Coexistence of Renal Vein Thrombosis and Adrenal Hemorrhage in Two Newborns Patricia A. Veiga, M.D.* James E. Springate, M.D.* Alan S. Brody, M.D.** James J. Cummings, M.D. Luis Mosovich, M.D.* Leonard G. Feld, M.D., Ph.D.*
Introduction
of
delivery,
a
biophysical profile
abnormal and led to a prenatal ultrasound which showed poor fetal movement and a left flank mass. Elective cesarean section was performed and the amnionic fluid was noted to be meconiumstained. Initial physical examination of this 3.2-kg male infant revealed a 5 x 5-cm left flank mass. Because of gross hematuria and respiratory distress, the infant was transferred to the Children’s Hospital of Buffalo. Admission laboratory evaluation showed hemoglobin 12.6 g/dL was
enal vein thrombosis and adrenal hemorrhage are to occur simultain The clininewborns.’, neously be cal may management renal complicated by impairment, hypertension, and inferior vena cava thrombus. We present two cases of coexisting renal vein thrombosis and adrenal hemorrhage in newborns. Definitive diagnosis was determined using ultrasonography and radioisotope renal scanning. Although both cases were complicated by inferior vena cava thrombosis, we show patient survival and recovery with conservative management alone.
known ~~L
Case I The
patient was born at term following a pregnancy complicated by a high blood sugar level, as determined by a glucose tolerance test, and obesity. On the day From the Department of Pediatrics* and Department of Radiology,** Children’s Hospital of Buffalo, Buffalo, New York. Address correspondence to: Leonard G. Feld, M.D., Ph.D., Division of Pediatric Nephrology, Children’s Hospital of Buffalo, 219 Bryant Street, Buffalo, NY 14222.
174
(126 g/L), platelets 70,000/mm~ (70 x 10VL), and total bilirubin 8.11 mg/dL (138 ¡..tmol/L). Prothrombin time and partial thromboplastin time were normal. Urinalysis showed dark red urine with 2+ protein. An abdominal real-time ultrasound demonstrated the left kidney to be 6.2 cm in length (normal for age, 4.5 ± 0.3 cm) with increased echogenicity and no hydronephrosis. Superior to the left kidney was an irregularly shaped fluid collection measuring 2.0-3.0 cm (Figure 1). Repeat ultrasound examination on day two of life showed thrombi in the left renal vein and inferior vena cava. Doppler examination revealed no flow to the left kidney. The child recovered from transient tachypnea of the newborn.
Downloaded from cpj.sagepub.com at UNIV OF PITTSBURGH on January 17, 2015
Urine output was normal and gross hematuria cleared on day three. Blood pressures were normal. All blood and urine cultures showed no growth. Twenty-four-hour urinary vanillylmandelic acid excretion was normal. On day seven, a diethylenetriaminepentaacetic acid (DTPA) renal scan revealed a nonfunctioning left kidney. The right kidney showed poor excretion of the isotope. The large adrenal hemorrhage showed organization on repeat ultrasound examination. The infant was discharged when 10 days old. Repeat renal ultrasound at four weeks of age showed a calcified, resolving adrenal hemorrhage. Serum creatinine was 0.5 mg/dL (44 ymol/L) and urinalysis was normal. Sonogram at 11 months of age showed the right kidney to be 7.9 cm in size and the left kidney to be atrophic (normal size for age, 6.2 cm ± 0.6). The infant has continued to thrive with no evidence of
hypertension or renal insufficiency. Case 2
Following the mother’s normal pregnancy, this infant was born at term via vaginal delivery. The baby did well until three weeks of age, when he was brought to a local
emergency department with an eight- to 10- hour history of poor feeding, lethargy, and feeling cold to the touch. Upon transfer to our institu-
tion, the infant was ill-appearing a firm, left-sided abdominal mass. Vital signs showed temperature 36.6°C, pulse 156 beats/min, respiratory rate 60 breaths/min,
with
and blood pressure ’70/49 torr. Laboratory evaluation revealed hemoglobin 11.0 g/dL (110 g/L),
