75
Journal of Neonatal-Perinatal Medicine 7 (2014) 75–79 DOI 10.3233/NPM-1474413 IOS Press
Case Report
Urinary ascites due to persistent urogenital sinus: A case report and review of literature P. Loganathana,∗ , M. Kamaluddeena and A.S. Soraishama,b a Department b Alberta
of Pediatrics, Section of Neonatology, University of Calgary, AB, Canada Children’s Hospital Research Institute, University of Calgary, AB, Canada
Received 9 August 2013 Revised 1 December 2013 Accepted 8 January 2014
Abstract. BACKGROUND: Persistent urogenital sinus is one of the rare urogenital anomalies, which commonly presents as hydrometrocolpos. Fetal urinary ascites as a presentation of persistent urogenital sinus is extremely rare. CASE REPORT: We report on a preterm infant with antenatal diagnosis of hydrometrocolpos and massive urinary ascites secondary to urogenital sinus without any bladder or renal abnormalities. CONCLUSION: This case report emphasizes the importance of maintaining a high index of suspicion in the diagnosis of persistent urogenital sinus especially in infants presenting with urinary ascites along with hydrometrocolpos. Keywords: Ascites, hydrometrocolpos, preterm, urogenital sinus
1. Background
2. Case report
Persistent urogenital sinus (UGS) is one of the rare forms of urogenital anomaly, in which there is one common opening for the vagina and urethra due to failure of urethrovaginal separation. Persistent UGS commonly presents as hydrometrocolpos but its presentation as fetal ascites is extremely rare [1, 2]. We report a preterm infant with antenatal diagnosis of hydrometrocolpos and massive urinary ascites secondary to persistent UGS without any bladder or renal abnormalities.
A preterm (31 weeks) female infant weighing 2.2 kg (>90th centile) was born to a healthy 38-year-old Gravida 3, Para 1 mother with one previous still born infant of unknown etiology. Routine antenatal ultrasound at 19 weeks for fetal anomaly was grossly normal. However, fetal scan at 286/7 weeks showed marked fetal ascites with normal urinary bladder and a small cystic mass posterior to the bladder. There was mild polyhydramnios with no evidence of hydrops or pleural effusion. Fetal magnetic resonance imaging (MRI) at 296/7 weeks showed marked ascites causing elevation of diaphragm and compression of lung. There was a large fluid filled tubular structure posterior to bladder, suggestive of dilated vagina
∗ Corresponding
author: Dr. Prakash Loganathan, Foothills Medical Centre, Rm-C-211, 1403, 29th Street NW, Calgary, Alberta T2N 2T9, Canada. Tel.: +1 403 944 1087; Fax: +1 403 944 4892; E-mail: [email protected].
1934-5798/14/$27.50 © 2014 – IOS Press and the authors. All rights reserved
76
P. Loganathan et al. / Urogenital sinus presenting as ascites
Fig. 2. Pelvic ultrasound showing dilated vagina (B), cervix and uterus (C) and collapsed bladder (A).
Fig. 1. Fetal MRI showing ascites (A) and large fluid filled tubular structure posterior to bladder, suggestive of dilated vagina and uterus.
