key: cord-0008718-3481pk8v authors: Khuri, F.J.; Alton, D.J.; Hardy, B.E.; Cook, G.T.; Churchill, B.M. title: Adrenal Hemorrhage in Neonates: Report of 5 Cases and Review of the Literature date: 2017-06-30 journal: J Urol DOI: 10.1016/s0022-5347(17)55609-8 sha: 53f0021d8a080242e20969756add2e4325ff118a doc_id: 8718 cord_uid: 3481pk8v The differential diagnosis of masses in the suprarenal area in neonates is discussed in relation to clinical, laboratory and radiologic findings. Neonatal adrenal hemorrhage can be accurately diagnosed clinically. In the neonate neuroblastoma in situ is self-limiting and exploration to exclude it is unnecessary. Operative intervention should be reserved for controlling massive adrenal hemorrhage or if an abscess forms Adrenal hemorrhage in the newborn is not rare. 1 The first report of massive adrenal hemorrhage diagnosed during life was by Corcoran and Strauss in 1924 and an operation was performed because of intractable bleeding. 2 Many cases have been reported since then, revealing a varied clinical picture and resulting in controversy over the treatment of choice. Some authors recommend surgical evacuation or excision, 3 -7 whereas others cite the reliability of clinical and radiological features to justify conservative management. 8 -12 A review of the medical records at this institution between 1965 and 1977 revealed 7 cases of suprarenal hemorrhage in the newborn. Two cases were diagnosed retrospectively by calcification in the region of the adrenals. The 5 cases diagnosed clinically are presented in tables l and 2, and the details of case 5 are presented herein. CASE REPORT Case 5. A male newborn was referred to us when he was 5 days old. An excretory urogram (IVP), which was done when he was 2 days old because of a left upper abdominal mass, had shown a non-functioning left kidney. Relevant laboratory data included serum bilirubin 10 mg./dl. (decreasing rapidly to 5 mg./dl.), urinary vanillyl mandelic acid 0.3 mg./24 hours, urinary catecholamines 7 µg./24 hours, and hemoglobin 15. 7 gm./ dl. An IVP when the patient was 6 days old showed a functioning left kidney, with the upper pole displaced laterally by a radiolucent suprarenal mass ( fig. 1) , which a renal scan with 99 mtechnetium showed to be avascular ( fig. 2, A) . Computerized axial tomography of the abdomen revealed the mass to be cystic, which was confirmed by ultrasonography ( fig. 2, B) . The newborn was treated conservatively. A followup IVP 2 months later revealed the left renal axis to be normal. No mass was palpable then or at 4-month followup. At 9 months an IVP was normal but calcification was absent. The pathogenesis of adrenal hemorrhage of the newborn is not known. Associated factors include birth trauma owing to difficult labor or delivery, 13 ' 14 asphyxia, 1 ' 13 septicemia, hemor-Accepted for publication January 18, 1980. Read at annual meeting of Northeastern Section, American Urological Association, Montreal, Canada, September 23-26, 1979. * Current address: Rochester General Hospital, Professional Bldg., Suite 304, 1445 Portland Ave., Rochester,_ New York 14621. 684 rhagic disorders 13 and hypoprothrombinemia. 1 ' 14 The hemorrhage is reported to affect the right adrenal 3 to 4 times more commonly than the left adrenal 1 and it is bilateral in 8 to 10 per cent of the cases. The usual explanation for susceptibility of the right adrenal is its greater likelihood of compression between the liver and spine and, because the right adrenal vein usually drains directly into the inferior vena cava, its proneness to changes in venous pressure. 13 Of our patients hemorrhage occurred on the right side in 2, the left side in 2 and both sides in 1 (table 1) . Birth weights were normal (range 3.49 to 4.63 kg.). No evidence of trauma was present at birth. Four newborns were vertex presentations and 1 had been delivered by cesarean section (indicated by a previous cesarean section). All were born at full term and only 2 had respiratory distress or cyanosis at birth. None had fever, evidence of septicemia, a significant coagulation defect or bloodgroup incompatibility. In all cases the hemorrhage occurred before 1 week of age (table 1) . Originally, clinical manifestations have been classified as owing to either adrenal insufficiency or acute hemorrhage. 13 Most recent reports indicate that the patient presents with prolonged neonatal jaundice and/or an abdominal mass with a decreasing hemoglobin. 9 Adrenal insufficiency is rare and we could find no proved case in our review of the English literature. In our patients the main clinical features were flank mass in all, prolonged jaundice in 4 and mild anemia in 3 (table 1). All had normal urinary excretion of vanillyl mandelic acid and catecholamines, and none had evidence of adrenal insufficiency. The radiologic findings have been described repeatedly. 8' 9 • 11 • 15 The early IVP reveals a smooth, homogeneously radiolucent mass displacing the kidney laterally and inferiorly, and indenting or flattening the superior pole. Non-visualization of the kidney on the side of the adrenal hemorrhage has been reported. 7 • 16 ' 17 In our last 2 cases the early IVP revealed nonfunction of the kidney on the involved side but the next IVP and initial films in the other 3 cases showed the characteristic deviation of the renal axis and lateral displacement of its upper pole by a radiolucent suprarenal mass ( fig. 1 ). The cystic nature of the mass was documented by an operation in case 1 (table 2) , arteriography in cases 2 to 4 ( fig. 3 ) and ultrasound, 9 9mtechnetium renal scan and computerized tomography in case 5 ( fig. 2) . The first patient, who had bilateral hemorrhage, underwent ,,milateral adrenalectomy and the subsequent 4 patients were managed conservatively (table 2). Shrinkage of the mass was Lt. Lt. on the left side and in many cases a normal 19 In l case of infected adrenal hem, to re,se,cn.on of the mass, m the belief that the mass was 1uam1su.,1.1J1c. 20 .tu.cuuu,~;u 1n:te<;t1,on of was not apparent in our The di.fferential u1a,:,1n,o,,1ec, area in the newborn include adrenal