platelets 199,000/mm~ (199 x 109/L), serum sodium 129 mEq/L (129 mmol/L), potassium 8.0 mEq/L (8.0 mmol/L), BUN 88 mg/dL (31.4 mmol/L), and creatinine 3.3 mg/dL (290 p,mol/L). Urinalysis showed 4+ protein and large blood. Abdominal real-time ultrasound revealed bilateral adrenal hemorrhages. The left kidney was enlarged, with a clot noted in the left renal vein. Both kidneys showed increased echogenicity. Initial therapy consisted of volume expansion, bicarbonate therapy, and blood products. Urine output and blood pressure were normal. Blood culture yielded Escherichia coli. A computed tomography scan of the abdomen demonstrated thrombosis in the inferior vena cava to the level of the heart. A DTPA renal scan showed a non-functioning left kidney and a right kidney with poor excretion of isotope. The infant improved clinically with conservative management. Repeat abdominal ultrasound showed persistent inferior vena cava clot and no Doppler signals from the left renal vessels. An early-morning serum cortisol level was normal. Serum BUN and creatinine concentrations declined daily and were normal on hospital day 10. Followup ultrasound examination on hospital day 22 showed small liquefying adrenal hemorrhages and a shrinking left kidney. The infant was dis-
charged on hospital day 27. Follow-up has shown a thriving normotensive infant. Serum creatinine has been 0.6 mg/dL (53 ¡..tmol/L) . A DTPA renal scan showed a nonfunctioning left kidney and a right kidney with normal flow and excretion of isotope. Renal ultrasound at five months of age showed the left kidney to be atrophic, the right kidney to be 6.6 cm in size (normal, 6.1 ± 0.6 cm), and residual clot remaining in the inferior vena cava.
Discussion The pathogenesis of neonatal adrenal hemorrhage is unknown. Associated factors include birth trauma, asphyxia, sepsis, and coagulopathy.3 Symptoms vary in extent of the hemorrhage, with presenting signs including flank mass, jaundice, and anemia. Adrenal insufficiency is rare in infants, even in cases of bilateral adrenal
involvement.’4 In the past, the detection of adrenal hemorrhage was made by
the appearance of a radiolucent displacing the kidney laterally and inferiorly with flattening of the upper calyces on intravenous pyelogram.’ It is now recommended that ultrasound be the initial radiographic procedure. 1,5 An echolucent mass indicates central hemorrhage, although the mass can be cystic with solid compomass
nents.
The major differential diagnosis is neonatal neuroblastoma with hemorrhage. Serial sonograms showing resolution of the hemorrhage and normal urine catecholamine excretion will exclude this diagnosis.5 Typically, the hemorrhage resolves in eight to 10 days with shrinkage of the mass in six to eight weeks. Calcification in the gland can be noted as early as one week or as late as one year after diagnosis.’ Major complications of neonatal adrenal hemorrhage are uncontrollable hemorrhage, abscess formation, ipsilateral renal vein thrombosis, and hypertension due to ipsilateral renal impairment.3,4,6 In infancy renal vein thrombosis
Figure 1. Longitudinal sonogram shows the left kidney (K) and a portion of the adrenal hemorrhage (H). Arrows mark the posterior margin of the kidney. The kidney is enlarged and heterogeneous in its echogenicity, with loss of normal corticomedullary differentiation.
Downloaded from cpj.sagepub.com at UNIV OF PITTSBURGH on January 17, 2015
175
initially results from thrombosis in the interlobular veins of the cortex, with extension to larger veins. This can occur in states of hemo-
concentration, hypercoagulability, and
hypoperfusion, including perinatal asphyxia, sepsis, dehydration, or maternal diabetes.’’ Hematuria, often gross, is seen along with a flank mass, anemia, and
thrombocytopenia. Therapy is supportive and includes removal of inciting factors and treatment of acute
renal failure if present.
Controversy surrounds the use of systemic heparin therapy or thrombectomy, as survival and renal recovery can occur with supportive therapy alone.’ There is recent evidence thrombolytic therapy may be beneficial, but its use in neonates is limited.’ Because of the uncertain frequency of hypertension and renal impairment, longterm follow-up is required.10 Our two cases illustrate many of the associated factors and complications of renal vein thrombosis and adrenal hemorrhage in the newborn. Specifically, we noted intrauterine asphyxia with gesta-
176
tional diabetes and sepsis with hypoperfusion. Both infants developed extrarenal thrombosis and lost function of the involved kidney. Ultrasound and renal isotope scans proved valuable in the diagnosis and continued evaluation of these infants. Conservative therapy for inferior vena cava thrombosis resulted in infant survival without the need for exploratory surgery. Follow-up of our patients has shown normal growth, blood pressure, and renal function. These cases of coexisting renal vein thrombosis and adrenal hemorrhage in newborns demonstrate the value of ultrasonography, radioisotope imaging, and conservative therapy in patient management.
