Archives of Disease in Childhood, 1988, 63, 737-742

Neonatal ovarian cysts: therapeutic dilemmaD J WIDDOWSON,* D W PILLING,* AND R C M COOKt

Department of "Radiology and tPaediatric Surgery, Royal Liverpool Children's Hospital, Liverpool

SUMMARY Seven cases of neonatal ovarian cysts that presented over the past seven years werestudied. Complications included torsion and rupture and usually occurred in cysts more than 5 cmin diameter. Surgical removal, either oophorectomy or cystectomy, was the treatment of choice.Because even cystectomy results in loss of normal ovarian tissue, and because spontaneousregression of cysts less than 5 cm in diameter can occur, a more conservative approach is nowproposed. Regular ultrasonography alone is recommended if the cysts are less than 5 cm indiameter, and aspiration of the cysts followed by regular ultrasonographs if the cysts are morethan 5 cm in diameter. Operation should be reserved for recurrent cysts or for those withcomplications. Cysts diagnosed antenatally may be aspirated in utero if there are signs of thoraciccompression.

Neonatal ovarian cysts are being diagnosed moreoften now that routine ultrasonography is carriedout antenatally and postnatally. A truly cysticabdominal mass in a baby girl is most likely to be anovarian cyst, although duplication cyst or mesentericcyst should be considered in the differential di-agnosis.There is controversy about the best treatment for

these cysts, opinions ranging from oophorectomy tofollow up by ultrasonography alone. We propose aregimen the main objective of which is to avoidunnecessary operation especially in those patientswith bilateral cysts.

Case reports

CASE IThe infant was born prematurely by emergencycaesarean section at 33 weeks' gestation weighing2700 g. The mother had insulin dependent diabetesand had been treated for thyrotoxicosis by thy-roidectomy six years previously. She had also hadone spontaneous abortion and two stillbirths.A routine antenatal ultrasound scan at 32 weeks

showed a cystic mass in the baby's abdomen. Thiswas confirmed when she was 2 weeks old as a cysticabdominal mass measuring 4x3 cm. At laparotomyon the following day a cyst arising from the rightovary was enucleated. The uterus and left ovarywere normal. On histological examination the cystwas found to be a simple follicular cyst withevidence of luteinisation. The patient made anuneventful recovery.

CASE 2

The infant was born by normal spontaneous vaginaldelivery at 35 weeks' gestation weighing 3200 g. Themother had had two spontaneous abortions and twopregnancies terminated; she was also a heroinaddict. Nine days before delivery two cystic centralabdominal masses that were separate from thekidneys were noted on ultrasound scan (fig la). Ascan performed when she was one day old (fig lb)confirmed the presence of cystic structures measur-ing 8x4 cm and 5X4 cm diameter, respectively. Atoperation bilateral ovarian cysts were found andbilateral cystectomies performed. Both cysts wereunilocular and contained clear yellow fluid. Onhistological examination the cyst walls were foundto be thin and contained some ovarian tissue with afew follicular cysts. The patient made a satisfactoryrecovery and was discharged home three weeks afterthe operation.

CASE 3The infant was born by normal vaginal delivery atfull term weighing 3300 g. The mother was well. Anultrasound examination was performed at 38 weeks'gestation for a possible abruptio placentae andshowed a cystic lesion 6 cm in diameter in the rightside of the baby's abdomen. This was confirmed by ascan performed when she was 1 day old, and thelesion contained a few fine septa. At laparotomy onday 8 a mobile, right sided ovarian cyst was found,which had twisted twice at the junction of the rightfallopian tube and the uterus. A right salpingo-oophorectomy was performed. The left ovary was

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Fig l a Transversescanoffetalabdomen (case2)showingtwo cystic masses (c); (s) =spine.Fig lb Postnatal oblique lower abdominal scan (case 2)confirming the presence of cysts (c) separate frombladder (b).

normal and contained a few small follicular cysts.An infarcted cyst was confirmed on histologicalexamination. The cyst was thickened (1 cm) inplaces and contained dark brown fluid. The patientwas discharged on the sixth day postoperativelyafter an uneventful recovery.

