Journal of Perinatology (2007) 27, 130–132 r 2007 Nature Publishing Group All rights reserved. 0743-8346/07 $30 www.nature.com/jp PERINATAL/NEONATAL CASE PRESENTATION Neonatal adrenal hemorrhage mimicking an acute scrotum

O Adorisio1, R Mattei2, E Ciardini2, N Centonze2 and B Noccioli2 1Department of Pediatric Surgery, Pediatric Urology Unit, Children’s Hospital ‘Anna Meyer’, Florence, Italy and 2Neonatal Surgery Unit, Children’s Hospital ‘Anna Meyer’, Florence, Italy

abdomen revealed a complex mass (3.6 Â 3.1 Â 2.6 cm) on the Twenty-two cases of scrotal hematoma caused by neonatal adrenal upper pole of the right kidney with anechoic zones in its contest. hemorrhage are reported in the literature and unnecessary surgical No peritoneal or retroperitoneal fluid collection was identifiable. exploration was performed in nine (41%), suspecting testicular torsion. In Magnetic resonance imaging (MRI) of the abdomen was performed this paper, we present a newborn male with right adrenal gland showing a suprarenal right mass, without enhancement and/or hemorrhage causing right scrotal swelling and discoloration of groin calcifications (Figure 2). All laboratory tests, including 24-h managed conservatively. urinary catecholamine were normal. Diagnosis of scrotal Journal of Perinatology (2007) 27, 130–132. doi:10.1038/sj.jp.7211638 hematoma caused by adrenal hemorrhage was made and Keywords: adrenal hemorrhage; scrotal hematoma; acute scrotum conservative management was the treatment chosen. Serial ultrasound examinations showed significant reduction of the fluid collection of both scrotum and adrenal gland with complete Introduction recovery after 1 month. At present, the patient is 6 months old with Scrotal swelling in newborn may result from different causes normal testes and adrenal glands. including hydrocele, testicular trauma with testicular edema, inguinal hernia, congenital tumors and meconium peritonitis. Acute and subacute torsion of the spermatic cord should also be Discussion considered. Although rare, neonatal adrenal hemorrhage (NAH) Scrotal swelling in newborns is unusual and may result from presenting as scrotal swelling can mimick the condition of acute different causes including hydrocele, testicular trauma with scrotum and result in surgical exploration. Differential diagnosis testicular edema, inguinal hernia, congenital tumors and between NAH and other causes of scrotal swelling in newborn is meconium peritonitis. Acute and subacute torsion of the spermatic mandatory to avoid unnecessary surgery. cord should also be considered. Although rarely NAH presents as scrotal swelling, mimicking a condition of acute scrotum and resulting in misdiagnosis,1–4 the differential diagnosis between this Case report A 3700 g male was born after 40 weeks’ gestation to a 32-year-old woman after an uneventful pregnancy. A difficult vaginal delivery, carried out without instrumental assistance, led to a fracture of the infant’s right clavicle. Apgar score was 9 and 10, respectively, at 1 and 5 min. Scrotal swelling and discoloration of groin were noted 48 h after birth and the infant was transferred to our hospital. Physical examination showed an enlarged and discolored right hemiscrotum. No abdominal mass was detected by physical examination. Ultrasonography revealed an edematous scrotum with marked thickening of subcutaneous tissue. The testes were normal, including Doppler examination. Fluid collection in the right hemiscrotum was seen (Figure 1). Ultrasound study of the

Correspondence: Dr O Adorisio, Department of Pediatric Surgery, Pediatric Urology Unit, Children’s Hospital ‘Anna Meyer’, via Luca Giordano 13, 50132 Florence, Italy. E-mail: [email protected] Figure 1 Ultrasonography showing hematic fluid collection in the Received 11 May 2006; revised 12 October 2006; accepted 9 November 2006 right side of the scrotum. Neonatal adrenal hemorrhage O Adorisio et al 131