Acknowledgment
neous idiopathic adrenal hemorrhage and renal vein thrombosis in the new-
Urol. 1983;129:574-576. J born. AD, Smazal SF. Ultrasound of
2.
Bowen
3.
coexisting right renal vein thrombosis and adrenal hemorrhage in a newborn. J Clin Ultrasound. 1981;9:511-513. Khuri FJ, Alton DJ, Hardy BE, Cook GT, Churchill BM. Adrenal hemorrhage in neonates: report of five cases and review of the literature.
J
Urol
1980;124:684-687. 4.
5.
Black J, Williams DI. Natural history of adrenal hemorrhage in the newborn. Arch Dis Child. 1973;48:183-190. Lawson EE, Teele RL. Diagnosis of adrenal hemorrhage by ultrasound. J Pediatr.
6.
1978;92:423-426.
Starinski R, Manor A, Segal M. Nonfunctioning kidney associated with neonatal adrenal hemorrhage. Radiol. 1986;16:427-429.
Pediatr
7.
Oliver WJ, Kelsch RC. Renal vein thrombosis in infancy. Pediatr Rev.
8.
Ricci
1982;4:61-66. MA, Lloyd DA. Renal vein throm-
bosis in infants and children. Arch Surg.
We would like to acknowledge the secretarial assistance of Ms. Clara M. Shields.
1990;125:1195-1199. 9.
10.
Renal vein thrombosis in long-term follow-up. Pediatr
REFERENCES 1.
Lebowitz
JM,
Bromberg WD, Firlit CF. Fibrinolytic therapy for renal vein thrombosis in the J Urol. 1990; 143:86-88. child. Mocan H, Beattie TJ, Murphy AV
Belman AB. Simulta-
Downloaded from cpj.sagepub.com at UNIV OF PITTSBURGH on January 17, 2015
1991;5:45-49.
infancy:
Nephrol.
Introduction
of
delivery,
a
biophysical profile
abnormal and led to a prenatal ultrasound which showed poor fetal movement and a left flank mass. Elective cesarean section was performed and the amnionic fluid was noted to be meconiumstained. Initial physical examination of this 3.2-kg male infant revealed a 5 x 5-cm left flank mass. Because of gross hematuria and respiratory distress, the infant was transferred to the Children’s Hospital of Buffalo. Admission laboratory evaluation showed hemoglobin 12.6 g/dL was
enal vein thrombosis and adrenal hemorrhage are to occur simultain The clininewborns.’, neously be cal may management renal complicated by impairment, hypertension, and inferior vena cava thrombus. We present two cases of coexisting renal vein thrombosis and adrenal hemorrhage in newborns. Definitive diagnosis was determined using ultrasonography and radioisotope renal scanning. Although both cases were complicated by inferior vena cava thrombosis, we show patient survival and recovery with conservative management alone.
known ~~L
Case I The
patient was born at term following a pregnancy complicated by a high blood sugar level, as determined by a glucose tolerance test, and obesity. On the day From the Department of Pediatrics* and Department of Radiology,** Children’s Hospital of Buffalo, Buffalo, New York. Address correspondence to: Leonard G. Feld, M.D., Ph.D., Division of Pediatric Nephrology, Children’s Hospital of Buffalo, 219 Bryant Street, Buffalo, NY 14222.
174
(126 g/L), platelets 70,000/mm~ (70 x 10VL), and total bilirubin 8.11 mg/dL (138 ¡..tmol/L). Prothrombin time and partial thromboplastin time were normal. Urinalysis showed dark red urine with 2+ protein. An abdominal real-time ultrasound demonstrated the left kidney to be 6.2 cm in length (normal for age, 4.5 ± 0.3 cm) with increased echogenicity and no hydronephrosis. Superior to the left kidney was an irregularly shaped fluid collection measuring 2.0-3.0 cm (Figure 1). Repeat ultrasound examination on day two of life showed thrombi in the left renal vein and inferior vena cava. Doppler examination revealed no flow to the left kidney. The child recovered from transient tachypnea of the newborn.
