Original Study. Hemorrhagic Corpus Luteum Cysts: An Unusual Problem for Pediatric Surgeons

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1 J Pediatr Adolesc Gynecol (2009) 22:163e167 Original Study Hemorrhagic Corpus Luteum Cysts: An Unusual Problem for Pediatric Surgeons C. Spinelli, MD 1, M. Di Giacomo, MD 1,N.Mucci,MD 1, and F. Massart, MD, PhD 2 1 Chair of Pediatric Surgery, Department of Surgery; 2 Pediatric Endocrine Center, Department of Pediatrics, University of Pisa, Pisa, Italy Abstract. Study Objective: Hemorrhagic corpus luteum cysts (HCLC) constitute a common disorder in pediatric subjects undergoing surgical intervention. HCLCs especially develop in the early period after menarche, and they are commonly associated with dysfunctional ovulation. Design: Retrospective analysis of surgery outcome of HCLC patients. Setting: Pediatric Surgery Unit, S. Chiara University Hospital. Participant: 13 girls with HCLC diagnosis. Interventions: Surgical treatment of HCLCs. Main Outcome Measures: We reviewed the clinical presentation and outcome of 13 post-menarcheal girls surgically treated for HCLCs in the Pediatric Surgical Unit from 2002 to Results: Primary presentation was persistent abdominal pain in 84.6% and acute abdominal pain in 15.4% of patients, respectively. Ultrasound examination showed complex ovarian masses in 77.23% cases and simple ovarian masses in 33.7% cases, respectively. Although laparoscopic excision of HCLC was performed in more than 45% cases, laparotomic approach was commonly required. After conservative surgery, ovarian size and viability were normal, as assessed by 6-month ultrasound scan. No recurrences of disease and regular menses were reported at 2 years follow-up. Conclusions: In pediatric subjects with HCLC that required surgical intervention, no complications or disorder recurrence were reported. In order to preserve ovarian function, conservative surgery has to be performed whenever feasible. Key Words. Hemorrhagic corpus luteum cysts Ovarian surgery Tumor markers Pediatric gynecology Pediatric surgery Address correspondence to: Prof. Claudio Spinelli, MD, Chair of Pediatric Surgery, Department of Surgery of the University of Pisa, Via Roma 67, Pisa, Italy; c.spinelli@dc.med.unipi.it Ó 2009 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc. Introduction Clinical presentation and outcome of ovarian disorders differ from those in adults requiring different diagnostic and management approaches. 1,2 In particular, hemorrhagic corpus luteum cysts (HCLC) are usually unilocular and lined by luteinized theca and granulosa cells. 3 Although hemorrhagic lesions can occur at any time of life, HCLCs develop during puberty especially in the early period after menarche, and they are commonly associated with dysfunctional ovulation. 3e5 Patients with these cysts may present with abdominal pain or signs of peritoneal irritation that can be confused with acute appendicitis. 3,6 After thorough diagnostic work-up, it is compulsory to decide the appropriate management for these patients. The HCLC spontaneously tend to regress on follow-up ultrasound few weeks later, and only a minority cause complications such as intracystic hemorrhage, rupture, and adnexal torsion. 3,4,7,8 The HCLC treatment depends on the symptoms and the size of the cyst; when surgery is necessary, enucleation is performed whenever possible in order to conserve ovarian tissue. 8 In fact ovarian surgery during childhood and adolescence could result in compromising future fertility, either from removal of normal ovarian tissue or from formation of adhesions. 1 This study was undertaken to gain more knowledge on the HCLC in the pediatric age and to optimize their diagnostic and therapeutic management. Patients and Methods In the present study, we retrospectively reviewed pediatric subjects who underwent surgical intervention in Pediatric Surgical Unit from January 1, 2002, to August 31, After reviewing the pathology reports, the medical records of the patients were retrospectively reviewed. Specific data collected on each patient included clinical presentation, age at /09/$36.00 doi: /j.jpag

