S. SchmahmannDepartment 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 pro-blems concerning the neonatalovary. Neonatal ovarian cysts mayundergo torsion and amputation.We discuss the pathogenesis, diag-nosis, and management of theseconditions.
We review the problems of the ovary in the normal neo-nate, neonatal ovarian cyst and neonatal ovarian tor-sion, with an emphasis on sonographic findings.
The normal neonatal ovary
Ovarian cysts have been found in the majority of neo-natal and infant ovaries [1-3]. The typical neonataland infant ovary is heterogeneous and cystic. In pa-tients 1 day to 3 months of age, cysts of less than 9 mmwere noted in 82 % and macrocysts (larger than 9 mm)were seen in 20 % of infants with cystic ovaries. Themean diameter of the largest cyst of each ovary was7.5 mm [3].
Follicle stimulating hormone (FSH), maternal estro-gens, and human chorionic gonadotropin (HCG) allcontribute to follicular growth in utero. The primarystimulus is FSH, which is secreted by the fetal pituitaryand which increases both the number and size of indi-vidual follicles. The hormonal balance present duringthe last months of fetal life contributes to further follicu-lar growth.
At birth, maternal estrogens and HCG levels fallwith the separation of the placenta from the neonate.FSH levels decline, the decline being slower in the pre-mature infant [3, 4].
Neonatal ovarian cysts: etiology
Neonatal ovarian cysts are primarily of follicularorigin and probably result from disordered folliculo-genesis [5]. Most of the cysts usually resolve spontane-ously.
De Sa [6], in an autopsy study, showed small follicu-lar cysts in 34 % of stillbirths and neonates within thefirst 28 days of life [1, 7].
Evidence suggests that excessive stimulation of thefetal ovary by both placental and maternal hormonesmay be a significant factor in cyst development [4, 5, 7].There is an increased incidence of cysts in infants ofmothers with diabetes, toxemia, or rhesus immuniza-tion, presumably from hypersecretion of placentalHCG or increased placental permeability to HCG [1,7-10]. Fetal ovarian cysts have been noted in neonates
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Fig.! Longitudinal US scan of the pelvis in the midline shows alarge simple cystic mass with one septation. The mass, an ovariancyst, occupies the entire abdominal cavityFig.2 a US scan of a fetal pelvis shows a right cystic mass in the re-gion of the neonatal ovary. b Postnatal US scan shows that thepreviously cystic ovary is now echogenic. The mass is approxi-mately the same size as it was originally; however, the echogenicmaterial in the mass suggests the diagnosis of torsion. A torsiveovary was found at surgery
with hypothyroidism as well [11]. (A similar associationoccurs in later childhood.)
Complications
The complications of ovarian cysts are divided into pri-mary, secondary, and maternal sequelae [1, 12, 13].
Torsion and hemorrhage are the most frequent pri-mary complications. Torsion is more common in largecysts, but has been reported in cysts as small as 2 cm [1,4, 7, 10]. Large studies report that torsion occurred in42 % of patients [7, 10].
Torsions, many of which occur antenatally [4, 7], maybe accompanied by pain, vomiting, fever, abdominaldistension, leukocytosis and peritonitis. However, manycases are asymptomatic [7, 10].
Hemorrhagic cysts almost uniformly result from tor-sion and associated infarction. A potentially lethal, butrare complication, is cyst rupture causing hemorrhagicascites, and/or peritonitis [1, 7]. Secondary complica-tions from large cysts include bowel obstruction, thorax
compression (with pulmonary hypoplasia), urinary tractobstruction or incarceration within an inguinal hernia[1, 4, 7, 9]. Polyhydramnios (5 %-12 %) and vaginal dys-tocia with cyst rupture are rare maternal complicationsof large fetal ovarian cysts [1, 7].
Sonographic appearance
Cysts vary in size and appearance. The larger cysts mayoccupy almost the entire abdomen (Fig. 1) [13, 14]. Theappearance of a cyst varies, depending on whether thecyst is uncomplicated or complicated by torsion or hem-orrhage. An uncomplicated cyst is anechoic with animperceptible wall. A complicated cyst invariably con-tains a fluid-debris level, a retracting clot, septa, or iscompletely filled with echoes producing a solid mass-like appearance (Fig. 2). These complex cysts oftenhave echogenic walls resulting from dystrophic calcifica-tion associated with infarction [7, 10].
In the study of Nussbaum et al. [7], a fluid-debrislevel and retracting clot were found to be specific signs
Fig.3 a Longitudinal US scanof the pelvis shows a leftadnexal mass with cystic andsolid components. b After2 weeks the mass had decreasedin size and appeared as a simplecyst, the diagnosis of hemor-rhagic ovarian cyst was made
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of torsion which had occurred before birth in the major-ity of cases. Muller-Leisse et al. [10] found uncompli-cated cysts developed intraluminal echoes of varyingmorphology postnatally, which the authors attributedto mechanical stress of delivery and not to torsion. Nuss-baum et al. [7] also found that the ovary contralateral toan ovarian cyst is frequently multicystic.
