Cardiovasc Intervent Radiol (2011) 34:S271–S276 DOI 10.1007/s00270-010-9814-1
CASE REPORT
Ultrasound-Guided Percutaneous Drainage of Neonatal Pyometrocolpos Under Local Anesthesia Oktay Algin • Cuneyt Erdogan • Nizamettin Kilic
Received: 17 November 2009 / Accepted: 8 January 2010 / Published online: 4 February 2010 Ó Springer Science+Business Media, LLC and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE) 2010
Abstract Hydrometrocolpos is an uncommon congenital disorder with cystic dilatation of the vagina and uterus that occurs as a result of accumulated secretions from the reproductive tract due to distal genital tract obstruction. Secondary infection may also occur, resulting in pyometrocolpos, a potentially lethal disease. Immediate drainage of the cystic mass in patients determined to have pyometrocolpos is required to prevent or treat uropathy and septicemia until definitive corrective surgery can be performed. We report an unusual cause of obstructive uropathy in three infants: pyometrocolpos due to lower genital tract atresia. Ultrasound-guided percutaneous drainage of the pyometrocolpos resulted in dramatically improved clinical and laboratory findings in these patients. Ultrasound-guided percutaneous drainage under local anesthesia is a simple, minimally invasive, safe, and effective procedure that facilitates later successful corrective surgery and avoids the need for more complex drainage procedures. Keywords Pyometrocolpos Interventional radiologym Ultrasound guided Percutaneous drainage Newborn Acute urinary retention Hydrometrocolpos
Introduction Hydrometrocolpos is a rare congenital disorder with cystic dilatation of the vagina and uterus that occurs as a result of accumulated secretions from the reproductive tract due to distal genital tract obstruction, or collected fetal urine that results from an obstructed cloacal common duct [1]. The true incidence of hydrometrocolpos is unknown but is reported to be 0.1–3.8% [2]. Secondary infection may also occur, resulting in pyometrocolpos [3]. Immediate drainage of the cystic mass in patients determined to have pyometrocolpos is required to prevent or treat uropathy and septicemia until definitive corrective surgery can be performed [3, 4]. For these infants, an ultrasound (US)-guided percutaneous drainage procedure that is minimally invasive, safe, and effective should be used [3, 5]. We present the use of US-guided percutaneous drainage under local anesthesia through the lower abdominal wall in three infants with pyometrocolpos who were acutely sick with obstructive uropathy and/or septicemia [3]. In addition, we discuss the pertinent literature of percutaneous drainage in pyometrocolpos.
Case Reports O. Algin (&) Department of Radiology, Atatu¨rk Training and Research Hospital, Bilkent, Ankara, Turkey e-mail:
[email protected] C. Erdogan Department of Radiology, Uludag University Medical Faculty, Gorukle, Bursa, Turkey N. Kilic Department of Pediatric Surgery, Uludag University Medical Faculty, Gorukle, Bursa, Turkey
Case 1 A 4-month-old girl weighing 2890 g born via normal vaginal delivery to a 37-year-old mother (gravida 2, para 2) was brought to the pediatric clinic of our hospital because of vomiting and inability to suck. The family history of the patient revealed nothing remarkable. Perineal examination of the infant showed a single orifice (urogenital sinus) and a normal anus. Radiographic contrast study was performed
123
S272
with a catheter, and a narrow vaginal cavity was revealed. Laboratory analysis showed increases in blood leukocyte, blood urea nitrogen (177 mg/dl), and creatinine (2.1 mg/dl) values. The patient had a temperature of 39°C. The infant was provided with prophylactic antibiotic therapy (cefotaxime, clindamycin, and amikacin). US and magnetic resonance imaging (MRI) examinations displayed bilateral hydronephrosis and a cystic lesion 62 9 60 9 96 mm in size in the posterior aspect of the bladder (Fig. 1). Because the US study on this cystic mass revealed internal echogenicities, it was evaluated as hydrometrocolpos