and uterus (Fig. 1). The bowel loops were of normal caliber. The bladder and kidneys were normal and there was no evidence of pleural effusion or hydrops. Amniocentesis revealed normal karyotype. At 301/7 weeks, massive fetal ascites and marked polyhydramnios warranted aspiration of 60 ml of ascitic fluid and 300 ml of amniotic fluid. Fetal ultrasound surveillance was performed regularly. At 31 weeks, cesarean section was performed in view of low biophysical profile along with variable fetal heart rate deceleration. Just prior to the delivery, fetal ascitic tap was performed under ultrasound guidance and 550 ml of fluid was drained. Ascitic fluid analysis was suggestive of urinary ascites with clear yellow fluid with total white blood cell 0.4 × 109 /L (50% neutrophils), specific gravity 1.013, pH 8.0, osmolality 286 mmol/kg, creatinine 58 mol/L, protein 13 g/L, and triglyceride
Journal of Neonatal-Perinatal Medicine 7 (2014) 75–79 DOI 10.3233/NPM-1474413 IOS Press
Case Report
Urinary ascites due to persistent urogenital sinus: A case report and review of literature P. Loganathana,∗ , M. Kamaluddeena and A.S. Soraishama,b a Department b Alberta
of Pediatrics, Section of Neonatology, University of Calgary, AB, Canada Children’s Hospital Research Institute, University of Calgary, AB, Canada
Received 9 August 2013 Revised 1 December 2013 Accepted 8 January 2014
Abstract. BACKGROUND: Persistent urogenital sinus is one of the rare urogenital anomalies, which commonly presents as hydrometrocolpos. Fetal urinary ascites as a presentation of persistent urogenital sinus is extremely rare. CASE REPORT: We report on a preterm infant with antenatal diagnosis of hydrometrocolpos and massive urinary ascites secondary to urogenital sinus without any bladder or renal abnormalities. CONCLUSION: This case report emphasizes the importance of maintaining a high index of suspicion in the diagnosis of persistent urogenital sinus especially in infants presenting with urinary ascites along with hydrometrocolpos. Keywords: Ascites, hydrometrocolpos, preterm, urogenital sinus
1. Background
2. Case report
Persistent urogenital sinus (UGS) is one of the rare forms of urogenital anomaly, in which there is one common opening for the vagina and urethra due to failure of urethrovaginal separation. Persistent UGS commonly presents as hydrometrocolpos but its presentation as fetal ascites is extremely rare [1, 2]. We report a preterm infant with antenatal diagnosis of hydrometrocolpos and massive urinary ascites secondary to persistent UGS without any bladder or renal abnormalities.
A preterm (31 weeks) female infant weighing 2.2 kg (>90th centile) was born to a healthy 38-year-old Gravida 3, Para 1 mother with one previous still born infant of unknown etiology. Routine antenatal ultrasound at 19 weeks for fetal anomaly was grossly normal. However, fetal scan at 286/7 weeks showed marked fetal ascites with normal urinary bladder and a small cystic mass posterior to the bladder. There was mild polyhydramnios with no evidence of hydrops or pleural effusion. Fetal magnetic resonance imaging (MRI) at 296/7 weeks showed marked ascites causing elevation of diaphragm and compression of lung. There was a large fluid filled tubular structure posterior to bladder, suggestive of dilated vagina
∗ Corresponding
author: Dr. Prakash Loganathan, Foothills Medical Centre, Rm-C-211, 1403, 29th Street NW, Calgary, Alberta T2N 2T9, Canada. Tel.: +1 403 944 1087; Fax: +1 403 944 4892; E-mail: [email protected].
1934-5798/14/$27.50 © 2014 – IOS Press and the authors. All rights reserved
76
P. Loganathan et al. / Urogenital sinus presenting as ascites
Fig. 2. Pelvic ultrasound showing dilated vagina (B), cervix and uterus (C) and collapsed bladder (A).
Fig. 1. Fetal MRI showing ascites (A) and large fluid filled tubular structure posterior to bladder, suggestive of dilated vagina and uterus.
and uterus (Fig. 1). The bowel loops were of normal caliber. The bladder and kidneys were normal and there was no evidence of pleural effusion or hydrops. Amniocentesis revealed normal karyotype. At 301/7 weeks, massive fetal ascites and marked polyhydramnios warranted aspiration of 60 ml of ascitic fluid and 300 ml of amniotic fluid. Fetal ultrasound surveillance was performed regularly. At 31 weeks, cesarean section was performed in view of low biophysical profile along with variable fetal heart rate deceleration. Just prior to the delivery, fetal ascitic tap was performed under ultrasound guidance and 550 ml of fluid was drained. Ascitic fluid analysis was suggestive of urinary ascites with clear yellow fluid with total white blood cell 0.4 × 109 /L (50% neutrophils), specific gravity 1.013, pH 8.0, osmolality 286 mmol/kg, creatinine 58 mol/L, protein 13 g/L, and triglyceride
