Clinical Perinatal/Neonatal Case Presentation ⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢ Lethal Pulmonary Hypoplasia and With Transverse Vaginal Septum in a Newborn: A Case Report and Review of the Literature

William F. Liu, MD resuscitation. Intubation with positive inspiratory pressure (Ͼ50 cm Orestes Borrego, MD water) ventilation was required for the extremely noncompliant Michael Weiss, MD lungs. Apgar scores were 1, 6, and 6 at 1, 5, and 10 minutes, respec- Craig R. Sweet, MD tively. The initial newborn examination was remarkable for a small This is a case report of an unusual cause of Potter thorax. The abdomen was very distended. The infant had a slightly sequence. Autopsy showed lethal pulmonary hypoplasia in flattened nose and excess nuchal tissue, although no other anomalies association with a transverse vaginal septum, hydrocolpos, were identified. The lower extremities and labia majora of the pheno- and a secondary obstructive uropathy. typic female were edematous, and the was difficult to visualize. The normally placed was catheterized, with the bladder drain- ing and the abdominal mass remaining unchanged. Potter syndrome was originally described in infants with bilateral Initial chest x-ray showed a small thoracic volume with a right and associated lethal pulmonary hypoplasia.1 Since pneumothorax while on maximal conventional ventilatory support. A this original report, Potter syndrome or oligohydramnios sequence thoracostomy tube was placed, and the infant’s respiratory status have been associated with renal dysplasia or hypoplasia, polycystic stabilized over the first 36 hours of life on high frequency jet ventila- kidneys, lower urinary tract obstruction from multiple etiologies, tory support combined with a priscoline drip. and chronic leaking amniotic fluid.2 The mass was tapped for 220 ml of straw-colored fluid at 5 hours To our knowledge, this is the first reported case in the English of life. A repeat tap was obtained at 50 hours of life. The initial fluid literature of a newborn with autopsy-confirmed Potter syndrome analysis included the following: sodium of 88 mmol/liter, potassium with pulmonary hypoplasia, hydrocolpos, and a transvaginal of 12.6 mmol/liter, chloride of 60 mmol/liter, creatinine of 14.6 mg/ septum. dl, albumin of 0.6 gm/dl, and a total serum protein of Ͻ2.0 gm/dl. A We present the case with a review of the literature. repeat tap had similar values, but its character was more cloudy yel- low in character. A cell count analysis on this second tap showed 3825 CASE REPORT red blood cells per cubic millimeter, 1850 white blood cells per cubic millimeter (with 91% neutrophils and 9% lymphocytes), and many A 2965-gm, 36-week preterm white infant girl of appropriate gesta- epithelial cells. tional age was delivered from a 29-year-old primigravida by elec- Abdominal x-ray demonstrated a large midline fluid-filled mass tive cesarean section due to a breech presentation. Antenatal ultra- with a suspected area of calcification in the right quadrant (Figure 1). sound had demonstrated hydronephrosis and a large midline Abdominal ultrasound showed a 12 ϫ 5 ϫ 5.6-cm cystic ab- abdominal mass. Oligohydramnios was apparent starting at 30 domino-pelvic mass that appeared separate and posterior to the blad- weeks’ gestation. Amniocentesis and karyotype at 30 weeks re- der. There was moderate bilateral hydronephrosis and hydroureter vealed the infant to be 46, XX (female karyotype), Fluid was col- (Figure 2). A repeat ultrasound 2 days after the initial tap did not lected from the mass, with chemical analysis showing the follow- demonstrate reaccumulation of the abdominal fluid. ing levels: sodium of 68 mmol/liter, chloride of 60 mmol/liter. Cranial ultrasound and echocardiography did not demonstrate Oligohydramnios persisted in this membrane-intact gestation. any definitive abnormalities. Blood, body fluid, and urine cultures Upon delivery at 36 weeks, the infant required immediate were negative for bacterial growth. Serum electrolytes were within Departments of Pediatrics/Neonatology (W. F. L.), Reproductive Endocrinology (C. R. S.), normal limits. Urinalysis was also unremarkable. The infant was Pathology (O. B.), and Radiology (M. W.), Lee Memorial Health Systems, The Children’s Hos- initially oligouric, but responded well to furosemide, with the urine pital of Southwest Florida, HealthPark Medical Center, Fort Myers, FL. output remaining between 1 and 2 ml/kg per hour. Address correspondence and reprint requests to William F. Liu, MD, The Children’s Hospital of Southwest Florida, Healthpark Medical Center, 9981 Healthpark Circle, Fort Myers, FL Respiratory status deteriorated, with progressive and persistent 33908. hypoxemia after 36 hours of life. There was no recurrent air block,

Journal of Perinatology (1999) 19(6) 454–459 © 1999 Stockton Press. All rights reserved. 0743–8346/99 $12 454 http://www.stockton-press.co.uk Pulmonary Hypoplasia and Hydrocolpos With Transvaginal Septum Liu et al.