Downloaded from cpj.sagepub.com at UNIV OF PITTSBURGH on January 17, 2015
Urine output was normal and gross hematuria cleared on day three. Blood pressures were normal. All blood and urine cultures showed no growth. Twenty-four-hour urinary vanillylmandelic acid excretion was normal. On day seven, a diethylenetriaminepentaacetic acid (DTPA) renal scan revealed a nonfunctioning left kidney. The right kidney showed poor excretion of the isotope. The large adrenal hemorrhage showed organization on repeat ultrasound examination. The infant was discharged when 10 days old. Repeat renal ultrasound at four weeks of age showed a calcified, resolving adrenal hemorrhage. Serum creatinine was 0.5 mg/dL (44 ymol/L) and urinalysis was normal. Sonogram at 11 months of age showed the right kidney to be 7.9 cm in size and the left kidney to be atrophic (normal size for age, 6.2 cm ± 0.6). The infant has continued to thrive with no evidence of
hypertension or renal insufficiency. Case 2
Following the mother’s normal pregnancy, this infant was born at term via vaginal delivery. The baby did well until three weeks of age, when he was brought to a local
emergency department with an eight- to 10- hour history of poor feeding, lethargy, and feeling cold to the touch. Upon transfer to our institu-
tion, the infant was ill-appearing a firm, left-sided abdominal mass. Vital signs showed temperature 36.6°C, pulse 156 beats/min, respiratory rate 60 breaths/min,
with
and blood pressure ’70/49 torr. Laboratory evaluation revealed hemoglobin 11.0 g/dL (110 g/L),
platelets 199,000/mm~ (199 x 109/L), serum sodium 129 mEq/L (129 mmol/L), potassium 8.0 mEq/L (8.0 mmol/L), BUN 88 mg/dL (31.4 mmol/L), and creatinine 3.3 mg/dL (290 p,mol/L). Urinalysis showed 4+ protein and large blood. Abdominal real-time ultrasound revealed bilateral adrenal hemorrhages. The left kidney was enlarged, with a clot noted in the left renal vein. Both kidneys showed increased echogenicity. Initial therapy consisted of volume expansion, bicarbonate therapy, and blood products. Urine output and blood pressure were normal. Blood culture yielded Escherichia coli. A computed tomography scan of the abdomen demonstrated thrombosis in the inferior vena cava to the level of the heart. A DTPA renal scan showed a non-functioning left kidney and a right kidney with poor excretion of isotope. The infant improved clinically with conservative management. Repeat abdominal ultrasound showed persistent inferior vena cava clot and no Doppler signals from the left renal vessels. An early-morning serum cortisol level was normal. Serum BUN and creatinine concentrations declined daily and were normal on hospital day 10. Followup ultrasound examination on hospital day 22 showed small liquefying adrenal hemorrhages and a shrinking left kidney. The infant was dis-
charged on hospital day 27. Follow-up has shown a thriving normotensive infant. Serum creatinine has been 0.6 mg/dL (53 ¡..tmol/L) . A DTPA renal scan showed a nonfunctioning left kidney and a right kidney with normal flow and excretion of isotope. Renal ultrasound at five months of age showed the left kidney to be atrophic, the right kidney to be 6.6 cm in size (normal, 6.1 ± 0.6 cm), and residual clot remaining in the inferior vena cava.
Discussion The pathogenesis of neonatal adrenal hemorrhage is unknown. Associated factors include birth trauma, asphyxia, sepsis, and coagulopathy.3 Symptoms vary in extent of the hemorrhage, with presenting signs including flank mass, jaundice, and anemia. Adrenal insufficiency is rare in infants, even in cases of bilateral adrenal
involvement.’4 In the past, the detection of adrenal hemorrhage was made by
the appearance of a radiolucent displacing the kidney laterally and inferiorly with flattening of the upper calyces on intravenous pyelogram.’ It is now recommended that ultrasound be the initial radiographic procedure. 1,5 An echolucent mass indicates central hemorrhage, although the mass can be cystic with solid compomass
nents.