2 164 Spinelli et al: Hemorrhagic Corpus Luteum Cysts operation, imaging studies (ultrasonography (US), computerized tomography (CT) and magnetic resonance imaging (MRI) scans), serum data (CA-125, a-fetoprotein and b-human chorionic gonadotropin), surgical route and outcome. The data have been reported as mean ( SD). After the local Institutional Review Board approval, the informed consent was obtained from all parents prior to the study. Results In the 5-year period, 67 patients were surgically evaluated for ovarian lesions (e.g. mature cystic teratoma, cystoadenoma, and yolk-sac tumor). Of them, 13 (19.4%) girls were affected by HCLC (Table 1). At first surgery evaluation, they were 17.2 (1.6) years (range 13e18 years) with menarcheal age of 12.3 ( 1.7) years. Four patients (30.8%) presented with a personal history of disruption of the menstrual cycle or dysmenorrhea. One patient presented with a family history of HCLCs: her mother was surgically treated for the same disease. No patients presented with clinical characteristics of precocious puberty, abnormal skin pigmentations, or other known syndrome markers at physical examination. Height, weight, and body mass index were 0.7 ( 0.41), 0.6 ( 0.28) and 0.4 ( 0.26) SDS, respectively. Abdominal US was performed as the first diagnostic test for all patients to characterize ovarian diseases as simple, complex, 9 or solid lesions. US examination showed unilateral HCLCs in all our patients: complex ovarian masses in 10 (77.0%) and simple ovarian masses in 3 cases (23.0%), respectively. An additional abdominal CT scan was also performed in 7 patients (53.8%) to better define the complex lesions, such as to exclude associated pathologies (Fig. 1). Serum levels of tumor markers CA-125, a-fetoprotein, and b-human chorionic gonadotropin were within in our normal ranges for all cases, respectively. The primary presentations that led to operation were persistent abdominal pain in 11 patients (84.6%) and acute abdominal pain in 2 patient (15.4 %), respectively. Eleven patients (84.6%) were surgically treated after an ongoing observation of weeks (range 4e8 weeks), with several US scans. Two patients (15.4%) were treated urgently, one with a cyst complicated by ovarian torsion and the other with a suspected rupture of the lesion, in whom we found intrapelvic blood at the surgery view (Fig. 2A, B). Six patients (46.2%) underwent operative laparoscopy: excision of the luteal cyst was performed in all cases. Seven patients (53.8%) underwent laparotomy: simple resection of the luteal cyst in 2 cases, oophorectomy in 2 cases, and salpingo-oophorectomy in one case. Incidental appendectomy was performed Table 1. Presentation of patients with hemorrhagic corpus luteum cysts Patient Age Ovary Symptoms US findings CT findings Pathologic findings Surgical approach Surgical procedure 1 13 RT Acute abdominal pain Complex Complex Hemorrhagic corpus luteum cyst Complicated by ovarian torsion Open Oophorectomy 2 14 LTRT Acute abdominal pain Complex Complex Hemorrhagic corpus luteum cyst With intrapelvic blood Open Enucleation 3 17 RT Persistent abdominal pain Complex - Hemorrhagic corpus luteum cyst Laparoscopy Enucleation 4 18 RT Persistent abdominal pain Complex Complex Hemorrhagic corpus luteum cyst Open Oophorectomy 5 18 RT Persistent abdominal pain Simple - Hemorrhagic corpus luteum cyst Laparoscopy Enucleation 6 18 RT Persistent abdominal pain Simple - Hemorrhagic corpus luteum cyst Laparoscopy Enucleation 7 18 RT Persistent abdominal pain Complex Complex Hemorrhagic corpus luteum cyst Open Ovariosal pingectomy 8 18 RT Persistent abdominal pain Complex Complex Hemorrhagic corpus luteum cyst Open Ovariosal pingectomy 9 18 RT Persistent abdominal pain Complex Mixed Hemorrhagic corpus luteum cyst Open Ovariosal pingectomy LT Persistent abdominal pain Complex - Hemorrhagic corpus luteum cyst Laparoscopy Enucleation LT Persistent abdominal pain Complex - Hemorrhagic corpus luteum cyst Open Enucleation RT Persistent abdominal pain Simple Simple Hemorrhagic corpus luteum cyst Laparoscopy Enucleation LT Persistent abdominal pain Complex - Hemorrhagic corpus luteum cyst Serous cystoadenoma Laparoscopy Enucleation