surgical exploration to improve testicular salvage rates in newborn.13,14 Testicular torsion in the neonatal period may be either antenatal or perinatal. The exact etiology of intrauterine torsion is unknown. Depending on when in pregnancy the torsion occurs, the presentation can range from edematous and fixed scrotal skin with an indurated scrotal mass, to a marble-like testis without any associated scrotal skin changes, to a vanished testes.14 Testicular torsion in the perinatal period is unusual. Possible causes include abnormal development of the gubernaculum, violent contraction of the cremaster, non-attachment of the tunica testis and high birth weight.15 Physical examination reveals firm, erythematous swollen testicles that do not transmit light. Ultrasound study can assist in the diagnosis of testicular torsion. Antenatal and postnatal sonographic finding include an enlarged testis and epididymis surrounded by hemorrhagic fluid organized in two concentric compartments (double ring hemorrhage image).16 In the diagnosis of testicular torsion, ultrasound has been reported to have a specificity of 100%, a sensitivity of 69.2%, a positive predictive value of 100% and a negative predictive value of 97.5%. Lam et al.17 reported an incidence of 1.2% false-negative Doppler flow studies in patients presenting with clinical signs of torsion. Extra caution must be exercised in interpreting blood flow Figure 2 MRI showing adrenal hemorrhage. findings in neonatal and infantile testes because of their naturally low-flow state. An extratesticular signal in these cases may appear condition and other causes of scrotal swelling in newborn is needed intratesicular.17 The technical key is to optimize color Doppler to avoid unnecessary surgical exploration.5,6 settings to detect low flow and to be certain that intratesticular flow NAH is uncommon with a reported incidence of 0.005 to 0.14% is detected and not the flow within extracapsular vessels, epididymis on autopsy series. Ten percent of the cases occur bilaterally.1 In the or surrounding structures.18 newborn, the adrenal gland is very large and vulnerable to vascular In our case, sonographic evaluation of the scrotum revealed an damage.2 NAH may be spontaneous or associated with perinatal edematous scrotum with marked thickening of the subcutaneous hypoxia or difficult or traumatic delivery.1,7 NAH usually occurs at tissue and a fluid collection inside without double ring hemorrhage birth or during the immediate postnatal period. As such, the image. The testes were normal, including Doppler examination. finding of an adrenal mass may lead to serious problems of Abdomen ultrasound evaluation showed the presence of a differential diagnosis as other serious pathological entities such as suprarenal mass that MRI of the abdomen confirmed to be an cystic neuroblastoma (CNB) must be considered. adrenal hemorrhage. Ultrasound evaluation of both testes and Ultrasound rarely distinguishes between NAH and other causes abdomen is necessary for differential diagnosis.10,11,19,20 of suprarenal mass and may suggest diagnosis of either NAH or Twenty-two cases of scrotal hematoma owing to NAH are CNB. Postnatal diagnosis of CNB may be obtained by indirect signs reported in the literature and unnecessary surgical exploration was such as collection of 24-h urine specimen to measure performed in nine cases (41%) (Table 1). In all the cases managed vanillymandelic and omovanillyc acid, and by radiological surgically, the decision to perform a surgical intervention was examinations such as TC or MRI.1 based only on the physical examination leading to the suspect of Clinical manifestations of NAH are anemia, abdominal mass, testicular torsion.8 jaundice and rarely scrotal swelling with skin discoloration.8–11 Surgical intervention showed a normal testis in all cases. Usually, if bleeding is moderate, blood remains in the adrenal Postoperative abdominal ultrasound revealed ipsilateral adrenal capsule. A large amount of blood can cause the rupture into the hemorrhage in all these babies. In the cases managed peritoneal cavity or retroperitoneal space. Blood reaches the conservatively, physician’s previous experiences suspecting NAH as scrotum through a patent processus vaginalis or by dissection of a cause of scrotal swelling avoided unnecessary surgical the tissue of the retroperitoneum.2,8,12 exploration.8,11,21 In other cases, ultrasound study of both the Several authors recommend considering an acute scrotal scrotum and abdomen performed before the surgery revealed a incident as a possible torsion of the testes and to provide promptly a fluid collection in the scrotum in association with an ipsilateral

