Abstract
The primordial germ cells are first found at the 24th gestational day near the allantois. The germ cells proliferate and migrate to reach the genital ridge, which, by the 5th week, becomes elevated and thickened. In ovarian agenesis, one or both ovaries are absent; it usually accompanies defects of the tubular reproductive organs; unilateral ovarian agenesis (UOA) and fallopian descent problems are very rare congenital defects.
Gonadal dysgenesis generally refers to a condition where gonadal development is abnormal, often only presenting streaks of connective tissue that are often called “streak gonads.”
A fetal ovarian cyst refers to an ovarian cyst detected antenatally in a female fetus. They are usually diagnosed in the 3rd trimester. The first fetal ovarian cyst was reported in 1889 as an autopsy finding in a stillborn preterm infant; large ovarian cysts may be seen in the neonatal period presenting as an abdominal mass.
An ovarian dermoid cyst is a benign tumor descending from the ovarian germinal cells; in approximately 80 % of the cases, this lesion occurs in young girls, and it represents 18–20 % of benign ovarian tumors. In most cases, dermoid cysts are unilateral, but they are bilateral in 10–15 % of cases. Ovarian neoplasms are extremely rare and account for less than 1 % of all pediatric tumors.
Undescended ovaries are characterized by the attachment of the upper ovarian pole to an area at or above the level of the iliac artery bifurcation, but ectopic ovaries may be unilateral or bilateral and may be associated with abnormalities of the urinary tract anomalies.
Supernumerary ovaries include cases in which a third ovary is entirely separated from the eutopic ovary and may be located in the omentum or retroperitoneally. The term “accessory ovary” refers to cases in which excess ovarian tissue is situated near and connected to the normally placed ovary.
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References
Ovary
Cohen HL, Shapiro MA, Mandel FS, Shapiro ML. Normal ovaries in neonates and infants: a sonographic study of 77 patients 1 day to 24 months old. Am J Roentgenol. 1993;160:583–6.
Ovarian Agenesis
Kaya H, Sezik M, Ozkaya O, Kose SA. Mayer–Rokitansky–Kuster–Hauser syndrome associated with unilateral gonadal agenesis. A case report. J Reprod Med. 2003;48:902–4.
Mylonas I, Hansch S, Markmann S, Bolz M, Friese K. Unilateral ovarian agenesis: report of three cases and review of the literature. Arch Gynecol Obstet. 2003;268:57–60.
Ottolenghi C, et al. Absence of mutations involving the lim homeobox domain gene LHX9 in 46,XY gonadal agenesis and dysgenesis. J Clin Endocrinol Metab. 2001;86(6):2465–70.
Bedaiwy MA, et al. Reproductive outcome after transplantation of ovarian tissue: a systematic review. Hum Reprod. 2008;23(12):2709–17.
Ovarian Dysgenesis
Turner HH. A syndrome of infantilism, congenital webbed neck, and cubitus valgus. Endocrinology. 1938;23:566–74.
Davenport ML. Approach to the patient with Turner syndrome. J Clin Endocrinol Metab. 2010;95(4):1487–95.
Carrell DT. Reproductive endocrinology and infertility: integrating modern clinical and laboratory practice. New York: Springer; 2010. p. 308. ISBN 978-1-4419-1435-4.
Alpman A, Cogulu O, Akgul M, et al. Prenatally diagnosed turner syndrome and cystic hygroma: incidence and reasons for referrals. Fetal Diagn Ther. 2008;25(1):58–61.
Bannink EM, van der Palen RL, Mulder PG, de Muinck Keizer-Schrama SM. Long-term follow-up of GH-treated girls with Turner syndrome: metabolic consequences. Horm Res. 2009;71(6):343–9.
Donaldson MD, Gault EJ, Tan KW, Dunger DB. Optimising management in Turner syndrome: from infancy to adult transfer. Arch Dis Child. 2006;91(6):513–20.
Ovarian Cyst
Carlson DH, Griscom TN. Ovarian cysts in the newborn. Am J Roentgenol. 1972;116:664–72.
Meizner I, Levy A, Katz M, Maresh AJ, et al. Fetal ovarian cysts: prenatal ultrasonographic detection and postnatal evaluation and treatment. Am J Obstet Gynecol. 1991;164(3):874–8.
Jian M, Pujiani M, Madan MN, Chadah R, Puri A. Congenital ovarian cyst: a report of two cases. J Lab Physicians. 2012;4(1):63–5.
Nussbaum AR, Sanders RC, Hartmann DS. Neonatal ovarian cysts: sonographic-pathologic correlation. Radiology. 1988;168:817–21.
Bryant AE, Laufer MR. Fetal ovarian cysts: incidence, diagnosis and management. J Reprod Med. 2004;49(5):329–37.
Mudholkar VG, Acharya AS, Kulkarni AM, Hirgude ST. Antenatally diagnosed neonatal ovarian cyst with torsion. Indian J Pathol Microbiol. 2011;54:228–9.
Akin MA, et al. Fetal-neonatal ovarian cysts-their monitoring and management: retrospective evaluation of 20 cases and review of the literature. J Clin Res Pediatr Endocrinol. 2010;2(1):28–33.
Neonatal Ovarian Cysts
Marshall JR. Ovarian enlargements in first year of life. Ann Surg. 1965;161:372–7.
Acikalin MF, Tokar B. Giant cyst of the rete ovarii in a child. J Pediatr Surg. 2005;40:17–9.