Figure 1. Initial x-ray demonstrating the small thoracic volume, right pneumo- thorax, large fluid-filled abdominal mass. A bladder catheter is in place. There is a faint suggestion of intra-abdominal calcification in the right lower quadrant (arrow).

Figure 3. Postmortem appearance of a large hydrocolpos (A). Superiorly, a small uterus (B) and right fallopian tube are noted.

An autopsy was performed; findings included pulmonary hyp- oplasia with a lung weight/body weight of 0.0081. A hydrocolpos with a transverse vaginal septum of the lower third of the vagina (Figure 3) was also identified. There was moderate bilateral hydronephrosis with hydroureters and a normal appearing bladder (Figure 4). There were no other urogenital anomalies.

DISCUSSION This case represents a rare cause of Potter syndrome or oligohydram- nios sequence. In this case, we believe that the transvaginal septum Figure 2. Abdominal ultrasound demonstrating the partially decompressed blad- led to hydrocolpos, which resulted in an obstructive uropathy. Hydro- der with a draining catheter in place. There is a large pelvic mass extending to the colpos has been associated with urinary obstruction causing bilateral abdomen, posterior to the bladder. hydroureter and hydronephrosis.3–9 When this is severe, there may be in utero oligo/anuria resulting in oligohydramnios. Some cases may be less severe and may present later in infancy as an abdominal mass and despite aggressive ventilatory support and pulmonary vasodila- with urinary retention and frequency.3,10 tion, the infant died of refractory respiratory failure at 55 hours of Oligohydramnios sequence is associated with fetal compression. A age. Serum creatinine was 1.7 mg/dl and blood urea nitrogen was 21 flattened nose, redundant nuchal tissue, low-set ears with inner can- mg/dl at death. thal skin folds, and a small, receding chin characterize Potter’s facies.