3 Spinelli et al: Hemorrhagic Corpus Luteum Cysts 165 rupture, and concern about the malignant potential of the lesions. The histology of the ovarian lesions revealed the following: HCLC lesion was detected in the right ovary in 8 patients (61.5%) and in the left in 5 patients (38.5%); one girl also had an ipsilateral serous cystoadenoma another girl also had a contralateral paraovarian cyst. After conservative surgery, ovarian size and viability, as assessed by 6-month ultrasound scan, has been normal. No recurrences of disease and regular menstrual cycles of 28e31 days were reported after months (range 9e53 months) of follow-up. Fig. 1. CT: Hemorrhagic corpus luteum cyst. in 5 patients (38.5%) with no associated complications. The decision of a laparoscopic or laparotomic surgical approach was based on the combination of two different parameters: the size of the lesion and its echographical aspects. Bigger lesions, especially if associated with a complex US aspect, have been treated with laparotomy due to technical difficulties, the higher risk of cyst Fig. 2. Ultrasonography (A) and laparotomy (B): hemorrhagic corpus luteum cyst complicated by ovarian torsion. Discussion Pediatric ovarian lesions, although rare, span a spectrum of pathology from functional nonneoplastic ovarian cysts to ovarian torsion, and from benign tumors to advanced neoplasms. 1,2,6,10 Functional ovarian cysts constitute from 17.1% to 43.6% (mean ) of all surgically treated ovarian lesions in pediatric age; corpus luteum cysts constitute from 14.2% to 26.4% (mean ) (Table 2). 1,2,6,11,12 The early period following menarche is commonly associated with dysfunctional ovulation, so ovulatory cysts are often seen as the result of aborted ovulations or persistence of the corpus luteum. 3e5 Ovarian cysts in teenage years, as we have seen, may be asymptomatic or associated with menstrual irregularities, abdominal pain, urinary frequency, constipation, or pelvic discomfort. 4,13,14. In adolescents, it is essential to elicit a full history including details of the menstrual and sexual history. 4,14 The differential diagnosis widens to include a variety of acquired reproductive disorders such as pregnancy, sequelae of sexually transmitted diseases or endometriosis. 13,14 The preoperative diagnostic work-up of ovarian pathologies in adolescents includes US scan and blood samples for tumor markers. 8,14 The diagnosis is greatly aided by the use of imaging; the widespread availability and use of US has resulted in higher detection rate of functional cysts. 13,14 US is non-invasive and has proven to be very valuable not only in diagnosis, but also in allowing observation of these lesions over time. Hemorrhage in a functional cyst can lead to a diagnostic dilemma; in fact it may appear ultrasonographically complex or solid. 9,12,15,16 Balan reported a patient in whom an ovarian lesion was considered as highly suspicious of malignancy at US but it was found to be an HCLC after surgery. 9 Ten patients (76.9%) enrolled in our study presented with complex lesions. In the presence of complex or solid lesions, other investigations such as CT or MRI scan must be performed, because of the malignancy risk. 9,15,17

