to underlying RAS-induced HTN. The RAS was likely due to fibromuscular dysplasia. Renal artery stenosis causes reduced renal arteriolar perfusion pressure, with activation of the renin-angiotensin-aldosterone system. The HTN was discovered serendipitously via routine vital sign measurement at ED triage. Because of young patient age, BP had not been routinely measured either a few days earlier at initial ED visit or at follow-up with the pediatrician 2 days later. Blood pressure
Table 1 Common etiologies of pediatric hypertension by age Infants
Ages 1-6 y
Ages 7-12 y
Adolescents
Thrombosis of renal artery or vein Congenital renal anomaly Coarctation of the aorta Bronchopulmonary dysplasia
Renal artery stenosis Renal parenchymal diseasea Wilms tumor Neuroblastoma Coarctation of the aorta
Renal parenchymal diseasea Renovascular abnormalities Endocrinopathyb Essential HTN
Essential HTN Renal parenchymal diseasea Endocrinopathyb
a b
Considerations include glomerulonephritis, reflux nephropathy, and congenital anomalies. Considerations include hyperthyroidism, Cushing syndrome, pheochromocytoma, and hyperaldosteronism.
Please cite this article as: Viteri B, et al, Facial nerve palsy in a 3-year-old child with severe hypertension—a case report, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.04.057
B. Viteri et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx Table 2 Etiologies of pediatric facial nerve paralysis Idiopathic (Bell's palsy) Infectious (Lyme disease, herpes simplex virus, varicella zoster virus, and acute otitis media) Trauma Genetic syndromes Tumor/malignancy Malignant HTN
measurement is often omitted in young children unless specifically indicated. Based on this case, we recommend BP measurement in all patients with FNP; when elevated, it is imperative to also measure BP in all 4 limbs to assess for discrepancies possibly indicating coarctation of the aorta. The etiologic considerations of malignant (secondary) pediatric HTN is extensive and involves multiple organ systems, especially renal, cardiac, and endocrine (Table 1) [22,23]. In children with newly diagnosed malignant HTN, an underlying renal parenchymal lesion is most likely [23]; other age-specific causes include RAS, coarctation of the aorta, neuroblastoma, pheochromocytoma, and a variety of other conditions [22,23]. The etiology of FNP (Table 2) most commonly includes idiopathic Bell's palsy and a variety of infectious causes; HTN, although rare, is a consideration. Our case demonstrates etiologic overlap between the 2 conditions. The causal association between HTN and FNP is unclear. A proposed mechanism for facial weakness with malignant HTN involves vascular compromise of cranial nerve 7. Possibilities include facial nerve swelling associated with vascular engorgement; hemorrhage into the facial canal with nerve compression, an ischemic stroke affecting the postnuclear nerve fibers, or bleeding in the facial nerve nucleus. Our patient received a full range of advanced contrast-enhanced neuroimaging, including head and facial magnetic resonance imaging; none of which identified any such abnormality. Our patient presented with malignant HTN and focal neurologic deficit. This initially caused concern for possible HTN-induced intracranial pathology, which led to a preliminary classification of “HTN emergency.” The first step in evaluating patients with acute facial paralysis is to determine whether the lesion is central or peripheral. Physical examination revealed paresis of the forehead musculature on the affected side, which helped localize the lesion to peripheral cranial nerve 7. Despite this, in addition to instituting immediate therapeutic measures to lower the BP, a priority in initial diagnostic maneuvering included performing cranial CT imaging to rule out intracranial pathology. The recommended ED management of pediatric malignant HTN includes prompt administration of antihypertensive medication to effect peripheral vasodilation (Table 3) [24]. Intravenous antihypertensive agents are usually recommended as first-line management of HTN Table 3 Drugs commonly used in ED treatment of malignant HTN • Nifedipine, SL or PO, 0.25-0.5 mg/kg (maximum, 10 mg), which reduces blood pressure within 5-20 min, with maximum effects at 60-90 min. Because it is only available in oral form, its absorption/effects can be erratic, thus making it most commonly recommended for children with hypertensive urgency. • Labetalol, IV, 0.2-1 mg/kg per dose (maximum, 20 mg), which can lower blood pressure within 5-10 min after IV administration by blocking α- and β-receptors peripherally at a ratio of 1:3 to effect vasodilation. It can be given as a IV bolus infusion every 10 min or by continuous IV infusion and is not associated with reflex tachycardia or increased cardiac output. • Nicardipine, IV infusion, 1-3 μg/kg per minute, controls BP rapidly by producing arterial vasodilation, thus reducing peripheral vascular resistance. It can be administered as an IV infusion. Advantages include lack of decreased cardiac output and limited effect on chronotropic and dromotropic heart function. • Sodium nitroprusside, IV infusion, 0.5-10 μg/kg per minute, which has potent, rapidly achieved vasodilating effects on arterioles and venules within minutes of administration. It is IV infused and can be precisely titrated in cases of acute hypertensive crises.