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Sfar et al.8 reported two cases of hydrocolpos with neonatal death secondary to pulmonary hypoplasia. The first case was an infant with hydrocolpos and postaxial polydactyly consistent with McKusick- Kaufman syndrome. The precise etiology of the hydrocolpos was not clear. The physical features of Potter syndrome were described in this infant. The second case was an infant with ambiguous genitalia with imperforate anus. Both cases reportedly had postmortem findings of obstructive uropathy and pulmonary hypoplasia. There have been several cases of neonatal death attributable to respiratory symptoms without clear pathologic documentation. Many of these cases may have been due to pulmonary hypoplasia.3,6,7,9,24–26 Nonlethal respiratory difficulties have also been reported and may be attributable to an abdominal mass effect with subsequent Figure 4. Postmortem photograph showing bilateral hydronephrosis. elevation of the diaphragm resulting in pulmonary compres- sion.4,27–31 The extremities may be malpositioned with flexion contractures, and there may be intrauterine growth retardation. These infants typically Antenatal Diagnosis of Hydrocolpos die of respiratory failure from pulmonary hypoplasia.11 Shimada et al.32 reviewed 45 fetuses with antenatal diagnosis of geni- tourinary abnormalities and oligohydramnios. The average gesta- Pulmonary Hypoplasia tional age at detection of the oligohydramnios was 30 weeks, as was Pulmonary hypoplasia has been defined by the following: decreased our case. There were two cases of hydrometrocolpos, with a 50% mor- radial alveolar counts and a lung-to-bodyweight ratio of Ͻ0.012 for tality rate. infants of Ն28 weeks’ gestation and Ͻ0.015 for infants of Ͻ28 In addition to oligohydramnios, our case presented with a large weeks’ gestation. Histologically, Wigglesworth12 showed that pulmo- midline pelvic abdominal mass and hydronephrosis. We could not nary hypoplasia combined with oligohydramnios detected at Ͻ20 make a definitive antenatal diagnosis because the bladder was so weeks’ gestation showed a failure of growth and maturation of the compressed and poorly visualized. The false diagnosis of primary peripheral parts of the pulmonary acinus. There is a general delay in 24 epithelial maturation, a lack of elastic development, and low concen- lower urinary tract obstruction is common. Antenatal diagnosis of hydrocolpos or hydrometra should be suspected when a cystic midline trations of lung phospholipids. In contrast, pulmonary hypoplasia 24,33–36 seen with normal or increased amniotic fluid has a pulmonary struc- mass is identified as arising from the fetal pelvis. ture appropriate for gestational age and normal phospholipid content. Abnormal Newborn Signs and Symptoms Associated The cause of fetal lung hypoplasia with oligohydramnios is gen- With Hydrocolpos erally felt to be due in part to direct physical chest compression from The newborn may present with a large abdominal mass. In some decreased amniotic fluid. This may be secondary to decreased urine cases, the abdomen may be so distended as to cause dystocia at deliv- output, chronically leaking amniotic fluid, or a greater efflux of tra- ery.4 In cases caused by , there is generally a bulg- 11,13,14 cheal fluid. ing seen at the vaginal introitus. On occasion, this may also occur Oligohydramnios may result in a decrease in the normal tracheo- with a low transverse vaginal septum, with these cases being misdiag- bronchial fluid volume while the fetal chest compression further nosed as an imperforate hymen. In atresia of the vagina with hy- compromises optimal fetal breathing patterns. Animal studies in drometrocolpos, the perineum may appear retracted upward, suggest- which pulmonary fluid was drained from the lung resulted in pulmo- 15–19 ing a traction effect from the uterine mass expanding up into the nary hypoplasia. Fetal respiratory activity and retention of fluid abdomen.3 within the pulmonary tree become more apparent in the second tri- In our case, the infant probably had edema of the lower extremi- mester, which may explain why this is a particularly vulnerable pe- ties and labia due to vena caval obstruction. Bowel obstruction and riod. The risk for pulmonary hypoplasia is high during the pseu- peritonitis have also been reported.4,9 Bowel obstruction may be re- doglandular (12 to 16 weeks) and canalicular (17 to 28 weeks) lated to primary anorectal atresias or to direct compression from the phases of lung development. Even a short period of oligohydramnios hydrocolpos. This rare mass effect has been reported to be so extreme during a critical period of lung development may lead to pulmonary as to cause in utero rupture of the cecum against the pelvic brim with hypoplasia.20–23 secondary peritonitis and obstruction.26 Primary rupture of the hydro- Potter Syndrome and Hydrocolpos colpos with peritonitis has also been reported in a patient with an To our knowledge, this is the first reported case of autopsy-docu- obstructed and a recto-cloacal fistula.31 Hirsh- mented Potter syndrome associated with hydrocolpos with a transverse sprung’s disease has also been reported as a cause of peritonitis in an vaginal septum in an infant without additional major malformations. infant with hydrocolpos. 26 The fluid contained in the vagina may

456 Journal of Perinatology (1999) 19(6) 454–459 Pulmonary Hypoplasia and Hydrocolpos With Transvaginal Septum Liu et al.