4 166 Spinelli et al: Hemorrhagic Corpus Luteum Cysts Table 2. Incidence of pediatric ovarian cysts: a review of the literature Freud et al, Templeman et al, Cass et al, Deligeoroglou et al, Skiadas et al, Bristow et al, Surgically treated ovaries Functional cysts, n (%) 14 (41.2) 61 (43.6) 26 (24.6) 12 (25.5) 10 (25.0) 14 (17.1) Follicular - 24 (17.2) 11 (10.4) 5 (10.6) 4 (10.0) - Corpus luteum - 37 (26.4) 15 (14.2) 7 (14.9) 6 (15.0) - The overall accuracy of US, CT, and MRI scans in the assessment of pelvic pathology are 77%, 87%, and 97% respectively. 9 CT, used to clarify images, is helpful in distinguishing ovarian disorders from other diseases such as acute appendicitis, appendix abscess, tubo-ovarian abscess, or hydrocolpos/hematocolpos. 4,13 The difficulty in identifying cyst histology before surgery is due to the fact that a histological lesion corresponds to several US pictures and vice versa. Moreover, sometimes, even macroscopically, it may be difficult to define the exact nature of a lesion, as reported by Doret and Raudrant. 5 Indeed, imaging modalities may present false positive and false negative findings for a presumed functional ovarian cyst. Only surgery and histopathological analysis allow precise diagnosis of a solid or a complex lesion. 6,14 Emergency presentations should be considered first. In fact these may be misleading and may compromise the long-term outcome, especially in cases of acute rupture, torsion, or hemorrhage. 8 Two patients came to our attention with acute abdominal pain; one presented with a cyst complicated by ovarian torsion. The HCLC management depends on clinical symptoms, lesion size, and US appearance. 14,18 These cysts, as they are hormonally driven, tend to resolve spontaneously in few weeks (4e6 on average) and only a minority will require surgical treatment. 3,7,13 In the presence of asymptomatic cysts under 5 cm in diameter and normal US-based and biochemical parameters, ongoing surveillance is a reasonable option. 7,13,14 Surgical indications, although not absolute, include cysts greater than 5 cm in diameter, a failure of the cyst to resolve or decrease in size spontaneously, complex or solid cysts indicative of suspected malignancy, severe persistent abdominal pain and complications such as ovarian torsion, hemorrhage or infarction. 7e9 Final treatment is determined by the diagnosis, but clearly significance is placed on future reproduction: in order to preserve ovarian function, we performed conservative surgery whenever feasible, according to the recent literature. 7,8,18 Conservative HCLC treatment consists of enucleation of the cyst with ovarian reconstruction, thereby preserving reproductive function as much as possible. 13 With large lesions, it may be impossible to preserve ovarian tissue with an oophorectomy, conserving, if possible, the ipsilateral Fallopian tube. 8 In our series, oophorectomy has also been the procedure of choice in ovarian cyst torsion. 7,13 Some authors reported that detorsion may result in a conserved subsequent follicular function despite the predetorsion size of the ovary or color of the adnexa, even if this is seen to be black. 13 There is considerable debate as to whether it is important to prophylactically fix the contralateral ovary after one has torsed. 6,8,19 We performed a laparoscopic surgery in 6 cases. Experience with both adult and pediatric patients has shown that the ovary is accessible and suitable for laparoscopic surgery. 20e22 The cyst s size continues to be a limiting factor for laparoscopic surgery, with most surgeons opting for laparotomy for larger cysts. 13,22 There is substantial evidence that laparoscopy is associated with fewer adhesions than is conventional open surgery and it may be useful for preserving the reproductive function. 22 In conclusion, the majority of HCLC can be managed in pediatric patients with observation. Only a minority has to be explored surgically. Indications for intervention, as shown in our experience, included the detection of complex or solid cysts indicative of suspected malignancy, severe, or persistent abdominal pain and complications of simple cysts greater than 5 cm in diameter. The cutoff of 5 cm for surgery indication is controversial; some authors, in fact, suggest that in a stable patient with an appearing benign, cystic, unilocular lesion under 10 cm in diameter, simple surveillance can be considered an acceptable option. 