or pyometrocolpos (Fig. 2). The patient demonstrated poor overall condition and was referred to the interventional radiology department of our hospital for immediate USguided drainage. The patient was closely monitored and sedated with rectally administered midazolam. The closest point to the cyst was determined with US, and the skin and subcutaneous structures at this level were anesthetized with 0.25% bupivacaine (1 mg/kg). By lower abdominal approach and under sterile conditions, an 18-gauge Seldinger needle was inserted into the cyst. A 10F nephrostomy catheter was placed into the cystic cavity via the Seldinger technique (Fig. 3). First 30 ml, then, within 8 h, 200 ml of purulent fluid was collected through the catheter. Bacterial growth was not observed in the aerobic and anaerobic cultures of the fluid. The patient was monitored for 2 weeks after the procedure. During the monitoring period, overall condition and laboratory parameters of the patient returned to normal, and bilateral hydronephrosis was regressed. Contrast agent was delivered through the drainage catheter under fluoroscopy; no passage of the contrast agent to the distal aspect of vagina was observed (Fig. 4). The patient was
O. Algin et al.: Ultrasound-Guided Percutaneous Drainage
Fig. 2 Abdominal ultrasound images of case 1. A cystic mass 62 9 60 9 96 mm in size with internal echogenicities is present in the pelvic region
Fig. 3 Ultrasound (US) examination performed 1 week after percutaneous US-guided drainage revealed marked decrease in the size of the cystic mass. The drainage catheter in the uterus can be observed (white arrow)
Fig. 1 Coronal and sagittal T2-weighted magnetic resonance images of a female infant (case 1). Bilateral hydronephrosis (A) and a cystic mass in the posterior-superior portion of the bladder (B, C)
123
diagnosed with vaginal atresia. Vaginoplasty was planned, and the patient was discharged with a recommendation for close clinical monitoring and, when required, temporal percutaneous drainage. When the patient was 8 months old, vaginoplasty was performed (uretero-vaginotomy) followed by vaginal dilatation in the next 2 months. At the patient’s last visit, when she was 1.5 years old, her overall health was well, and bilateral hydronephrosis was significantly regressed.
O. Algin et al.: Ultrasound-Guided Percutaneous Drainage
S273
Fig. 4 Fluoroscopic examination showing drainage catheter in the uterus (A). Contrast medium failing to pass into the vagina may be observed (B)
Case 2 A 6-month-old girl born via normal vaginal birth at term was brought to the pediatrics polyclinic of another hospital because of a swelling in the abdomen. US and MRI studies showed bilateral hydronephrosis and a cystic mass 87 9 55 9 85 mm in size extending to the posteriorsuperior portion of the bladder (Fig. 5). As a result of these findings, the patient was referred to our hospital. Physical examination of the patient in our hospital revealed a slowing of her movements and a tendency to sleep constantly. Laboratory studies provided no abnormal findings, other than an increased temperature (38°C). An US-guided drainage procedure was performed in the interventional radiology department of our hospital (with a
protocol similar to the one applied to case 1) (Fig. 6). First, the catheter produced 60 ml of purulent fluid. Contrast medium was delivered through the drainage catheter under fluoroscopy, and we observed no passage of contrast into the distal aspect of the vagina. The cervical canal was observed to be present as a thin space, and dilatation was detected in the proximal portion of the vagina. The culture of the fluid collected from the catheter grew Escherichia coli. The case was thus recognized as pyometrocolpos associated with cervical-proximal vaginal atresia. Antibiotic therapy was initiated. After 2 weeks of monitoring, the condition of the patient returned to normal. It was recommended that the patient undergo close clinical monitoring and, if needed, temporal percutaneous drainage. Corrective surgery was planned for treatment.