move through the uterus and fallopian tubes into the abdominal meningocele, upper urinary tract dilation, ureterocele with duplica- cavity, causing fibrinous peritonitis with peritoneal calcification, as tion, mesenteric cyst, gastrointestinal duplication, urachal or ovarian we suspect in our case, and even bowel obstruction.24 cyst, hydrocolpos, or hydrometrocolpos. Ovarian, mesenteric, or omental and duplication cysts may be unilocular or multilocular.27,40 Radiology Studies of Hydrocolpos Hydrocolpos and hydrometrocolpos have been reported to be the An abdominal x-ray will usually demonstrate a midline globular soft third most common cause of an abdominal mass in the newborn,51 tissue mass arising from the pelvis displacing the intestine superiorly.4 accounting for 15% of all abdominal masses in female newborns with There may be features of intestinal obstruction or perforation. If there an estimated occurrence rate of 1 per 16,000 female deliveries.4 is peritonitis, there may be intra-abdominal calcifications.26 Hydrocolpos was first described secondary to imperforate hymen Ultrasound findings demonstrate a pelvic-abdominal fluid-filled by Godefrey52 in 1856. Since 1940, there have been several review mass located between the bladder and the rectum or spine.25,37 Inter- articles describing hydrocolpos or hydrometrocolpos in the litera- nal echoes are a result of mucoid debris made up of cervical mucus, ture.3,4,7,43,53 desquamated epithelium, and leukocytes.38–40 In cloacal malforma- tion, this fluid level may be related to a mixture of urine, uterovaginal Etiology of a Hydrocolpos secretions, and meconium. The causes of hydrocolpos or hydrometrocolpos include transvaginal To distinguish between a dilated uterus and vagina, the distended septum, , and imperforate hymen.4,54 Imperforate uterus has a thickened wall, whereas a distended vagina has a thin, hymen will present with a characteristic interlabial bulge. These are almost imperceptible wall.25 In either case, a fluid-debris level may be usually asymptomatic at birth and may present as a at an important finding to distinguish between the mass and the some- the time of menarche.3 times difficult to visualize distended bladder.25,38 Hydrocolpos can also be seen with obstructed urogenital sinus or cloacal malformations.25 Associated anomalies are relatively rare with Fluid Analysis/Hydrocolpos an imperforate hymen but are more common with vaginal atresia, Hydrometrocolpos and hydrocolpos are thought to arise from the urogenital sinus, or cloacal malformation.3,9,55 entrapment of the infant’s cervical mucus gland secretions, which have been stimulated by maternal estrogen.4,6,39,41 Hahn-Pedersen et Reported urinary abnormalities described with a hydrocolpos or al.9 suggested a distinction between secretory and urinary hydrocolpos hydrometrocolpos include unilateral renal agenesis, renal hypoplasia that is dependent on a genitourinary communication. or dysplasia, polycystic kidneys, ureteral duplication, vesicovaginal The fluid has been described as acid and serous or as mucoid fistula, and genital duplication including uterine didelphis, double 3,9,25,33,37,50,56–58 containing large numbers of desquamated epithelial cells, leukocytes, , and bifid clitoris. and erythrocytes.3 The range of descriptions include clear,35,42–44 Associated gastrointestinal and musculoskeletal anomalies in- yellowish,28 