Bower R, Dehner LP, Dehner JL. Bilateral ovarian cysts in the newborn a triad of neonatal abdominal masses, polyhydramnios, and maternal diabetes mellitus. Am J Dis Child. 1974;128(5):731–3.
Chiaramonte C, Piscopo A, Cataliotti F. Ovarian cysts in newborns. Pediatr Surg Int. 2001;17(2–3):171–4.
Fahmy M. Early minimal surgical intervention for antenatally detected ovarian cyst. Sc J Med Fac (girls). 2005;26(1):903–10.
Ovarian Dermoid Cyst (DC)
Alymlahi E, Dafiri R. Bilateral ovarian dermoid cyst. Appl Radiol. 2005;34(9):38–42.
Bazot M, Cortez A, Sananes S, et al. Imaging of dermoid cysts with foci of immature tissue. J Comput Assist Tomogr. 1999;23:703–6.
Patel MD, Feldstein VA, Lipson SD, et al. Cystic teratomas of the ovary: diagnostic value of sonography. AJR Am J Roentgenol. 1998;171:1061–5.
Troiano RN, McCarthy S. Magnetic resonance imaging evaluation of adnexal masses. Semin Ultrasound CT MR. 1994;15:38–48.
Congenital Ovarian Tumors
Vaysse C, et al. Ovarian germ cell tumors in children. Management, survival and ovarian prognosis. A report of 75 cases. J Pediatr Surg. 2010;45:1484–90.
Outwater EK, Siegelman ES, Hunt JL. Ovarian teratomas: tumor types and imaging characteristics. Radiographics. 2001;21:475–90.
Skinner MA, Schlatter MG, Heifetz SA, Grosfeld JL. Ovarian neoplasms in children. Arch Surg. 1993;128(8):849–54.
Talerman A. Blaustein’s pathology of the female genital tract. In: Kurman RJ, editor. Germ cell tumors of the ovary. 5th ed. New York: Springer; 2002. p. 967–1034.
Song ES, Lee JP, Han JH, Kim HY, Mun SH, Ryu HS, et al. Dysgerminoma of the ovary with precocious puberty: a case report. Gynecol Endocrinol. 2007;23(1):34–7.
Levitin A, Haller KD, Cohen HL, et al. Endodermal sinus tumor of the ovary: imaging evaluation. AJR Am J Roentgenol. 1996;167(3):791–3.
Undescended and Maldescent Ovary
Bardo DM, Black M, Schenk K, Zaritzky MF. Location of the ovaries in girls from newborn to 18 years of age: reconsidering ovarian shielding. Pediatr Radiol. 2009;39:253–9.
Healey A. Embryology of the female reproductive tract. In: Mann G, et al., editors. Imaging of gynecological disorders in infants and children, medical radiology. Diagnostic imaging, 2010,128. Berlin, Heidelberg: Springer; 2012.
Özbey H, Ratscheka M, Schimpla G, Höllwarth NE. Ovary in hernia sac: prolapsed or a descended gonad? J Pediatr Surg. 1999;34(6):977–80.
Allen JW, Cardall S, Kittijarukhajorn M, Siegel CL. Incidence of ovarian maldescent in women with Müllerian duct anomalies: evaluation by MRI. Am J Roentgenol. 2012;198(4):W381–5.
Dabirashrafi H, Mohammad K, Moghadami-Tabrizi N. Ovarian malposition in women with uterine anomalies. Obstet Gynecol. 1994;83:293–4.
Ectopic Ovary
Machiels F, De Maeseneer M, Desprechins B, Casteels A, De Backer A, Osteaux M. Unusual location of an ovary: ultrasonographic features and surgical correlation. J Belge Radiol. 1998;81(6):288.
Munden M, McEniff N, Mulvihill D. Sonographic investigation of female infants with inguinal masses. Clin Radiol. 1995;50(10):696–8.
Supernumerary Ovary
Printz JL, Choate JW, Townes PL, Harper RC. The embryology of supernumerary ovaries. Obstet Gynecol. 1973;41:1246–52.
Pearl M, Plotz EG. Supernumerary ovary, report of a case. Obst Gynecol. 1963;12(2):253–5.
Wharton LR. Two cases of supernumerary ovary and one of accessory ovary within an analysis of previously reported cases. Am J Obstet Gynecol. 1959;78:1101.
Hogan ML, Barber DD, Kaufmann RH. Dermoid cyst in supernumerary ovary: the greater omentum: report of a case. Obstet Gynecol. 1967;29:405.
Ovotestes Anomalies
Schlaubitz S, Yatsenko SA, Smith LD, Keller KL, Vissers LE, Scott DA, Cai WW, Reardon W, et al. Ovotestes and XY sex reversal in a female with an interstitial 9q33.3-q34.1 deletion encompassing NR5A1 and LMX1B causing features of Genitopatellar syndrome. Am J Med Genet A. 2007;143A(10):1071–81.
Ortenberg J, Oddoux C, Craver R, McElreavey K, Salas-Cortes L, Guillen-Navarro E, Ostrer H, Sarafoglou K, Clarke V, Yee H. SRY gene expression in the ovotestes of XX true hermaphrodites. J Urol. 2002;167(4):1828–31.
Schwarz, Stephens. The persisting mesonephric duct: high junction of vas deferens and ureter. J Urol. 1978;120(5):592–6
Vordermark JS. The persisting mesonephric duct syndrome: the description of a new syndrome. J Urol. 1983;130(5):958–61.
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Fahmy, M.A.B. (2015). Ovary. In: Rare Congenital Genitourinary Anomalies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43680-6_14
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