Fig. 5 Midline sagittal plane T2 (A), paramedian sagittal plane T2 (B), and paramedian sagittal plane T1 (C) weighted magnetic resonance images of case 2. All images display a cystic mass exhibiting a compression on the bladder and uterus. Right kidney shows hydronephrosis (white arrow, B), and right ureter exhibits dilatation (black arrow, C)
123
S274
O. Algin et al.: Ultrasound-Guided Percutaneous Drainage
Fig. 6 Ultrasound images of case 2 before and after placement of a drainage catheter. Hydronephrosis and a cystic mass extending toward the abdominal region are observed in the left kidney (A). After drainage of 60 ml of purulent fluid, minimal regression of the dilatation of the left kidney can be seen (B). The control ultrasound examination performed 1 week after the procedure reveals marked regression of the hydronephrosis (C). One week after the procedure, complete removal of endometrial purulent fluid and the sole presence of a catheter in the endometrial area can be seen (D)
Case 3 A 2-month-old girl delivered by caesarian section and with a birth weight of 2730 g (mother gravida 1, para 1) was brought to another hospital because of an inability to feed. The patient was diagnosed with type C tracheoesophageal fistula, annular pancreas, and persistent cloaca before being referred to our hospital. US examination of the patient showed minimal pelvocaliectasis in the left kidney. US and
Fig. 7 Scintigraphy images of case 3 (A, anterior-posterior view; B, oblique view). Right kidney is not observed on any of the images. A marked hydronephrosis was not detected in the left kidney
123
scintigraphic studies failed to display the right kidney (Fig. 7). The patient was treated with diamond-shape duodenostomy, primary tracheoesophageal fistula repair was applied, and colostomy was performed. The patient was well overall after surgery and was discharged with a recommendation that she be brought in for frequent clinical monitoring and check-up visits. Two months after discharge, the girl was brought to the pediatric surgery department with poor overall health, fever, abdominal distension, and respiratory distress. US revealed hydronephrosis of kidney and a cystic lesion of 8 9 6 9 5.5 cm in the posterior-superior portion of the bladder (Fig. 8). Right kidney was found to be atrophic and displayed a pelvic localization. Laboratory studies revealed no pathological findings other than fever and leukocytosis. Defined cystic lesion was drained under US guidance (the same procedure provided in patient 1). Three hundred milliliters of a purulent fluid was collected through the catheter. The culture of this fluid grew Pseudomonas aeruginosa. After appropriate antibiotic therapy and uterine drainage were provided, the overall condition of the patient improved, and hydronephrosis was found to be regressed. Posterior-sagittal anorectovaginoplasty procedure was planned, and the patient was discharged.
O. Algin et al.: Ultrasound-Guided Percutaneous Drainage
Fig. 8 After the primary repair of the tracheoesophageal fistula, hydronephrosis was found in the left kidney of patient 3 (A). One week after the ultrasound-guided percutaneous drainage, a marked regression was determined in hydronephrosis (B)
Discussion Acute urinary retention, or the inability to pass urine, is an uncommon diagnosis encountered in newborns [6]. Many situations are associated with acute urinary retention. Among these conditions, obstructive lesions of the genital tract (e.g., imperforate hymen, cervical-vaginal atresia, and cloacal malformation) are very rare [5]. The accumulation of menstrual blood (e.g., hematometrocolpos) and purulent fluid (e.g., pyometrocolpos) in the vagina and uterus may result in a mechanical effect on the urethra and/or bladder and lead to the obstructive urinary symptoms [7]. Also, inordinate intrauterine stimulation of the newborn’s cervical mucous glands by maternal estrogen in the presence of an obstruction leads to hydrometrocolpos, where secretions accumulate in the vagina and uterus [8, 9]. Hemato-, hydro-, and pyometrocolpos or a combination of these pathologies may lead to variable degrees of hydroureter, hydronephrosis, and/or nephronia [6]. The first lower genital tract obstruction with a labia minora tumor in an infant was reported by Godefroy in 1856, and Rocher and Balard reported a similar infant with vulvar mass and pyometrocolpos in 1932 [10]. Lack of awareness among physicians and the relative rarity of lower genital tract obstructive malformations and pyometrocolpos have resulted in errors in diagnosis, unnecessary abdominal exploration, and hysterectomies, with mortality up to 50% [2]. The most common signs and symptoms of pyometrocolpos among infants are abdominal distention due to the presence of a mass, respiratory distress, septicemia, and/or hydronephrosis [2]. When a lower genital tract obstruction is suspected, detailed physical