turbid yellow,26 white or milky white,8,29,36,39,42,45–48 yel- clude sacral hypoplasia, congenital hip dysplasia, esophageal and lowish creamy,44 or bloody mucoid fluid.37,39 duodenal atresia, colon duplication, and imperforate anus with recto- 3,4,6,43,59 Chemical analysis of the fluid has rarely been described. Hender- vaginal fistula. disorders and congenital adrenal son46 noted that a serous fluid aspirated from a newborn with imper- hyperplasia have been reported with hydrometrocolpos associated with 9,60,61 forate hymen had a protein level of 1.7 gm/dl. In cases of a urogenital a urogenital sinus. sinus as a cause of hydrometrocolpos or ectopic and hydrocol- The triad of hydrometrocolpos, polydactyly, and cardiac malfor- pos, the distended mass may be largely filled with urine.9,49,50 Sharma mation is called McKusick-Kaufman syndrome, which has an autoso- 62,63 et al.28 described a case of probable urogenital sinus with analysis of mal recessive inheritance pattern. Isolated inheritable syndactyly yellowish fluid with a high urea content (200 to 300 mg/dl) consis- has also been reported.64 tent with urine. Although the high creatinine level in our fluid analy- Embryology of the Vagina ses suggested a urinary source, needle aspiration with follow-up ultra- The embryology of the vagina is complex. The coelomic epithelium sound did not demonstrate reaccumulation of the mass, such as invaginates, forming the mu¨llerian duct system. The fusion of the would be expected if there was a communication between the urinary distal portion of the mu¨llerian ducts forms the uterus and the distal system and vagina. In this case, our autopsy findings also did not vagina. This fusion initially creates a solid cord of tissue called the reveal any evidence of a genitourinary communication. mu¨llerian tubercle. The urogenital sinus grows upward, meeting the In cases of delayed diagnosis, the fluid may become infected or downward-growing mu¨llerian tubercle at ϳ10 weeks’ gestation. The inflamed and may be described as “purulent” or as “foul cloudy mu¨llerian portion becomes the uterovaginal primordium, and the fluid” reflecting a mass with chronic or acute infection.3,27,30,49 urogenital sinus grows upward to form the vaginal plate.65 It is gener- Differential Diagnosis of a Midline Pelvic-Abdominal ally thought that the upper two-thirds of the vagina is derived from Mass in the Newborn the mu¨llerian ducts, whereas the distal one-third of the vagina origi- The differential diagnosis of a pelvic-abdominal mass in the newborn nates from the urogenital sinus. A vaginal lumen normally forms by includes neuroblastoma, presacral teratoma and lymphoma, anterior the involution of the central core.

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Failure of the mu¨llerian ducts to fuse to the urogenital sinus may 10. Mandell J, Stevens PS, Lucey DT. Diagnosis and management of hydrometrocol- result in a transverse vaginal septum, a type of vertical fusion defect.66 pos in infancy. J Urol 1978;120:262–5. This process can occur at a variable distance above the hymenal ring. 11. Thomas IT, Smith DW. Oligohydramnios, cause of the nonrenal features of Pot- Histologically, the cells above the transvaginal septum are usually ter’s syndrome, including pulmonary hypoplasia. J Pediatr 1974;84:811–5. columnar epithelium and stratified squamous epithelium below the 12. Wigglesworth JS. Perinatal Pathology. Vol 15. 