Pediatr Radio!

(1997) 27: 101-105 Springer-Verlag 1997

Sandra Schmahmann Jack O. Haller

Neonatal ovarian cysts: pathogenesis, diagnosis and management

Received: 29 March 1996 Accepted: 15 August 1996 S. Schmahmann Department of Radiology, Long Island College Hospital, 320 Henry Street, Brooklyn, New York, USA J. 0. Haller () Department of Radiology, State University of New York, Health Science Center at Brooklyn, 450 Clarkson Avenue, Box 1208, Brooklyn, NY, 11203, USA

Abstract This review discusses problems concerning the neonatal ovary. Neonatal ovarian cysts may undergo torsion and amputation. We discuss the pathogenesis, diagnosis, and management of these conditions.

We review the problems of the ovary in the normal neonate, neonatal ovarian cyst and neonatal ovarian torsion, with an emphasis on sonographic findings.
The normal neonatal ovary

At birth, maternal estrogens and HCG levels fall with the separation of the placenta from the neonate. FSH levels decline, the decline being slower in the premature infant [3, 4].
Neonatal ovarian cysts: etiology

Ovarian cysts have been found in the majority of neonatal and infant ovaries [1-3]. The typical neonatal and infant ovary is heterogeneous and cystic. In patients 1 day to 3 months of age, cysts of less than 9 mm were noted in 82 % and macrocysts (larger than 9 mm) were seen in 20 % of infants with cystic ovaries. The mean diameter of the largest cyst of each ovary was 7.5 mm [3]. Follicle stimulating hormone (FSH), maternal estrogens, and human chorionic gonadotropin (HCG) all contribute to follicular growth in utero. The primary stimulus is FSH, which is secreted by the fetal pituitary and which increases both the number and size of individual follicles. The hormonal balance present during the last months of fetal life contributes to further follicular growth.

Neonatal ovarian cysts are primarily of follicular origin and probably result from disordered folliculogenesis [5]. Most of the cysts usually resolve spontaneously. De Sa [6], in an autopsy study, showed small follicular cysts in 34 % of stillbirths and neonates within the first 28 days of life [1, 7]. Evidence suggests that excessive stimulation of the fetal ovary by both placental and maternal hormones may be a significant factor in cyst development [4, 5, 7]. There is an increased incidence of cysts in infants of mothers with diabetes, toxemia, or rhesus immunization, presumably from hypersecretion of placental HCG or increased placental permeability to HCG [1, 7-10]. Fetal ovarian cysts have been noted in neonates

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large simple cystic mass with one septation. The mass, an ovarian cyst, occupies the entire abdominal cavity Fig.2 a US scan of a fetal pelvis shows a right cystic mass in the region of the neonatal ovary. b Postnatal US scan shows that the previously cystic ovary is now echogenic. The mass is approximately the same size as it was originally; however, the echogenic material in the mass suggests the diagnosis of torsion. A torsive ovary was found at surgery

Fig.! Longitudinal US scan of the pelvis in the midline shows a

with hypothyroidism as well [11]. (A similar association occurs in later childhood.)

Complications

compression (with pulmonary hypoplasia), urinary tract obstruction or incarceration within an inguinal hernia [1, 4, 7, 9]. Polyhydramnios (5 %-12 %) and vaginal dystocia with cyst rupture are rare maternal complications of large fetal ovarian cysts [1, 7].

The complications of ovarian cysts are divided into primary, secondary, and maternal sequelae [1, 12, 13]. Torsion and hemorrhage are the most frequent pri- Sonographic appearance mary complications. Torsion is more common in large Cysts vary in size and appearance. The larger cysts may cysts, but has been reported in cysts as small as 2 cm [1, occupy almost the entire abdomen (Fig. 1) [13, 14]. The 4, 7, 10]. Large studies report that torsion occurred in appearance of a cyst varies, depending on whether the 42 % of patients [7, 10]. cyst is uncomplicated or complicated by torsion or hemTorsions, many of which occur antenatally [4, 7], may orrhage. An uncomplicated cyst is anechoic with an be accompanied by pain, vomiting, fever, abdominal imperceptible wall. A complicated cyst invariably condistension, leukocytosis and peritonitis. However, many tains a fluid-debris level, a retracting clot, septa, or is cases are asymptomatic [7, 10]. completely filled with echoes producing a solid massHemorrhagic cysts almost uniformly result from tor- like appearance (Fig. 2). These complex cysts often sion and associated infarction. A potentially lethal, but have echogenic walls resulting from dystrophic calcificarare complication, is cyst rupture causing hemorrhagic tion associated with infarction [7, 10]. ascites, and/or peritonitis [1, 7]. Secondary complicaIn the study of Nussbaum et al. [7], a fluid-debris tions from large cysts include bowel obstruction, thorax level and retracting clot were found to be specific signs

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of the pelvis shows a left adnexal mass with cystic and solid components. b After 2 weeks the mass had decreased in size and appeared as a simple cyst, the diagnosis of hemorrhagic ovarian cyst was made

Fig.3 a Longitudinal US scan

of torsion which had occurred before birth in the majority of cases. Muller-Leisse et al. [10] found uncomplicated cysts developed intraluminal echoes of varying morphology postnatally, which the authors attributed to mechanical stress of delivery and not to torsion. Nussbaum et al. [7] also found that the ovary contralateral to an ovarian cyst is frequently multicystic.