CASE 4The infant was born by spontaneous vaginal deliveryat 27 weeks' gestation weighing 200() g. She hadmany problems in the immediate neonatal periodincluding respiratory distress syndrome that re-quired ventilation, a grade II intraventricularhaemorrhage on the left, jaundice, and haemolyticanaemia that required two transfusions. No causewas found for the anaemia but during investigationan ultrasound examination was performed to ex-clude polysplenia. The spleen was normal but acystic lesion in the abdomen was found. A lapar-otomy was performed at 18 weeks at which a twistedcyst of the right ovary 5 cm in diameter was foundtogether with a cyst of the left ovary 2-5 cmin diameter. Right salpingo-oophorectomy anddrainage of the left cyst with biopsy were carriedout.

On histological examination the specimen fromthe left cyst showed normal ovarian tissue whereasthe right cyst was totally infarcted with areas ofcalcification within it and only a simple flattenedepithelial lining; it contained brown necroticmaterial. The patient was discharged home 13 dayspostoperatively.

CASE 5The infant was born by normal vaginal delivery atfull term weighing 3800 g after an uneventfulpregnancy. The mother was well. At the age of 5months the baby was admitted to hospital withabdominal distension and a three month history ofintermittent abdominal swelling that had causeddyspnoea at night. Ultrasound examination showeda unilocular cyst 10 cm in diameter in the centre ofthe abdomen, separate from the kidneys and thebladder. At laparotomy a large left sided ovariancyst was found that had twisted two and a half timesround the left fallopian tube. A cystectomy wasperformed, and an ovarian remnant and the leftfallopian tube were left behind. The right adnexawas normal. On histological examination the cystcontained dysplastic ovarian parenchyma with a few

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Neonatal ovarian cysts: therapeutic dilemma 739

oogonia and an occasional primary graafian follicle.The patient was discharged home six days post-operatively.

CASE 6The infant was born by normal vaginal delivery atfull term weighing 3300 g. The mother was well. Aroutine antenatal ultrasound scan at 32 weeks'gestation showed a cystic mass 4x3 cm in diameterin the left side of the abdomen separate from thekidneys. This was confirmed when she was 5 weeksold, but this time the lesion lay on the right side ofthe abdomen. The mass was not palpable. By 25weeks of age the mass had become palpable andrepeat ultrasound examination confirmed an in-crease in its size to 6x5 cm. A large cyst of the leftovary was removed at laparotomy. The right ovarywas normal. She also had a malrotation of the boweland this was corrected. On histological examinationthe ovarian mass was found to be a simple follicularcyst. The patient made an uneventful recovery.

CASE 7The infant was born by normal vaginal delivery atfull term weighing 3100 g. The mother was well, butthe pregnancy was complicated by hydramnios. Aroutine antenatal ultrasound scan at 34 weeks'gestation showed a cystic mass 7x5 cm in diameterin the abdomen (fig 2). This was confirmed whenshe was 1 day old, the mass having decreased in sizeto 6x4 cm. The patient was otherwise normal, inparticular there was no evidence of gut atresia toaccount for the hydramnios. A scan two weeks latersuggested that the mass was multilocular. At lapar-otomy bilateral ovarian cysts were found, the leftbeing about twice the size of the right. The right cystwas chronically twisted and a right salpingo-oophorectomy was performed, together with a leftcystectomy. On histological examination the rightcyst was found to be multilocular and follicular, andthe left unilocular and follicular. There were nopostoperative complications and the patient wasdischarged home three days later.

Discussion

Before the introduction of ultrasonography, ovariancysts in neonates were thought to be rare and couldonly be diagnosed postnatally. 1-3 Only 71 cases werereported before 1976.4 Cysts were only discovered ifthey were palpable or became symptomatic, but,with ultrasonography asymptomatic ovarian cystscould be diagnosed.5 6 Ovarian cysts can now bediagnosed antenatally, and 11 such cases hadbeen reported up to 1987.7 x The pathogenesis ofneonatal ovarian cysts is unknown. Examination of

Fig 2 Transverse section offetal abdomen (case 7). Largeabdominal cyst (c) is shown separate from kidneys(k) and spine (s).

