Iran J Pediatr Case Report Mar 2008; Vol 18 ( No 1), Pp:67-70

Hydrometrocolpos Due to Persistent Urogenital Sinus Mimicking Neonatal Ascites

Rizwan Ahmad Khan*1, Senior Resident, Pediatric Surgery; Imran Ghani1, MCh, Professor, Pediatric Surgery; Shagufta Wahab2, Senior Resident, Radiology

1. Division of Pediatric Surgery, Department. of Surgery, JNMCH, Aligarh, India 2. Department of Radiology, JNMCH, Aligarh, India

Received: 12/07/07; Revised: 17/12/07; Accepted: 26/12/07

Abstract Objective: A urogenital sinus is a common channel into which both the urethra and genital tracts open. Occasionally there might be transverse obstructing membrane leading to hydrometrocolpos from either uterine secretions or urine. Although the presentation is varied, a neonatal presentation of a large hydrometrocolpos mimicking gross ascites is rare. Case Presentation: A 3‐day‐old female infant was referred to us from periphery as a case of gross ascites of unknown cause. Patient was having rapid and shallow breathing and had gross ascites. The patient was evaluated and was diagnosed to have large hydrometrocolpos. Conclusion: To avoid unnecessary delay in diagnosis and proper treatment the possibility of hydrometrocolpos secondary to urogenital sinus presenting as generalized abdominal distension should be kept in mind. A focused genital examination can help achieve the diagnosis.

Key Words: Hydrometrocolpos, Urogenital sinus, Neonatal ascites

Introduction specialties. Urogenital sinus is an important, Hydrometrocolpos is cystic dilatation of the but often missed, cause for hydrometro‐ and due to congenital vaginal colpos[1]. Large hydrometrocolpos can present obstruction. It may be secretory or urinary in as tense ascites and therefore attending character and manifests in the neonatal period clinician must be inclining to such a possibility with abdominal distension. Congenital thereby preventing the morbidity associated obstructing lesions of vagina leading to with delays in diagnosis, including the rapid hydrometrocolpos, and , present progression of symptoms as in our case. We at a variable time during early childhood and present an unusual presentation of a patient of adolescence to different medical and surgical hydrometrocolpos.

* Correspondence author; Address: 4/817-F, Sir Syed Nagar Mallah Nagla Road, Aligarh, UP, INDIA 202002 E-mail: [email protected] 68 Hydrometrocolpos Mimicking as Neonatal Ascites; RA Khan, et al

Case Presentation possible origin from pelvis (Fig 1). Based on clinical and CT findings patient was taken up A 3‐day‐old female neonate was born at term for exploratory laparotomy. Her operative gestation by vaginal delivery with no history findings were a hugely distended vagina with of birth difficulty. 1‐min Apgar score was 8. uterus lying over it. Rest of the abdominal Antenatal history did not reveal exposure to viscera was normal. Tube vaginostomy was teratogen, infection or diabetes. There was no done. It drained about 200 ml of brownish family history of similar disorder. At birth fluid. In the postoperative period patient patient had gross abdominal distension but progressed well and vaginostomy drain was there were no features suggestive of bowel removed on 25th postoperative day. obstruction. Urethral catheterization was not Thereafter the patient was in follow‐up and successful. patient remained stable. Patient has been Abdominal X‐ray and ultrasonography done planned for definitive repair in future. outside revealed findings of ground glass appearance and gross ascites respectively. At presentation the baby was well hydrated and her cry, tone and activity were fairly good. Per Discussion abdomen examination revealed generalized distension with some prominent veins. Fluid The common urogenital sinus is a normal thrill was present and bowel sounds were stage of embryonic development in both sexes. heard well above in both hypogastric regions. The persistent urogenital sinus with common Perineal examination revealed a single channel for the urinary and genital tracts in opening in the introitus region and a normally female represents developmental arrest after placed anal opening. Patient was investigated the paramesonephric duct has fused with the and her laboratory investigation revealed urogenital sinus at about 9 weeks of gestation white cell count to be 14.5x103/mm3 and before the urogenital septum has migrated to hemoglobin was 17 g%. Patient was subjected divide the urinary and genital tracts[2]. to CECT abdomen. A long urogenital sinus with a short vagina It revealed a large hypodense lesion and a high urethral opening results when the posterior to urinary bladder and anterior to defect occurs at an early stage. A short rectum occupying whole of abdomen with a urogenital sinus with almost normal length