u~auw,.ua;;t, neu:roblastoma, Vl/ilms tumor and renal u,,i-,u1.::,n.1vu. of the upper segmenL This last condition can be excluded or retrograde or masses can be renal scan and/ or tumor and neuroblastoma can be excluded readily. There is no adequate explanation for the initial non-visualization of a kidney in a newborn with adrenal hemorrhage, which has been reported by others and occurred in our last 2 cases, but it should not be interpreted as renal invasion by a tumor. Determination of the 24-hour urinary excretion of vanillyl mandelic acid, homovanillic acid and catecholamines is relevant, since an increase in these substances, particularly vanillyl . mandelic acid, is virtually diagnostic of neuroblastoma. In all 'our patients (in some cases twice) normality of these levels was established before adrenal hemorrhage was diagnosed. Thus, the only remaining entity to be excluded is neuroblastoma in situ or hemorrhage into a neuroblastoma. Farber considers the latter a mechanism of spontaneous remission of this malignant tumor. 22 Sober and Hirsch described a case of neuroblastoma in a hemorrhagic adrenal gland in a neonate, which probably represented neuroblastoma in situ. 18 Other cases of neuroblastoma in situ in neonatal adrenal cysts have been reported. 23 The significance of neuroblastoma in situ in the newborn is questionable. Beckwith and Perrin, who estimated its incidence in their necropsy material to be about 40 times greater than expected from reported figures for clinically manifest neuro- blastoma, postulated that many of these microscopic tumors degenerated or differentiated to normal tissue. 24 This theory gains support from the report by Guin and associates of findings at necropsy of infants <6 months old, who had died of unrelated problems. 25 In patients ~3 months old the incidence of neuroblastoma in situ was 258 times the frequency expected according to rates for the clinically manifest tumor. In infants 3 to 6 months old, however, they found no such tumors. Therefore, Guin and associates postulated a time-growth relationship in the natural history of neuroblastoma in situ, illustrating the potential for spontaneous resolution. We conclude that the condition can be diagnosed accurately by history, physical examination, laboratory data and radiographic imaging. An immediate operation to exclude neuroblastoma in situ is not justifiable. By 6 to 8 weeks shrinkage of the mass will be apparent clinically and this, together with straightening of the renal axis, will be visible radiographically. The adrenal will contain calcified deposits by 1 year. Thus, an operation should only be done if the hemorrhage is uncontrollable or to drain the abscess if infection of the hematoma occurs. This protocol of conservative management applies only for newborns in the first month of life. Thereafter, adrenal hemorrhage may represent necrosis of a malignant tumor or bleed- ing into it, warranting surgical exploration. Craig and associates reported adrenocortical carcinoma in a IO-month-old child in whom the radiological findings were similar to those of an adrenal hematoma. 26 Hemorrhage and subsequent calcification of the suprarenal Suprarenal hemorrhage in the newborn Neonatal adrenal hemorrhage Large asymptomatic adrenal haematomas in the neonate Perinatal haemorrhagic necrosis of the adrenal gland. A clinical and radiological evaluation of 24 consecutive cases Haematoma of the adrenal gland in newborns presenting as an asymptomatic abdominal mass Massive adrenal hemorrhage in the newborn: 2 surviving cases by surgical treatment The clinical and radiographic features of massive neonatal adrenal hemorrhage Prolonged jaundice as presenting sign of massive adrenal hemorrhage in newborn. Radiographic diagnosis by IVP with total-body opacification Natural history of adrenal hemorrhage in the newborn Angiography and the conservative management of neonatal adrenal hemorrhage Massive adrenal hemorrhage in the newborn Syndrome of adrenal hemorrhage in the newborn Hematoma of adrenal gland in newborn The radiologic features of acute massive adrenal hemorrhage of the newborn Adrenal hematoma simulating tumor in a newborn Diagnosis and treatment of neonatal adrenal hemorrhage Unilateral massive adrenal hemorrhage in newborn infant Perirenal hemorrhage in newborn. Urology Adrenal abscess in a neonate Adrenal cyst. A case report and a review of the pediatric literature In situ neuroblastoma associated with an adrenal cyst In situ neuroblastomas: a contribution to the natural history of neural crest tumors Incidental neuroblastoma in infants Selective adrenal venography in an infant with virilizing adrenal cortical carcinoma The diagnosis of idiopathic adrenal hemorrhage in the newborn is suspected when a mass, jaundice and anemia are associated with inferior dislocation of the kidney on an IVP or renal scan. Ultrasonography, often showing a mixed lucent pattern, is confirmatory.We have seen 2 patients with simultaneous idiopathic adrenal hemorrhage and renal vein thrombosis. One had ipsilateral and the other contralateral involvement, with non-visualization of the involved kidney on the IVP. This may be the explanation for the non-visualization in 2 patients presented. The presence of hematuria would lead one to suspect renal vein thrombosis and a decreased platelet count or other evidence of consumptive coagulopathy strongly supports this presumption. The treatment remains the same-that is fluid support-without the necessity for surgical intervention.These authors review another example of the role of multi-modality evaluation in the child with an abdominal mass. Exploratory surgery becomes less and less common as we learn to base our diagnostic studies in an organized manner, using the tools available to our radiologic associates. With knowledge of the natural history of various problems and an accurate diagnosis patients can be managed non-operatively with confidence. Does multicystic renal disease (cystic dysplasia) fall into a similar category?A. Barry Belman Department of Pediatric Urology Children's Hospital National Medical Center Washington, D. C.