After birth, the decrease in hormonal stimulation is associated with spontaneous regression of most small cysts. Simple ovarian cysts of less than 4 cm in diameter can be observed using serial ultrasonography. Most cysts will resolve in 3-4 months, but longer periods of observation may be necessary if the cyst is decreasing in size [4, 8]. The treatment of larger cysts and complex cysts remains controversial [4, 7-9, 17]. Cysts larger than 4 cm are associated with a greater risk of torsion. Some surDifferential diagnosis geons feel that because ovarian torsion may lead to loss It may be impossible to differentiate an ovarian cyst of the ovary, early intervention is required. The managefrom a mesenteric or enteric cyst [6, 7, 10, 15]. Enteric ment options include surgery, laparoscopic needle aspiduplication cysts have been described by Barr et al. ration, or laparoscopy. Others, however, argue that [16] as having a hyperechoic mucosal layer with an un- since surgery has nothing to offer when the ovary is torderlying hypoechoic layer in all cases (thick wall sign). sive (and nonviable), observation may be the preferred A similar image can occasionally be found with ovarian management choice. When surgery is performed, it is cysts as well. important to preserve as much gonadal tissue as possiOther cystic structures which may be confused with ble and if practical merely to remove or unroof the ovarian cysts are hydrometrocolpos, cystic meconium cyst. Ovarian cysts are often adherent to the ovary and peritonitis, urachal cyst, bowel atresia or obstruction, re- frequently oophorectomy cannot be avoided [4, 8]. Ulnal cysts and anterior meningocele [1, 7, 8, 10, 15, 17]. trasound-guided needle aspiration of the cyst has no reComplex cysts, especially those presenting as a solid ported complications and offers excellent possibilities mass or a cystic mass with a retracting clot, may be im- with respect to preservation of the ovary [4]. possible to differentiate from a neoplasm (Fig. 3). OvaA successful laparoscopic approach to the managerian neonatal neoplasms are extremely rare, and ment of ovarian cysts has been reported. This technique include cystadenomas, cystic teratomas, and granulosa appears to be promising and offers both diagnostic cacell tumors [4, 7, 8, 17]. pabilities (visualization and biopsy) as well as a smooth transition to therapeutic measures when necessary. Cysts can be decapsulated by use of a laser or surgical incision, thus saving the ovary [18]. Management Torsion of the normal ovary and tube can occur at The management options for treating a neonatal ova- any age although it is rare in the infant (Fig.4) [19, 20]. rian cyst depend largely upon cyst size and sonographic The normal adnexa in girls may be very mobile, allowcharacteristics. Another factor is the potential risk of ing torsion at the mesosalpinx with changes in intra-abcomplications. Rarely, there is a need for differentiation dominal pressure or body position [21, 22]. of ovarian cysts from other ovarian tumors and from The ovary and fallopian tube undergo necrosis and other intraperitoneal cystic masses [4, 8]. amputation. The amputated ovary may either corn-

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organs by a long, twisted pedicle containing thin-walled vascular spaces. In the older child and adolescent the masses were adnexal, while in the neonate and infant they were extrapelvic. In all neonates and several infants' complex masses with multiple large cysts, septations and debris levels were observed. No clearly recognizable ovarian tissue has been reported in the majority of amputated ovaries. It is thought that the original ovaries were probably normal, pletely resorb or detach and become a loose calcified fi- and the cystic and calcified nature of the mass reprebrotic nodule which moves freely in the peritoneal cav- sented sequelae of necrosis and hemorrhage. It is not ity, often in the cul-de-sac (Fig.5) [19, 23]. Currarino possible to exclude the presence of an ovarian cyst or and Rutledge [19] reported that occasionally the ovary possibly a teratoma in the ovary prior to this torsion which became detached in the neonate resulted in a cys- and amputation [19, 23]. tic mass containing necrotic material and a solid, partially calcified mural node. The cysts were attached to Acknowledgement The authors thank Walter E. Berdon for his the omentum, the mesentery, or other intraperitoneal considerable editorial assistance with this paper.
Fig.4 Longitudinal US scan of the left ovary shows an enlarged

edematous ovary with hypoechoic rim. The mass was palpable and was accompanied by laboratory findings of an increased white blood cell count. The child was irritable and in pain Fig.5 CT scan of the pelvis for other reasons showed an incidental calcific density behind the left side of the bladder and anterior to the rectum. On exploration the left ovary was not present. The mass was diagnosed as a previously calcified, twisted and amputated left ovary

