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Arch Pediatr Adolesc Med. 2001;155(3):416. doi:10.1001/archpedi.155.3.415.
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DENOUEMENT AND DISCUSSION: INFERIOR VENA CAVA AND RENAL VEIN THROMBOSIS IN A NEONATE

Figure 1. Computed tomographic scan of the abdomen demonstrating a markedly enlarged right kidney (thick arrow), calcification of the inferior vena cava (arrowhead), and a small, calcified left kidney (thin arrow).

Figure 2. Computed tomographic scan of the abdomen demonstrating calcifications in the left renal vein extending into the inferior vena cava (arrowhead).

All abdominal masses detected in the neonatal period require further investigation with ultrasonography.1 Most of these masses originate in the genitourinary tract,12 and ultrasonography should be performed to localize and differentiate between cystic and solid structures. The differential diagnosis of cystic abdominal masses includes hydronephrosis; multicystic-dysplastic kidney disease; intestinal duplication; hydrometrocolpos; and choledochal, ovarian, renal, and pancreatic cysts. The differential diagnosis of solid abdominal masses includes neuroblastoma, teratoma, and Wilms tumor. A solid flank mass of a neonate in the presence of hematuria, hypertension, renal failure, and thrombocytopenia suggests a diagnosis of renal vein thrombosis.

Two types of renal vein thrombosis are recognized. In the first, the thrombus begins intrarenally at the level of the arcuate and interlobular veins, successively extending into the larger renal veins and to the inferior vena cava.3 It is thought that this type is more frequent during infancy.45 However, Demirci et al6 presented a case of bilateral renal vein thrombosis in which the inferior vena cava was completely filled with thrombus, suggesting that the thrombus arose initially in the inferior vena cava with subsequent retrograde extension into the renal veins.

Clinically, renal vein thrombosis is characterized by the presence of a palpable flank mass associated with acute renal failure. Hematuria, proteinuria, and oliguria are usually present. Predisposing factors are dehydration, asphyxia, polycythemia, sepsis, shock, coagulopathies, and maternal diabetes.7 Ultrasound examination will show perivascular streaking with increased echogenicity of the renal parenchyma.8 Computed tomography will demonstrate persistent parenchymal opacification and enlargement of the affected kidney, dilation of the renal vein, and renal vein and/or inferior vena caval thrombus.9

Surgical management with removal of the affected kidney has historically been the treatment of choice,10 but it has been recognized more recently that with supportive therapy alone, the short-term prognosis of renal vein thrombosis occurring during infancy is good. However, the involved kidney progressively becomes atrophic resulting in a small, scarred kidney.1112 Treatment with systemic anticoagulation agents may result in a better long-term prognosis.1314 In our patient, treatment with low-molecular-weight heparin resulted in a return of normal renal function by age 4 months.

Wilson  DA Ultrasound screening for abdominal masses in the neonatal period. AJDC. 1982;136147- 151
Henderson  KCTorch  EM Differential diagnosis of abdominal masses in the neonate. Pediatr Clin North Am. 1977;24557- 578
Metreweli  CPearson  R Echographic diagnosis of neonatal renal venous thrombosis. Pediatr Radiol. 1984;14105- 108
Evans  DJSilverman  MBowley  NB Congenital hypertension due to unilateral renal vein thrombosis. Arch Dis Child. 1981;56306- 308
Patel  RBConnors  JJ In utero sonographic findings in fetal renal vein thrombosis with calcifications. J Ultrasound Med. 1988;7349- 352
Demirci  ASelcuk  MBYazicioglu  I Bilateral adrenal hemorrhage associated with bilateral renal vein and vena cava thrombosis. Pediatr Radiol. 1991;21130
Orazi  CFariello  GMalena  SSchingo  PFerro  FBagolan  P Renal vein thrombosis and adrenal hemorrhage in the newborn: ultrasound evaluation of 4 cases. J Clin Ultrasound. 1993;21163- 169
Lin  G JYang  PHWang  ML Neonatal renal vein thrombosis: a case report describing serial sonographic changes. Pediatr Nephrol. 1994;8589- 591
Gatewood  OMBFishman  EKBurrow  CRWalker  WGGoldman  SMSiegelman  SS Renal vein thrombosis in patients with nephrotic syndrome: CT diagnosis. Radiology. 1986;159117- 122
Touloukian  RJ Idiopathic venal caval thrombosis with renal infarction in the newborn infant: survival following nephrectomy. Surgery. 1969;65978- 983
Mocan  HBeattie  TJMurphy  AV Renal venous thrombosis in infancy: long-term follow-up. Pediatr Nephrol. 1991;545- 49
Ricci  MALloyd  DA Renal venous thrombosis in infants and children. Arch Surg. 1990;1251195- 1199
Markowitz  GSBrignol  FBurns  ERKoenigsberg  MFolkert  VW Renal vein thrombosis treated with thrombolytic therapy: case report and brief review. Am J Kidney Dis. 1995;25801- 806
Pohl  MZimmerhackl  LBHeinen  F  et al.  Bilateral renal vein thrombosis and venous sinus thrombosis in a neonate with factor V mutation (FV Leiden). J Pediatr. 1998;132159- 161