Fig1­ CECT of the patient showing large hypodense lesion occupying whole of abdomen with ring enhancement

Iran J Pediatr, Vol 18 (No 1); Mar 2008 69

vaginal vestibule and low urethral opening hamartoblastoma of the hypothalamic results when the defect occurs late in the region[12]. Rarely, symptoms of hydrometro‐ development[2]. Common presentations in colpos may manifest antenatally. It should be neonates can be asymptomatic, abdominal considered systematically when an ascites, mass, neonatal sepsis, and respiratory multicystic pelvic mass, bilateral hydro‐ distress. It may also present as hydro‐ nephrosis, abdominopelvic cystic mass is nephrosis[3] or peritoneal calcification[4]. The diagnosed in a female fetus. Because the abdominal lump is due to the distended vagina prognosis and neonatal management of (hydrocolpos) or vagina and uterus isolated hydrocolpos with spontaneous (hydrometrocolpos). Vaginal and cervical resolution differs greatly from that of epithelium secretes mucus in response to hydrocolpos associated with a cloacal maternal estrogens. This pools in the malformation, it is important to ascertain obstructed vagina. The distended vagina may prenatally whether there are associated press on the bladder outflow causing anomalies. Antenatally presence of a fluid‐ obstructive uropathy with hydrouretero‐ debris level inside the cystic anechoic mass nephrosis or urinary infection. must be considered a crucial finding. Multiple There are other causes of hydrometro‐ echoes are due to vaginal secretions. This colpos which need to be ruled out. finding must alert the treating clinician to is uncommon, occurring in presence of the anomaly before hand[13]. 0.1 % of newborn females[5]. It presents If a patient presents with such a gross usually in the adolescent period with primary abdominal distension due to urogenital sinus , cyclic abdominal pain or then initially laparotomy is indicated with the hematocolpos. Less often it may present in aim to achieve the vaginal drainage and neonates or infants with an abdominal lump relieve the symptoms. Recently laparoscopy and an interlabial bulging membrane[6] or has come up as the option for diagnostic and causing severe renal compromise and therapeutic purposes but the optimal timing of abdominal ascites.[7] The true incidence of the procedure still needs to be established.[14] these anomalies is unknown but is reported Subsequently patient can be taken up for total between 0.1 and 3.8%[2]. Neonatal urinary urogenital mobilization via a posterior sagittal ascites caused by bladder perforation is approach for repairing short and long rare[8,9]. common channels. If the urogenital sinus is The diagnostic approach is based on good low, with a short common channel, a simple U‐ clinical evaluation especially thorough flap will be effective[10]. If the perineal examination and adequately defining vagina enters the urogenital sinus too high up, the anatomy with the help of CT scan and a division of vaginal moiety from the sinus will retrograde dye study[10]. Endoscopy is be required together with pull through particularly helpful in defining the length of vaginoplasty. urethra especially in patients with long Some patients with a long common channel common channel. The high vagina usually may require additional maneuvers after drains well via a urogenital sinus and rarely mobilization to complete the reconstruction pools sufficient urine to be a source of for example construction creation of infection or interfere with normal bladder neourethra from anterior vaginal wall.[10] A emptying[11]. good surgical correction should provide an Hydrocolpos and hydrometrocolpos may be adequate vaginal opening into the perineum, associated with other malformations, such as create a normal‐looking vaginal introitus, fully postaxial polydactyly, anal atresia, esophageal separate the urethral from the vaginal orifice, atresia, renal agenesis, genital anomalies, and remove phallic erectile tissue while preserving cardiopathy. Each neonate presenting with glandular enervation and blood supply, and hydrocolpos should be evaluated for other preventing urinary tract complications[15]. clinically silent malformations, such as 70 Hydrometrocolpos Mimicking as Neonatal Ascites; RA Khan, et al

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