the ovaries of 121 children at necropsy showed thatthere was more pronounced luteinisation of thetheca interna in the neonatal ovaries studied whencompared with the older age groups9; this wassubsequently confirmed by a study of normalneonatal and infantile ovaries."' Another series of332 necropsies of stillbirths and neonatal deathsshowed that 34% had small follicular cysts within 28days of birth." It is almost certain, therefore, thatthe stimulus for the formation of neonatal ovariancysts is chorionic gonadotrophin that stimulates thefetal ovary during pregnancy. Luteinising cysts areoccurring increasingly more often in babies whosemothers were diabetic (case 1) or had toxaemia, ormaternal isoimmunisation, because these conditionsare all associated with raised concentrations ofhuman chorionic gonadotrophin.'2 '3 There is alsoan increasing incidence in premature infants, pro-bably because of their greater sensitivity to humanchorionic gonadotrophin (cases 1, 2, and 4).14 On

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histological examination the cysts are follicular orluteinising cysts. Because human chorionic gonado-trophin is important in the pathogenesis of neonatalovarian cysts, it is logical to expect the cysts toregress spontaneously in the neonatal period as thehormone concentrations fall and the stimulus forgrowth disappears. This often seems to happenunless some complication supervenes. 12 All thereported cases of spontaneous resolution haveoccurred within four months of birth.

Both sides seem to be affected with equalfrequency, also a finding in our series. Bilateral cystswere said to be rare, only three cases having beendescribed by 1974, 12 though the incidence hasincreased since the introduction of ultrasonography;three of our cases had bilateral cysts.

Malignant change in neonatal ovarian cysts thatare simple fluid filled structures is extremely rare,and is usually seen only in more complex lesions.1Other complications, however, are common. Mostcommonly reported complications of untreatedovarian cysts are torsion of the pedicle (cases 3, 4, 5,and 7), haemorrhage into the cyst or abdominalcavity, and rupture of the cyst.' 3 5 6The cysts may be of any size, the largest re-

ported being over 20 cm in diameter. The largercysts are associated with other complications, in-cluding intestinal obstruction with one reported caseof caecal perforation in an infant,1 6 and hydramnios.One of our cases was associated with hydramnios(case 7) and another probably had intermittentintestinal obstruction (case 5). Hydramnios hasbeen reported in 5 to 10% of pregnancies in which afetal ovarian cyst was subsequently diag-nosed. 12 17 18 19 This is probably caused by pressureof the mass on the small bowel together with inter-ference with the swallowing mechanism of the fetus,which reduces ingestion and absorption of amnioticfluid.

There is a risk of pulmonary hypoplasia develop-ing in fetuses with large cysts.7 Pulmonary hypo-plasia is associated with other lesions which com-press and reduce the intrathoracic space (forexample, large abdominal fluid collections andmasses, and diaphragmatic hernias and eventration).Though there have been no definite cases reportedof pulmonary hypoplasia developing seconidary toan ovarian cyst, the risk exists if the cyst is largeenough to cause thoracic compression as with anyother large abdominal mass.Nowadays the diagnosis of ovarian cysts is by

ultrasound scan and may be made antenatally orpostnatally. Mesenteric and duplication cysts mtay,however, be ultrasonically indistinguishable fromovarian cysts." Most neonaital ovarian cysts areasymptomatic and found incidentally. Some of the

cysts are extremely mobile and appear as a 'wander-ing' tumour (as seen in cases 3 and 6.)5The treatment of these ovarian cysts is controver-

sial. Until recently the recommended treatment wassurgical. If the cyst was unilateral or if there hadbeen torsion, oophorectomy was performed. Therationale for this when a simple unilateral cyst waspresent was the risk of possible torsion, or thereplacement of the entire ovary by cyst makingcystectomy impossible, or the inability to distinguishcyst from remaining normal but distorted ovariantissue on histological examination.3 4 6 More re-cently simple cystectomy was recommended butwhether any function remained in the ovary isunknown." The cystectomy specimens in our seriesall contained normal ovarian tissue, thus supportingthe above findings. This is not of any greatimportance if there is a normal ovary on the otherside.