S275
examination, US, and MRI should initially be performed to identify any abnormalities in the genital tract [2, 11]. Urinary system anomalies are associated with this situation in up to 30% of cases because of the close embryologic relationship between the paramesonephric and mesonephric ducts [11]. Further evaluation may require voiding cystourethrography, radiographic contrast study (cystogram), intravenous pyelogram, renal scintigraphy, and endoscopy [2]. Development of pyometrocolpos associated with lower genital tract atresia is a condition that should be treated immediately. When imperforate hymen is the underlying reason, the treatment can be carried out by surgical incision of the hymen [6, 7, 10]. Management of other etiologies (e.g., common urogenital sinus and cloacal malformation) is complex, and a preliminary colostomy and urinary diversion may be required [2]. In the presence of vaginaluterine infection, sepsis, or acute renal failure, performing those corrective surgeries may lead to various severe consequences [3, 5, 9]. Moreover, those definitive corrective procedures are recommended to be applied to patients who are 1 year old or older, rather than neonates [3, 9]. Until the appropriate surgical procedure can be performed, pyometrocolpos should be drained immediately [2, 5]. To our knowledge, there are only two case reports on percutaneous drainage of neonatal hydropyometrocolpos [3, 5]. Although one of those case reports involves application of percutaneous drainage under general anesthesia, the other includes drainage under guidance of computed tomography [3, 5]. To our knowledge, the literature contains no case report or study concerning US-guided percutaneous drainage of pyometrocolpos under local anesthesia. US-guided percutaneous drainage under local anesthesia is a superior technique than the CT-guided method because it involves no radiation exposure, and therefore, it should be the preferred technique. This procedure may be performed local anesthesia, which eliminates the risks of general anesthesia and its possible complications [3]. The three patients we report here showed marked improvement in their overall health and regression of hydronephrosis after we performed US-guided percutaneous drainage under local anesthesia. The patients were monitored until the definitive surgical procedure could be performed, and no complications or side effect were observed. The most important step in the diagnosis of neonatal lower genital tract obstruction and/or pyometrocolpos in infants is a thorough, detailed physical examination; modern imaging methods such as US and MRI are also helpful. In these patients, US-guided percutaneous drainage under local anesthesia is a simple, minimally invasive, safe, and effective procedure that facilitates a later successful corrective surgical procedure and avoids the need for more complex and invasive drainage procedures.
123
S276
References 1. Liu YP, Chen CP (2009) Fetal MRI of hydrometrocolpos with septate vagina and uterus didelphys as well as massive urinary ascites due to cloacal malformation. Pediatr Radiol 39:877 2. Nazir Z, Rizvi RM, Qureshi RN et al (2006) Congenital vaginal obstructions: varied presentation and outcome. Pediatr Surg Int 22:749–753 3. Imamoglu M, Cay A, Sarihan H et al (2005) Two cases of pyometrocolpos due to distal vaginal atresia. Pediatr Surg Int 21:217–219 4. Lashley DB, Thomas RD, Silberger PJ, Skoog SJ (1998) Management of an infected hematometrocolpos in a patient with congenital adrenal hyperplasia and vaginal stenosis. J Urol 160:508–509 5. Dursun I, Gunduz Z, Kucukaydın M et al (2007) Distal vaginal atresia resulting in obstructive uropathy accompanied by acute renal failure. Clin Exp Nephrol 11:244–246
123
O. Algin et al.: Ultrasound-Guided Percutaneous Drainage 6. Chang JW, Yang LY, Wang HH et al (2007) Acute urinary retention as the presentation of imperforate hymen. J Chin Med Assoc 7012:559–561 7. Dane C, Dane B, Erginbas M, Cetin A (2007) Imperforate hymen—a rare cause of abdominal pain: two cases and review of the literature. J Pediatr Adolesc Gynecol 20:245–247 8. Aygun C, Ozkaya O, Ayyildiz S et al (2006) An unusual cause of acute renal failure in a newborn: hydrometrocolpos. Pediatr Nephrol 21:572–573 9. Shaw LMA, Jones WA, Breton RJ (1983) Imperforate hymen and vaginal atresia and their associated anomalies. J R Soc Med 76:560–566 10. Ash DG, Smith IM (1965) Neonatal hematocolpos. Can Med Assoc J 92:1225–1226 11. Junqueira BLP, Allen LM, Spitzer RF et al (2009) Mu¨llerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics 29:1085–1103