References
1.Kurjak A (1994) Ultrasound and the ovary. Parthenon, London, pp 7, 59, 3344 2. Cohen HL, Eisenberg P, Mandel F, Haller JO (1992) Ovarian cysts are common in premenarchal girls. A sonographic study of 101 children 2-12 years old. AJR 159: 89-91 3. Cohen HL, Shapiro MA, Mandel FS, Shapiro ML (1993) Normal ovaries in neonates and infants. A sonographic study of 77 patients 1 day-24 months old. AJR 160: 583-586 4. Brandt ML, Luks FI, Filiatrault D, Garel L, Desjardins JG, Youssef S (1991) Surgical indications in antenatally diagnosed ovarian cysts. J Pediatr Surg 26: 276-282 5.Montag TW, Auletta FW, Gibson M (1983) Neonatal ovarian cyst: prenatal diagnosis and analysis of the cyst fluid. Obstet Gynecol 61: 38-41 6. De Sa DJ (1975) Follicular ovarian cysts in stillbirths and neonates. Arch Dis Child 50: 45-50 7. Nussbaum AR, Sanders RC, Hartman DS, Dudgeon DL, Parmley TH (1988) Neonatal ovarian cysts: sonographicpathologic correlation. Radiology 168: 817-821 8. Garel L, Filiatrault D, Brandt M, Grignon A, Boisvert J, Perreault G, Patriquin H (1991) Antenatal diagnosis of ovarian cysts: natural history and therapeutic implications. Pediatr Radiol 21: 182-184 9.Nussbaum AR, Sanders RC, Benator RM, Haller JA, Dudgeon DL (1987) Spontaneous resolution of neonatal ovarian cysts. AJR 148: 175-176 10.Muller-Leisse C, Bick U, Paullussen K, Troger J, Zachariou Z, Holzgreve W, Schumacher R, Horvitz A (1992) Ovarian cysts in the fetus and neonate changes in sonographic pattern in their follow-up and management. Pediatr Radiol 22: 395-400 11. Jafri SZH, Bree RL, Silver TM, Ouimette M (1984) Fetal ovarian cysts: sonographic detection and association with hypothyroidism. Radiology 150: 809-812

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12.Suita S, Ikeda K, Koyanagi T, Nakano H (1991) Neonatal ovarian cyst diagnosed antenatally: report of two patients. J Clin Ultrasound 12: 517-519 13.Meizner J, Levy A, Katz M, Maresh AJ, Glezerman M (1991) Fetal ovarian cysts: prenatal ultrasonographic detection and postnatal evaluation and treatment. J Obstet Gynecol 164: 874-878 14.Bagolan P, Rivosecchi M, Giorlandino C, Bilancioni E, Nahom A, Zaccara A, Trucchi A, Ferro F (1992) Prenatal diagnosis and clinical outcome of ovarian cysts. J Pediatr Surg 27: 879-881 15.Stoupis C, Ros PR, Abbitt PL, Burton SS, Gauger J (1994) Bubbles in the belly: imaging of cystic, mesenteric or omental masses. Radiographics 14: 729737

16.Barr LL, Hayden CK Jr, Stansberry SD, Swischuk LE (1990) Enteric duplication cysts in children: are their ultrasonographic wall characteristics diagnostic? Pediatr Radiol 20: 326 17.Croitoru DP, Aaron LE, Laberge JM, Neilson JR, Guttman FM (1991) Management of complex ovarian cysts presenting in the first year of life. J Pediatr Surg 26: 1366-1368 18.Van der Zee DC, van Seumeren IGC, Bax KMA, Rovekamp MH, ter Gunne AJP (1995) Laparoscopic approach to surgical management of ovarian cysts in the newborn. J Pediatr Surg 30: 42-43 19.Currarino G, Rutledge JC (1989) Ovarian torsion and amputation resulting in partially calcified pedunculated cystic mass. Pediatr Radiol 19: 395-399

20.Alrabee A, Galliani CA, Giacomantino M, Heifetz SA, Lau H (1988) Neonatal ovarian torsion: report of three cases and a review of the literature. Pediatr Pathol 8: 143-149 21.Farrel TP, Boal DK, Teele RL, Ballantine TV (1982) Acute torsion of normal uterine adnexa in children: sonographic demonstration. AJR 139: 1223-1225 22.Worthington-Kirsch RL, Raptopolous V, Cohen IT (1986) Sequential bilateral torsion of normal ovaries in a child. J Ultrasound Med 5: 663-664 23.Kennedy LA, Pinckney LE, Currarino G, Votteler TP (1981) Amputated calcified ovaries in children. Radiology 141: 83-86

The European Society of Paediatric Radiology 6th Annual Course on Paediatric Radiology 19-23 October 1997 Liverpool, UK

Subject: Chest Radiology. Organisers: Prof. Helen Carty, RLC NHS Trust Alder Hey, Liverpool, UK. Dr. Don Shaw, The Hospital for Sick Children, Great Ormond Street, London, UK. The course will include lectures, film viewing and interactive teaching. There will be an emphasis on practical approaches and clinical and pathological correlation. Suitable for both radiologists and clinicians.