Problems arise, however, when the cysts arebilateral. Oophorectomy on one side and cystec-tomy on the other have been done, as have bilateralcystectomies. These operations carry a high risk ofremoving all normal ovarian tissue (despite attemptsto preserve some of each ovary), thereby renderingthe patient sterile.

All the cases in our series had either cystectomyor salpingo-oophorectomy. We therefore decided tosee whether alternative and more conservativetreatment had been reported, and, indeed surgicalconservatism had been advocated for some time andthe advent of cyst aspiration was predicted in 1972.3

In a recent paper, Nussbaum et al described threecases in which cysts had resolved spontaneouslywithin four months of discovery.8 All were less than5 cm in diameter anid were followed up by serialultrasound scans. Though there is a potential risk oftorsion, only two cases have been reported in cystsof less than 5 cm in diameter. 6 In both cases thetorsion was detected on the ultrasound scan becausethe previously echo free cyst developed fluid anddebris levels within it. Both cases underwentoperations, though it is not clear whether there is arisk in leaving a twisted, infarcted cyst untreated.Rupture of the cyst is rare and has only beenreported in larger lesions (10 to 12 cm in diameter);it would not therefore seem to be a risk factor inlesions less than 5 cm in diameter.'1

Lesions more than 5 cm in diameter present agreater problem as there is an increased risk ofcomplications if they are left untreated. In view ofthe natural tendency of these cysts to regressspontaneously we suggest that cyst puncture shouldbe performed, and the patient followed up withserial ultrasound scans. Recurrence of the cyst couldbe treated by repeated aspiration, surgical removal

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being reserved for the few intractable or compli-cated cases. There have been two recent casereports of intrauterine ovarian cyst aspiration inwhich there was thought to be an appreciablerisk of pulmonary hypoplasia secondary to apronounced degree of thoracic compression.7 Themost important risk associated with cyst puncture iscyst rupture leading to peritonitis. This is, however,uncommon. 20

Oestradiol concentrations should be measured inaspirated fluid to confirm its ovarian origin,21 thoughhigh levels of oestrogen are found in the normalneonatal circulation.22 This oestrogen, the exactstructure of which is unknown, is probably manu-factured and secreted by the fetal adrenal glands andmay interfere with the oestradiol estimation by someof the direct assay kits used in some laboratories.Spuriously high levels of oestradiol may, therefore,be detected in the asipirate if the sample is conta-minated with blood. This should be borne in mind if

Neonatal ovarian cysts: theralpetic dilemmittia 741

the laboratory uses these kits.23 A high oestradiolconcentration in clear aspirate from a cyst indicateswith certainty that it is of ovarian origin.A conservation approach to this problem should

therefore be attempted before embarking on surgi-cal treatment. The advantages of this are firstly, thatthe patient may require no treatment if the cystregresses spontaneously on serial ultrasound scans.Secondly, if cyst aspiration is effective then thepatient is spared an operation, and thirdly perhapsmost importantly if the cysts are bilateral theconservative approach prevents the possible re-moval of all normal ovarian tissue. We thereforepropose therapeutic regimens for the treatment ofuncomplicated neonatal ovarian cysts (tables 1 and2). Complicated cysts should be treated with asconservative an operation as possible.

Wc thank Mr RE Cudmorc for pcrimiission to rcport somc of thecascs that were under his crae and Mrs J Scott fot- tvping theimianuscript.

Table 1 Regimen for anternatal diagnosis of uncomplicated neon(atal ovariati cYsts

Rcsolution

No cvidcncc of thoracic comprcssion o Scrial ultrasound scans

Rccuri-cnccEvidcncc of thoracic comprcssion - Intrauterine aspiration of cvst

Rcpcat aispiriationor opcration('? cystcctoni)

Table 2 Regimen for postiatal diagnosis of uncomplicated mieonatal C)variani cysts

Cyst <5 cm indiamctcr

Scrial ultrasoundscans7~~~~~~~~~~~~I

Spontancousrcsolution

Cyst 5 cim or mtiorcin dialmtccr

No resolution orincrcasc in sizCwithin six months

I lb Aspiration'of cyst

Scriadl Ultilasotind scans

Rcsolution Rcuirt-cncc

Rcpcat aspii ationor opcration (? cystectonmv)

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References

Marshall JR. Ovarian enlargements in the first year of life:Review of 45 cases. Annii Surg 1965;161:372-7.