Accepted for publication February 12, 1999.

This report was sponsored by the Chief Bureau of Medicine and Surgery, Navy Department, Washington, DC, Clinical Investigation Program, report 84-16-1968-665, as required by NSHSBETHINST 6000.41A.

The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or the US Government.

Reprints: Clinical Research Department, Medical Editing Division, Naval Medical Center San Diego, 34800 Bob Wilson Dr, Suite 5, San Diego, CA 92134-1005 (e-mail: wmacal@snd10.med.navy.mil).

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References

Wilson  DA Ultrasound screening for abdominal masses in the neonatal period. AJDC. 1982;136147- 151
Henderson  KCTorch  EM Differential diagnosis of abdominal masses in the neonate. Pediatr Clin North Am. 1977;24557- 578
Metreweli  CPearson  R Echographic diagnosis of neonatal renal venous thrombosis. Pediatr Radiol. 1984;14105- 108
Evans  DJSilverman  MBowley  NB Congenital hypertension due to unilateral renal vein thrombosis. Arch Dis Child. 1981;56306- 308
Patel  RBConnors  JJ In utero sonographic findings in fetal renal vein thrombosis with calcifications. J Ultrasound Med. 1988;7349- 352
Demirci  ASelcuk  MBYazicioglu  I Bilateral adrenal hemorrhage associated with bilateral renal vein and vena cava thrombosis. Pediatr Radiol. 1991;21130
Orazi  CFariello  GMalena  SSchingo  PFerro  FBagolan  P Renal vein thrombosis and adrenal hemorrhage in the newborn: ultrasound evaluation of 4 cases. J Clin Ultrasound. 1993;21163- 169
Lin  G JYang  PHWang  ML Neonatal renal vein thrombosis: a case report describing serial sonographic changes. Pediatr Nephrol. 1994;8589- 591
Gatewood  OMBFishman  EKBurrow  CRWalker  WGGoldman  SMSiegelman  SS Renal vein thrombosis in patients with nephrotic syndrome: CT diagnosis. Radiology. 1986;159117- 122
Touloukian  RJ Idiopathic venal caval thrombosis with renal infarction in the newborn infant: survival following nephrectomy. Surgery. 1969;65978- 983
Mocan  HBeattie  TJMurphy  AV Renal venous thrombosis in infancy: long-term follow-up. Pediatr Nephrol. 1991;545- 49
Ricci  MALloyd  DA Renal venous thrombosis in infants and children. Arch Surg. 1990;1251195- 1199
Markowitz  GSBrignol  FBurns  ERKoenigsberg  MFolkert  VW Renal vein thrombosis treated with thrombolytic therapy: case report and brief review. Am J Kidney Dis. 1995;25801- 806
Pohl  MZimmerhackl  LBHeinen  F  et al.  Bilateral renal vein thrombosis and venous sinus thrombosis in a neonate with factor V mutation (FV Leiden). J Pediatr. 1998;132159- 161

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