The major differential diagnosis is neonatal neuroblastoma with hemorrhage. Serial sonograms showing resolution of the hemorrhage and normal urine catecholamine excretion will exclude this diagnosis.5 Typically, the hemorrhage resolves in eight to 10 days with shrinkage of the mass in six to eight weeks. Calcification in the gland can be noted as early as one week or as late as one year after diagnosis.’ Major complications of neonatal adrenal hemorrhage are uncontrollable hemorrhage, abscess formation, ipsilateral renal vein thrombosis, and hypertension due to ipsilateral renal impairment.3,4,6 In infancy renal vein thrombosis
Figure 1. Longitudinal sonogram shows the left kidney (K) and a portion of the adrenal hemorrhage (H). Arrows mark the posterior margin of the kidney. The kidney is enlarged and heterogeneous in its echogenicity, with loss of normal corticomedullary differentiation.
Downloaded from cpj.sagepub.com at UNIV OF PITTSBURGH on January 17, 2015
175
initially results from thrombosis in the interlobular veins of the cortex, with extension to larger veins. This can occur in states of hemo-
concentration, hypercoagulability, and
hypoperfusion, including perinatal asphyxia, sepsis, dehydration, or maternal diabetes.’’ Hematuria, often gross, is seen along with a flank mass, anemia, and
thrombocytopenia. Therapy is supportive and includes removal of inciting factors and treatment of acute
renal failure if present.
Controversy surrounds the use of systemic heparin therapy or thrombectomy, as survival and renal recovery can occur with supportive therapy alone.’ There is recent evidence thrombolytic therapy may be beneficial, but its use in neonates is limited.’ Because of the uncertain frequency of hypertension and renal impairment, longterm follow-up is required.10 Our two cases illustrate many of the associated factors and complications of renal vein thrombosis and adrenal hemorrhage in the newborn. Specifically, we noted intrauterine asphyxia with gesta-
176
tional diabetes and sepsis with hypoperfusion. Both infants developed extrarenal thrombosis and lost function of the involved kidney. Ultrasound and renal isotope scans proved valuable in the diagnosis and continued evaluation of these infants. Conservative therapy for inferior vena cava thrombosis resulted in infant survival without the need for exploratory surgery. Follow-up of our patients has shown normal growth, blood pressure, and renal function. These cases of coexisting renal vein thrombosis and adrenal hemorrhage in newborns demonstrate the value of ultrasonography, radioisotope imaging, and conservative therapy in patient management.
Acknowledgment
neous idiopathic adrenal hemorrhage and renal vein thrombosis in the new-
Urol. 1983;129:574-576. J born. AD, Smazal SF. Ultrasound of
2.
Bowen
3.
coexisting right renal vein thrombosis and adrenal hemorrhage in a newborn. J Clin Ultrasound. 1981;9:511-513. Khuri FJ, Alton DJ, Hardy BE, Cook GT, Churchill BM. Adrenal hemorrhage in neonates: report of five cases and review of the literature.
J
Urol
1980;124:684-687. 4.
5.
Black J, Williams DI. Natural history of adrenal hemorrhage in the newborn. Arch Dis Child. 1973;48:183-190. Lawson EE, Teele RL. Diagnosis of adrenal hemorrhage by ultrasound. J Pediatr.
6.
1978;92:423-426.
Starinski R, Manor A, Segal M. Nonfunctioning kidney associated with neonatal adrenal hemorrhage. Radiol. 1986;16:427-429.
Pediatr
7.
Oliver WJ, Kelsch RC. Renal vein thrombosis in infancy. Pediatr Rev.
8.
Ricci
1982;4:61-66. MA, Lloyd DA. Renal vein throm-
bosis in infants and children. Arch Surg.
We would like to acknowledge the secretarial assistance of Ms. Clara M. Shields.
1990;125:1195-1199. 9.
10.
Renal vein thrombosis in long-term follow-up. Pediatr
REFERENCES 1.
Lebowitz
JM,
Bromberg WD, Firlit CF. Fibrinolytic therapy for renal vein thrombosis in the J Urol. 1990; 143:86-88. child. Mocan H, Beattie TJ, Murphy AV
Belman AB. Simulta-
Downloaded from cpj.sagepub.com at UNIV OF PITTSBURGH on January 17, 2015
1991;5:45-49.
infancy:
Nephrol.