2 Ahmed S. Neonatal and childhood ovarian cysts. J Pediatr Surg1971 ;6:7t)2-8.

3 Carlson DH, Griscon NT. Ovarian cysts in the newborn.American Journal of Roentgenology, Radiumn Therapy andNuclear Medicine 1972; 1 16:664-72.

4 Alvear DT, Rayfield MM. Bilateral ovarian cysts in earlyinfancy. J Pediatr Surg 1976;11:993-5.

5 Avni EF, Godart S, Israel C, Schmitz C. Ovarian torsion cystpresenting as a wandering tumor in a newborn: antenataldiagnosis and postnatal assessment. Pediatr Radiol 1983;13:169-71.

6 Montagne JPH. Neuenschwander S, Prot D, Cordier MD.Solitary intraperitoneal cystic lesions in newborn girls: ten casesstudied by ultrasound. Ann Radiol 1982;25:131-5.

7 Landrum B, Ogburn PL, Feinberg S, et al. Intrauterineaspiration of a large fetal ovarian cyst. Obstet Gynecol 1986;68(suppl 3):11-14.

8 Nussbaum AR, Sanders RC, Benator RM, Hallcr JA, Dud-geon DL. Spontaneous resolution of neonatal ovarian cysts.AJR 1987;148:175-6.

9 Kraus FT, Neubecker RD. Luteinisation of the ovarian theca ininfants and children. Am J Clin Pathol 1962;37:389-97.Curtis EM. Normal ovarian histology in infancy and childhood.Obstet Gynecol 1962;19:444-54.De Sa DJ. Follicular ovarian cysts in stillbirths and neonates.Arch Dis Child 1975;50:45-50.

12 Bower R, Dehner LP, Ternber JL. Bilateral ovarian cysts in thenewborn. A triad of neonatal abdominal masses, polyhydram-nios and maternal diabetes. Am J Dis Child 1974;128:731-3.

' 3Ahlvis RC, Bauer WC. Lutcinised cysts in the ovaries of infaintshorn of diabetic ilmothers. Amn J Dis Childl 1957;93:107-9.

14 Sedin G, Berquist C, Lindren PG, Ovarian hyperstimulationsyndrome in preterm infants. Pedit- Res 198-5;19:548-52.

5 Manson R, Rodgers BM. Nelson RM, Young TK. Rupturedovarian cyst in a new born infant. J Pedialr 1978X;73:324-5.

16 Schotz PM, Key L, Filston HC. Large ovarian cyst causing cecalperforation in a new horn infanit. J Pediatr Soirg 1982;17:91-2.

17 Kramer EE. HIydramnios, oligohydramnios and fctal malforma-tions. Clin Obstet Gvnecol 1966;9:51)8-19.

18 Nacye RL, Milic AMB, Blanc W. Fetal endocrine and renaldisorders. Clues to the origin of polyhydramnios. Amn J ObstetGvnyecol 1970,108:1251-6.

19 Ncikirk WI, Hudson HW Jr. Giant ovarian cyst in newborninfant. N Enigl J Med 1948;239:468-9.Valcnti C, Kaissner EG, Yermakov et al. Antenatal diagnosis offetal ovarian cyst. Ain J Obstet Gvnecol 1975:123:216-9.

21 Montag TW, Auletta FJ, Gibson M. Neonatal ovarian cyst:Prenatal diagnosis and analysis of the cyst fluid. Obstet Gynecol1983;61(suppl 3):385-415.

22 Winter JSD, Hughes IA, Reycs Fl et al. Pituitary-gonadrelationships in infancy: 2. Patterns of serum gonadial steroidconcentrations in man from birth to 2 years of age. J CliniEnidocrioiol Metab 1976;42:679-85.

23 Diver MJ, Nisbet JA. Warning on plasma oestradiol measurc-ment. Lancet 1987;ii:1097.

Correspondence to Dr DW Pilling, Department of Radiology,Royal Liverpool Children's Hospital. Aldcr Hey, Eaton Road,Liverpool L12 2AP.

Accepted 28 January 1988

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