2nd ed. Philadelphia: W. B. Saun- septum, but may be modified by the effect of maternal estrogens and ders; 1996. p. 164–9. 65 exposure of the epithelium to normal vaginal flora. 13. Perlman M, Williams J, Hirsch M. Neonatal pulmonary hypoplasia after pro- Clinically, one-half of transverse vaginal septa are found in the longed leakage of amniotic fluid. Arch Dis Child 1976;51:349–53. upper third of the vagina, one-third in the middle segment, and the 14. Bain AD, Smith IT, Gadd IK. Newborn after prolonged leakage of liquor amnii. remainder in the lower third of the vagina.67 BMJ 1964;2:598–9. Treatment 15. Alcorn D, Adamson TM, Lambert TF, et al. Morphological effects of chronic tra- In cases in which there is a simple imperforate hymen or bulging low cheal ligation and drainage in the fetal lamb. J Anat 1977;123:649–60. transverse vaginal septum, direct drainage by complete incision or an 16. Fewell JE, Hislop AA, Kitterman JA, et al. Effect of tracheostomy on lung develop- 68 incisional Z-plasty technique may be most effective. Postoperative ment in fetal lambs. J Appl Physiol 1983;55:1103–8. dilatation may be needed, because circumferential stenosis is a com- 9,27,44,47 17. Wigglesworth JS, Winston RML, Bartlett K. Influence of the central nervous sys- mon complication. tem on fetal lung development: experimental study. Arch Dis Child 1977;52: With a mid or high transvaginal septum, a combined abdomi- 965–7. nal-perineal approach may be necessary. Vaginal drainage is used 18. Wigglesworth JS, and Desai R. Effect on lung growth of cervical cord section in postoperatively.9,18,43,47,69 Postoperative dilatation is generally neces- the rabbit fetus. Early Hum Dev 1979;3:51–65. sary to maintain patency.44 Additional surgery may be necessary at puberty to allow for satisfactory menstruation and coitus.49 19. Liggins GC, Vilos GA, Campos GA, et al. The effect of bilateral thoracoplasty on lung development in fetal sheep. J Dev Physiol 1981;3:275–82. 20. Hislop A, Hey E, Reid L. The lungs in congenital bilateral renal agenesis and SUMMARY dysplasia. Arch Dis Child 1979;54:32–8. We report a newborn case of hydrocolpos caused by a transverse vagi- 21. Nimrod C, Varela-Gittings F, Machin G, et al. The effect of very prolonged mem- nal septum with secondary obstructive uropathy leading to Potter brane rupture on fetal development. Am J Obstet Gynecol 1984;148:540–3. syndrome with lethal pulmonary hypoplasia. A transverse vaginal 22. Moessinger AC, Collin MH, Blanc WA, et al. Oligohydramnios-induced lung hyp- septum should be considered as a potential cause of midline pelvic- oplasia: the influence of timing and duration in gestation. Pediatr Res 1986;20: abdominal masses in the newborn. Effective diagnosis and treatment 951–4. should include a multidisciplinary approach with a thorough evalua- 23. Thibeault DW, Beatty EC Jr, Hall RT, et al. Neonatal pulmonary hypoplasia with tion for associated anomalies. premature rupture of fetal membranes and oligohydramnios. J Pediatr 1985;20: 107:273–7. References 24. Ceballos R, Hicks GM. Plastic peritonitis due to neonatal hydrometrocolpos: 1. Potter EL. Bilateral renal agenesis. J Pediatr 1946;29:68–76. radiologic and pathologic observations. J Pediatr Surg 1970;5:63–70. 2. Jones KL. Smith’s Recognizable Patterns of Human Malformation. 