23 US and CT may be helpful in differentiating functional ovarian cysts from other surgical conditions characterized by abdominal pain but, as confirmed in our experience, a certain diagnosis may be made only at the time of surgery. Finally, the goal of surgical management of pediatric HCLC is to remove the lesion preserving the underlying ovary in order to optimize the conservation of steroidogenesis and fertility. References 1. Deligeoroglou E, Eleftheriades M, Shiadoes V, et al: Ovarian masses during adolescence: clinical, ultrasonographic and pathologic findings, serum tumor markers and endocrinological profile. Gynecol Endocrinol 2004; 19:1

5 Spinelli et al: Hemorrhagic Corpus Luteum Cysts Freud E, Golinsky D, Steinberg RM, et al: Ovarian masses in children. Clin Pediatr 1999; 38: O Neill JA Jr. In: O Neill JA, editor. Gonadal tumors. Principles of Pediatric Surgery, (2nd ed.). Mosby, pp 285e Strickland JL: Ovarian cysts in neonates, children and adolescents. Curr Opin Obstet Gynecol 2002; 14: Doret M, Raudrant D: Functional ovarian cysts and the need to remove them. Eur J Obstet Gynecol Reprod Biol 2001; 100:1 6. Cass DL, Hawkins E, Brandt ML, et al: Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period. J Pediatr Surg 2001; 36: Creighton S: Pediatric and adolescent gynaecology. In: Burge DM, Mervyn Griffiths D, Steinbrecher HA, Wheeler RA, editors. Paediatric Surgery, (2nd ed.). Oxford University Press, Hodder Arnold, Publication, Oxford, UK, 2005, 555e Valla JS: Gonadal tumors. In: Mouriquand PDE, editor. Paediatric Surgery and Urology: Long Term Outcome. Cambridge, Cambridge University Press, 2006, pp 707e Balan P: Ultrasonography, computed tomography and magnetic resonance imaging in the assessment of pelvic pathology. Eur J Radiol 2006; 58: Bristow RE, Nugent AC, Zahurak ML, et al: Impact of surgeon specialty on ovarian-conserving surgery in young females with an adnexal mass. J Adolesc Health 2006; 39: Templeman C, Fallat ME, Blinchevsky A, et al: Noninflammatory ovarian masses in girls and young women. Obstet Gynecol 2000; 96: Skiadas VT, Koutoulidis V, Eleytheriades M, et al: Ovarian masses in young adolescents: imaging findings with surgical confirmation. Eur J Gynaecol Oncol 2004; 25: Wood PL: Pelvic pain, ovarian cysts and endometriosis in adolescent girls. In: Balen AH, editor. Pediatric and Adolescent Gynaecology. Cambridge, Cambridge University Press, 2004, pp 359e372. A Multidisciplinary Approach 14. Pienkowski C. In: Sultan C, editor. Pediatric and Adolescent Gynecology: Evidence-based Clinical Practice, Ovarian masses in adolescent girls, vol. 7. Basel, Karger, 2004, pp 359e Narducci F, Orazi G, Cosson M: Ovarian cyst: surgical indications and access. J Gynecol Obstet Biol Reprod 2001; 30:S Ignacio EA, Hill MC: Ultrasound of the acute female pelvis. Ultrasound Q 2003; 19: Hayes-Jordan A: Surgical management of the incidentally identified ovarian mass. Semin Pediatr Surg 2005; 14: Logsdon VK: Common problems in pediatric and adolescent gynecologic surgery. Curr Opin Obstet Gynecol 2001; 13: Celik A, Ergun O, Aldemir H, et al: Long-term results of conservative management of adnexal torsion in children. J Pediatr Surg 2005; 40: Jawad AJ, Al-Meshari A: Laparoscopy for ovarian pathology in infancy and childhood. Pediatr Surg Int 1998; 14: Eltabbakh GH, Kaiser JR: Laparoscopic management of a large ovarian cyst in an adolescent: A case report. J Reprod Med 2000; 45: Goh SM, Yam J, Loh SF, et al: Minimal access approach to the management of large ovarian cysts. Surg Endosc 2007; 21: Myers ER, Bastian LA, Havrilesky LJ, et al: Management of adnexal mass. Evid Rep Technol Assess 2006; 130:1