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Table 1 NAH presenting as scrotal hematoma: cases reported 5 Avolio L, Fusillo M, Ferrari G, Chiara A, Braghieri R. Neonatal adrenal hemorrhage manifesting as acute scrotum: timely diagnosis prevents Author (year) Cases (n) Surgical Conservative unnecessary surgery. Urology 2002; 59: 601 viii–601 x. Putnam (1989) 1 1 0 6 Yang WT, Ku KW, Metreweli C. Case report. Neonatal adrenal haemorrhage Karpe and Nybonde (1989) 3 1 2 presenting as an acute right scrotal swelling (haematoma) – value of Giacoia and Cravens (1990) 1 1 0 ultrasound. Clin Radiol 1995; 50(2): 127–129. Liu et al. (1994) 2 1 1 7 Milele V, Patti G, Mazzoni G, Calisti A, Valenti M. Ultrasonography in the Thambi-Dorai et al. (1994) 1 1 0 diagnosis of neonatal adrenal gland hemorrhage. Radiol Med 1994; 87: Yang et al. (1995) 1 0 1 111–117. ¨ ¨ Miele et al. (1997) 3 1 2 8 Duman N, Oren H, Gu¨lcan H, Kunmral A, Olguner M, Ozkan H. Scrotal Anding et al. (2000) 3 1 2 hematoma due to neonatal adrenal hemorrhage. Pediatr Int 2004; 46: Miele et al. (2000) 1 0 1 360–362. Kirby and Davidson (2000) 1 0 1 9 Liu KW, Ku KW, Cheung KL, Chan YL. Acute scrotal swelling: a sign of Avolio et al. (2002) 2 1 1 neonatal adrenal haemorrhage. J. Pediatr Child Health 1994; 30(4): 368– O’Neill et al. (2003) 1 0 1 369. Duman et al. (2004) 1 1 0 10 Iban˜ez Godoy I, Mora Navarro D, Delgado Rioja MA, Losada Martinez A, Ibanez et al. (2004) 1 0 1 Herrera del Rey C. Unilateral scrotal hematoma. Ann Pediatr (Barc) 2004; 60: 477–478. Abbreviation: NAH, neonatal adrenal hemorrhage 11 Anding R, Fastnacht-Urban F, Walz PH. ‘Acute scrotum’ in the neonate. Adrenal haemorrhage as cause. Urologe A 2000; 39(1): 48–51. 12 Milele V, Galluzzo M, Patti G, Mazzoni G, Calisti A, Valenti M. Scrotal hematoma due to neonatal adrenal haemorrhage: the value of adrenal hemorrhage avoiding unnecessary surgical ultrasonography in avoiding unnecessary surgery. Pediatric Radiol 1997; 10,11 exploration. 27: 672–674. In conclusion, adrenal hemorrhage may present as scrotal 13 Das S, Singer A. Controversies of perinatal torsion of the spermatic cord: a swelling mimicking an acute scrotum. The differential diagnosis review, survey and recommendation. J Urol 1990; 143: 231–233. between this condition and other causes of scrotal swelling is based 14 Al-Salem AH. Intra-uterine testicular torsion: early diagnosis and treatment. on physical examination, clinical history and ultrasound BJU Int 1999; 83: 1023–1025. examination of both testes and abdomen. This association allows 15 Sakellaris G, Sifakis S, Hatzidaki E, Daskalojannaki M, Giannacopoulou C, conservative treatment avoiding unnecessary surgical exploration Charissis G. Early exploration in perinatal torsion of the spermatic cord: a of the scrotum. case report. J Matern Fetal Neonatal Med 2004; 15(3): 207–209. 16 Herman A, Schvimer M, Tovbin J, Sandbank J, Bukovski I, Strauss S et al. Antenatal sonographic diagnosis of testicular torsion. Ultrasound Obstet Gynecol 2002; 20: 522–524. 17 Lam WWC, Yap TL, Jacobsen AS, James H, Teo EL. Colour doppler References ultrasonography replacing surgical exploration for acute scrotum: myth or 1 O’Neill JMD, Hendry GMA, MacKinlay GA. An unusual presentation of reality? Pediatr Radiol 2005; 35: 587–600. neonatal adrenal hemorrhage. Eur J Ultrasound 2002; 16: 261–264. 18 Cartwright PC, Brent W, Reid BS, Shultz PK. Color doppler ultrasound in 2 Bergami G, Malena S, Di Mario M, Fariello G. Sonographic follow-up of newborn testis torsion. Urology 1995; 45: 667–670. neonatal adrenal hemorrhage. Fourteen case reports. Radiol Med 1990; 79: 19 Giacoia GP, Cravens JD. Neonatal adrenal hemorrhage presenting as scrotal 474–478. hematoma. J Urol 1990; 143: 567–568. 3 Putnam MH. Neonatal adrenal hemorrhage presenting as a right scrotal 20 Thambi-Dorai CR, Smith AJ, Dewan PA. Adrenal hemorrhage presenting as mass. JAMA 1989; 261: 2958. acute scrotal swelling in a neonate. J Pediatr Chlid Health 1994; 30: 72–73. 4 Karpe B, Nybonde T. Adrenal hemorrhage versus testicular torsion-a 21 Kirby CP, Davidson PM. Inguinoscrotal bruising: a sign of neonatal adrenal diagnostic dilemma in the neonate. Pediatr Surg Int 1989; 4: 337–340. hemorrhage. Pediatr Emerg Care 2000; 16: 179.

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