5th ed. Phila- 25. Blask AR, Sanders RC, Gearhart JP. Obstructed uterovaginal anomalies: demon- delphia: W. B. Saunders; 1988. p. 632–3. stration with sonography. Part I. Neonates and infants. Radiology 1991;179:79– 3. Spencer R, Levy DM. Hydrometrocolpos: report of three cases and review of the 83. literature. Ann Surg 1962;155:558–71. 26. Gupta I, Barson AJ. Hydrocolpos with peritonitis in the newborn. J Clin Pathol 4. Westerhout FC, Hodgman JE, Anderson GV, Sack RA. Congenital hydrocolpos. 1980;33:679–83. Am J Obstet Gynecol 1964;89:957–61. 27. Dell’Agnola CA, Fedele L, Zamberletti D, et al. The management of hydrocolpos. 5. Spence HM. Congenital hydrocolpos: a review with emphasis on urologic aspects Acta Eur Fertil 1984;15:51–7. and a report of four additional cases. JAMA 1962;180:1100–5. 28. Sharma S, Aggarwal AK, Puri S, et al. Hydrometrocolpos with respiratory distress. 6. Reed MH, Griscom NT. Hydrometrocolpos in infancy. Am J Roentgenol Radium Indian Pediatr 1988;25:461–4. Ther Nucl Med 1973;118:1–13. 29. Robin M, Clouzeau J, Lamba P, Gerbaka B, Aigrain Y, Jejeune C. Hydrometrocol- 7. Cook GT, Marshall VF. Hydrocolpos causing urinary obstruction. J Urol 1964;92: pos neonatal. Une cause de detresse respiratoire immediatement neonatale. Arch 127–32. Fr Pediatr 1987;44:185–7. 8. Sfar E, Mahjoubi S, Gaigi S, et al. L’hydrocolpos foetal. Rev Fr Gynecol Obstet 30. Belghith M, Walha F, Saad H, et al. Hydrocolpos neo-natal avec atresie vaginale 1994;89:91–5. distale: a propos de deux cas. Chir Pediatr 1990;31:352–3. 9. Hahn-Pedersen J, Kvist N, Nielsen O. Hydrometrocolpos: current views on patho- 31. Stephenson CA, Ball TI Jr, Ricketts RR. An unusual case of meconium peritonitis genesis and management. J Urol 1984;132:537–40. associated with perforated hydrocolpos. Pediatr Radiol 1992;22:279–80.

458 Journal of Perinatology (1999) 19(6) 454–459 Pulmonary Hypoplasia and Hydrocolpos With Transvaginal Septum Liu et al.

32. Shimada K, Hosokawa S, Sakaue K, Kishima Y. Fetal genitourinary abnormali- 52. Godefrey M. Imperforation de la membrane hymen. Lancette Francaise. Gasette ties associated with oligohydramnios. Nippon Hinyokika Gakkai Zasshi 1994;85: Des hopitaux Civils Et Militaires 1856;29:567. 990–5. 53. Mahoney PJ, Chamberlain JW. Hydrometrocolpos in infancy: congenital atresia 33. Davis GH, Wapner RJ, Kurtz AB, et al. Antenatal diagnosis of hydrometrocolpos by of the vagina with abnormally abundant cervical secretions. J Pediatr 1940;17: ultrasound examination. J Ultrasound Med 1984;3:371–4. 772–80. . 34. Winderl LM, Silverman RK. Prenatal diagnosis of congenital imperforate hymen. 54. Ulfelder H. Agenesis of the vagina. Am J Obstet Gynecol 1968;100:745–51. Obstet Gynecol 1995;85:857–60. 55. Martin H, Reed MD, Griscome T. Hydrometrocolpos in infancy. AJR 1973;118:1– 35. Rasmussen MJ, McCarthy C, Badawi N, Bhatti HA. Antenatal ultrasound diagno- 13. sis of fetal hydrometrocolpos. Ir Med J 1993;86:28–9. 56. Burbige KA, Hensle TW. and vaginal duplication with unilateral 36. Mirk P, Pintus C, Speca S. Ultrasound diagnosis of hydrocolpos: prenatal findings obstruction presenting as a newborn abdominal mass. J Urol 1984;132:1195–8. and postnatal follow-up. J Clin Ultrasound 1994;22:55–8. 57. Sweet CR, Galle PC, McRae MA, Denby JL, Edwards MD. Transverse vaginal sep- 37. Tsukahara H, Fujimoto I, Hiraoka M, et al. Congenital hydrocolpos combined tum: a diagnosis at three months of age. Adolesc Pediatr Gynecol 1990;3:35–8. with unilateral renal agenesis. Acta Paediatr Jpn 1991;33:729–33. 