Differential diagnosis
It may be impossible to differentiate an ovarian cystfrom a mesenteric or enteric cyst [6, 7, 10, 15]. Entericduplication cysts have been described by Barr et al.[16] as having a hyperechoic mucosal layer with an un-derlying hypoechoic layer in all cases (thick wall sign).A similar image can occasionally be found with ovariancysts as well.
Other cystic structures which may be confused withovarian cysts are hydrometrocolpos, cystic meconiumperitonitis, urachal cyst, bowel atresia or obstruction, re-nal cysts and anterior meningocele [1, 7, 8, 10, 15, 17].
Complex cysts, especially those presenting as a solidmass or a cystic mass with a retracting clot, may be im-possible to differentiate from a neoplasm (Fig. 3). Ova-rian neonatal neoplasms are extremely rare, andinclude cystadenomas, cystic teratomas, and granulosacell tumors [4, 7, 8, 17].
Management
The management options for treating a neonatal ova-rian cyst depend largely upon cyst size and sonographiccharacteristics. Another factor is the potential risk ofcomplications. Rarely, there is a need for differentiationof ovarian cysts from other ovarian tumors and fromother intraperitoneal cystic masses [4, 8].
After birth, the decrease in hormonal stimulation isassociated with spontaneous regression of most smallcysts. Simple ovarian cysts of less than 4 cm in diametercan be observed using serial ultrasonography. Most cystswill resolve in 3-4 months, but longer periods of obser-vation may be necessary if the cyst is decreasing in size[4, 8].
The treatment of larger cysts and complex cysts re-mains controversial [4, 7-9, 17]. Cysts larger than 4 cmare associated with a greater risk of torsion. Some sur-geons feel that because ovarian torsion may lead to lossof the ovary, early intervention is required. The manage-ment options include surgery, laparoscopic needle aspi-ration, or laparoscopy. Others, however, argue thatsince surgery has nothing to offer when the ovary is tor-sive (and nonviable), observation may be the preferredmanagement choice. When surgery is performed, it isimportant to preserve as much gonadal tissue as possi-ble and if practical merely to remove or unroof thecyst. Ovarian cysts are often adherent to the ovary andfrequently oophorectomy cannot be avoided [4, 8]. Ul-trasound-guided needle aspiration of the cyst has no re-ported complications and offers excellent possibilitieswith respect to preservation of the ovary [4].
A successful laparoscopic approach to the manage-ment of ovarian cysts has been reported. This techniqueappears to be promising and offers both diagnostic ca-pabilities (visualization and biopsy) as well as a smoothtransition to therapeutic measures when necessary.Cysts can be decapsulated by use of a laser or surgicalincision, thus saving the ovary [18].
Torsion of the normal ovary and tube can occur atany age although it is rare in the infant (Fig.4) [19, 20].The normal adnexa in girls may be very mobile, allow-ing torsion at the mesosalpinx with changes in intra-ab-dominal pressure or body position [21, 22].
The ovary and fallopian tube undergo necrosis andamputation. The amputated ovary may either corn-
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Fig.4 Longitudinal US scan of the left ovary shows an enlargededematous ovary with hypoechoic rim. The mass was palpableand was accompanied by laboratory findings of an increased whiteblood cell count. The child was irritable and in pain
Fig.5 CT scan of the pelvis for other reasons showed an incidentalcalcific density behind the left side of the bladder and anterior tothe rectum. On exploration the left ovary was not present. Themass was diagnosed as a previously calcified, twisted and ampu-tated left ovary
pletely resorb or detach and become a loose calcified fi-brotic nodule which moves freely in the peritoneal cav-ity, often in the cul-de-sac (Fig.5) [19, 23]. Currarinoand Rutledge [19] reported that occasionally the ovarywhich became detached in the neonate resulted in a cys-tic mass containing necrotic material and a solid, par-tially calcified mural node. The cysts were attached tothe omentum, the mesentery, or other intraperitoneal
organs by a long, twisted pedicle containing thin-walledvascular spaces.
In the older child and adolescent the masses were ad-nexal, while in the neonate and infant they were ex-trapelvic. In all neonates and several infants' complexmasses with multiple large cysts, septations and debrislevels were observed.
No clearly recognizable ovarian tissue has been re-ported in the majority of amputated ovaries. It isthought that the original ovaries were probably normal,and the cystic and calcified nature of the mass repre-sented sequelae of necrosis and hemorrhage. It is notpossible to exclude the presence of an ovarian cyst orpossibly a teratoma in the ovary prior to this torsionand amputation [19, 23].
Acknowledgement The authors thank Walter E. Berdon for hisconsiderable editorial assistance with this paper.
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The European Society of Paediatric Radiology6th Annual Course on Paediatric Radiology19-23 October 1997Liverpool, UK
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