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emergency to rapidly and reliably effect lowering of BP. Although our patient was initially classified as such due to malignant HTN with focal neurologic deficit, he appeared to be well and was otherwise asymptomatic, and neurologic examination was otherwise normal; thus, we considered the relationship between HTN and FNP to be an association, rather than causal per se. As such, our initial therapeutic maneuver involved administration of the oral antihypertensive nifedipine, after which the parenteral medication labetalol was used. The goal was to initially decrease the BP by approximately 20%, avoiding an excessively rapid or steep fall in BP, which can result in underperfusion of vital organs. Ancillary management aspects for patients with malignant HTN include arterial line placement to promote continuous BP monitoring and accurately assess progress with therapeutic intervention. As with most patients with HTN-induced FNP, nerve dysfunction was transient and returned to normal after controlling BP elevation. The ultimate treatment goal for patients with RAS-associated HTN is relief of increased renal artery intravascular resistance. Definitive measures include percutaneous transluminal angioplasty, with or without stent placement, and surgical bypass. Angioplasty with stent placement was unsuccessful in resolving our patient's HTN; he had to continue taking antihypertensive medication. There is an association between FNP and severe HTN in children. All children presenting with FNP require accurate BP measurement. Those with malignant HTN should receive prompt pharmacologic intervention to partially lower the BP and a thorough evaluation to identify the organ system responsible for BP elevation. In most instances, the FNP resolves completely after BP normalizes. Bernarda Viteri MD Nicholas Koch MD Heda Dapul MD William Bonadio MD ⁎ Department of Pediatrics, Maimonides Medical Center, Brooklyn, NY ⁎ Corresponding author E-mail address: [email protected] http://dx.doi.org/10.1016/j.ajem.2015.04.057 References [1] Rownlands S, Hooper R, Hughes R, Burney P. The epidemiology and treatment of Bell's palsy in the UK. Eur J Neurol 2002;9:63–7. [2] Lorch M, Teach SJ. Facial nerve palsy: etiology and approach to diagnosis and treatment. Pediatr Emerg Care 2010;26(10):763–9. [3] Gilden DH. Clinical practice. Bell's palsy. N Engl J Med 2004;351(13):1323–31. [4] Bag O, Karaarslan U, Acar S, Unalp A. Alternating facial paralysis in a girl with hypertension. Case report. Arch Argent Pediatr 2013;111:133–6. [5] Agarwal R, Manandhar L, Saluja P, Grandi B. Pontine stroke presenting as isolated facial nerve palsy mimicking Bell's palsy: a case report. J Med Case Rep 2011;5:287–9. [6] Aynaci FM, Sen Y. Peripheral facial paralysis as initial manifestation of hypertension in a child. Turk J Pediatr 2002;44:73–5. [7] Bialestock D. Hyperplasia of the adrenal medulla in hypertension of children. Arch Dis Child 1961;36:465–73. [8] Harms M, Rotteveel J, Kar N, Gabreels F. Recurrent alternating facial paralysis and malignant hypertension. Neuropediatrics 2000;31:318–20. [9] Laufer J, Passwell J, Keren G, Cohen B. Raised plasma renin activity in the hypertension of the Guillain-Barre syndrome. Br Med J 1981;282:1272–3. [10] Lewis VE, Peat DS, Tizard EJ. Hypertension and facial palsy in middle aortic syndrome. Arch Dis Child 2001;85:240–1. [11] Lloyd AV, Jewitt DE, Still JD. Facial paralysis in children with hypertension. Arch Dis Child 1966;41:292–4. [12] Margabanthu G, Brooks J, Barron D, Miller P. Facial palsy as a presenting feature of coarctation of aorta. Interact Cardiovasc Thorac Surg 2003;2:91–3. [13] Moore P, Fiddler GI. Facial palsy in an infant with coarctation of the aorta and hypertension. Arch Dis Child 1980;55:315–6. [14] Siegler RL, Brewer ED, Corneli HM, Thompson J. Hypertension first seen as facial paralysis: case reports and review of the literature. Pediatrics 1991;87:387–9. [15] Smilari P, Incorpora C, Polizzi A, Sciacca P. Recurrent facial paralysis in a child with renovascular hypertension. Pediatr Med Chir 1995;17:461–3. [16] Smith N, Grattan-Smith P, Andrews IP, Kainer G. Acquired facial palsy with hypertension secondary to Guillain-Barre syndrome. J Paediatr Child Health 2010;46:125–7. [17] Tirodker UH, Dabbagh S. Facial paralysis in childhood hypertension. J Paediatr Child Health 2001;37:193–4. [18] Voorhees RL, Zeitzer LD, Ross M. Hypertension and associated peripheral facial paralysis. Laryngoscope 1972;82:899–902.
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