58. Russ PD, Zavitz WR, Pretorius DH, et al. Hydrometrocolpos, uterus didelphys, and 38. Sawhney S, Gupta R, Brerry M, Bhatnagar V. Hydrometrocolpos: diagnosis and septate vagina: an antenatal sonographic diagnosis. J Ultrasound Med 1986;5: follow-up by ultrasound. A case report. Australas Radiol 1990;34:93–4. 211–3. 39. Wilson DA, Stacy TM, Smith EI. Ultrasound diagnosis of hydrocolpos and hy- 59. Johnston JH. The female genital tract. In: Rickham P, Lister J, Irving IM, editors. drometrocolpos. Radiology 1978;128:451–4. Neonatal Surgery. 2nd ed. London: Butterworths; 1978. p. 587. 40. Banerjee AK, Clarke O, MacDonald LM. Sonographic detection of neonatal hy- 60. Polasek PM, Erickson LD, Stanhope CR. Transverse vaginal septum associated drometrocolpos. Br J Radiol 1992;65:268–71. with tubal atresia. Mayo Clin Proc 1995;70:965–8. 41. Parsons L, Sommers SC. Gynecology. Philadelphia: W. B. Saunders; 1962. p. 14. 61. Bhasin RP, Pal DN, Mehdiratta KS. Congenital hydrocolpos, hydrometra, and hydrosalpinx with imperforate anus. Indian J Pediatr 1970;37:19–23. 42. Dawson WG. A case of hydrocolpos. BMJ 1973 21:155. 62. McKusick VA, Bauer RL, Koop CE, et al. Hydrometrocolpos as a simply inherited 43. Gravier L. Hydrocolpos. J Pediatr Surg 1969;4:563–8. malformation. JAMA 1964;189:813–6. 44. Nguyen L, Youseef S, Guttman FM, Ahlgren LS, Schlechter R. Hydrometrocolpos 63. Robinow M, Shaw A. The McKusick-Kaufman syndrome: recessively inherited in neonate due to distal vaginal atresia. J Pediatr Surg 1984;19:510–4. vaginal atresia, hydrometrocolpos, uterovaginal duplications, anorectal anoma- 45. Davis C Jr, Fell EH. Double hydrometrocolpos and imperforate anus in a newborn lies, postaxial polydactyly, and congenital heart disease. J Pediatr infant. Am J Dis Child 1950;80:79–84. 1979;94:776–8. 46. Henderson KC. Hydrometrocolpos in a newborn. Am J Dis Child 64. Wang J, Ezzat W, Davidson M. Transverse vaginal septum: a case report. J Reprod 1975;129:1190–1. Med 1995;40:163–6. 47. Galviya UD, Howell DG, McMahon MJ, Mogg RA. Hydrocolpos. Br J Urol 197;47: 65. Kanagasuntheram R, Dassanayake AGS. Nature of the obstructing membrane in 449–52. primary cryptomenorrhoea. J Obstet Gynaecol Br Emp 1958;65:487–92. 48. MacBeth WA, Rubin J. Hydrocolpos in infancy. Br J Surg 1963;50:887–9. 66. Woodward PJ, Sohaey R, Wagner BJ. Congenital uterine malformations. Curr 49. Mandell J, Stevens P, Lucey D. Diagnosis and management of hydrometrocolpos Probl Diagn Radiol 1995;24:178–97. in infancy. J Urol 1978;120:262–5. 67. Rock J, Schlaff W. The obstetric consequences of uterovaginal anomalies. Fertil 50. Sutton TJ, Gauthier N. Two unusual conditions simulating ectopic ureterocele: Steril 1985;43:681–92. unilateral hydrometrocolpos with ipsilateral renal agenesis or hypoplasia, and 68. Garcia R. Z-plasty for correction of congenital transverse vaginal septum. Am J ectopic ureteral opening into a seminal vesicle. Radiology 1975;117:381–4. Obstet Gynecol 1967;99:1164–5. 51. Raffensperger JG, Abousleiman A. Abdominal masses in children under one year 69. Ramenofsky M, Raffensperger J. An abdomino-perineal-vaginal pull-through for of age. Surgery 1968;63:514–21. definitive treatment of hydrometrocolpos. J Pediatr Surg 1971;6:381–7.

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