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A CASE REPORT Bali Medical Journal (Bali Med J) 2016, Volume 5, Number 3: 543-546 P-ISSN.2089-1180, E-ISSN.2302-2914 Fetal cardiac : a case report A Case Report Tjokorda Gde Agung Suwardewa,1* I Ketut Surya Negara,1 AAN Jaya Kusuma,1 AAP Wiradnyana,1 Ryan Mulyana Surya,1 I Ketut Tunas2 CrossMark Doi: http://dx.doi.org/10.15562/bmj.v5i3.346 Published by DiscoverSys ABSTRACT

Volume No.: 5 Background: Fetal cardiac rhabdomyoma is a rare condition. tomography scan, to rule out anything related to . Case: We report a case with cardiac mass discovered in utero by The prognosis depends on the size, site, number of tumors, and co- prenatal ultrasonography at 33 weeks of gestational age. An echogenic existing congenital abnormalities. Management highly depends on Issue: 3 round-oval shape mass at the interventricular septum protrudes to left the presence of outflow tract obstruction of the . However, some ventricle was observed. cases may regress after birth. Results: After birth, the baby was followed up for 7 months with echocardiography, physical examination, and computerized First page No.: 543

Keywords: rhabdomyoma, fetal heart, tuberous sclerosis. Cite This Article: Suwardewa, T., Surya Negara, I., Jaya Kusuma, A., Wiradnyana, A., Surya, R., Tunas, I. 2016. Fetal cardiac rhabdomyoma: a case P-ISSN.2089-1180 report. Bali Medical Journal 5(3): 543-546. DOI:10.15562/bmj.v5i3.346

1Department of Obstetrics and INTRODUCTION rhabdomyoma, which found incidentally during a E-ISSN.2302-2914 Gynecology, Udayana University / routine antenatal visit. Sanglah Hospital Denpasar, Bali- The presence of a primary congenital tumor of Indonesia. heart is a rare occurrence. Its incidence is 1-2/10000 2 Departement of Public Health, and 90% of these tumors are benign out and Dhyana Pura University, Bali- 1 CASE REPORT Indonesia. rhabdomyoma is the most common. Fetal heart rhabdomyoma is a closely related to A 30-year-old woman on her third pregnancy with tuberous sclerosis. Even though it is rare, however, two offspring came to the obstetrics-gynecology rhabdomyoma is the most common tumor found outpatient clinic in Sanglah General Hospital on among other heart tumors (50-78%).1,2 20th of October 2004 to have her routine prenatal Rhabdomyoma is often found in neonates care. She has had two caesarean sections for her and toddlers but rarely diagnosed before birth.3,4 previous pregnancies. The last menstrual period was Usually rhabdomyoma is diagnosed when compli- on 26thof February 2004. On the ultrasonography cations such as heart valve obstruction, present at examination, a single live fetus was found, 33 weeks the first year of life. and 6 days’ gestational age, the estimated due date Prenatal detection can be performed in the was on 2nd of December 2004. In the fetal heart, we second trimester of pregnancy.5 Until now the found a round-oval shape mass protruding into the etiology still remains unknown. The pathogenesis left ventricle, and the distal part of the tumor was is unclear. It is stated that embryonal myoblast attached to the interventricular septum. The tumor proliferation, which forms a single or multiple moved freely following the heartbeat. The size of solid mass which can reside anywhere within the the tumor was 17 mm × 9 mm, with uniform echo- myocardium of the atrium or ventricle protrude genic internal structure (figure 1). The mass did into the heart cavity. The differential diagno- not fill the entire heart ventricle figure( 2) and did ses are , , hemangioma, and not obstruct the fetal heart outflow tract. figure 3( ). .1,2 On routine prenatal care, the fetus did not suffer The prognosis depends on the size, site, number growth restriction. It was then decided to perform th *Correspondence to: Tjokorda Gde of tumors, and co-existing congenital abnormali- elective cesarean section on 24 of October 2004. Agung Suwardewa, Department of ties. Management highly depends on the presence A vigorous baby was born weighing 3200 grams, Obstetrics and Gynecology, Maternal of outflow tract obstruction of the heart. However, and no major anatomical anomalies were found. Fetal Medicine Division, Medical some cases may regress after birth. The most During physical examination of the baby on 25th Faculty of Udayana University / Sanglah Hospital Denpasar, important point is that there should be an evalu- of October, hypopigmented macules were found on Bali-Indonesia ation on tuberous sclerosis in the patients or their the medial part of left arm, right thigh, and back [email protected] families.1,2 We reported one case of fetal cardiac with varying size between 4-8 mm (figure 4).

Open access: www.balimedicaljournal.org and ojs.unud.ac.id/index.php/bmj 543 A CASE REPORT

Figure 1 T ransversal view of the fetal thorax. The intracardiac tumor is seen with round- Figure 4 Hypopigmented macule (vitiligo) at the oval shape, hyperechoic, at the inter- medial part of the left arm ventricular septum and protrudes to the left ventricle, and size of the tumor was 17 mm × 9 mm

Figure 2 Th e tumor is seen filling a small part of the left ventricle Figure 5 M ultiple calcifications in the left ventri- cle of the brain (white dots) Hospital, the heart valves were found to be normal; a mass was found in the left ventricle which moves freely (during systolic and diastolic); VSD, ASD, or PDA were not found. There was no proof of tuberous sclerosis upon examination of the patient’s parents. On 6th of April 2005, the baby had tonic-clonic seizures for 5 minutes, no fever was detected. Head CT-Scan found multiple calcifications in left lateral ventricles of the brain and it supports the diagnosis of tuberous sclerosis (figure 5).

Figure 3 The figure shows aorta coming out of DISCUSSION the left ventricle. The tumor is seen located in the left ventricle and not Primary solid tumor in the heart is a rare occur- blocking the aortic valve rence; however, rhabdomyoma is the most commonly found heart tumor in intrauterine life On echocardiography (26thof November 2004) (58%), among other tumors such as teratoma (19%), by a Pediatric Cardiologist in Sanglah General myxoma, hemangioma, and mesothelioma.1 Other

544 Published by DiscoverSys | Bali Med J 2016; 5 (3): 543-546 | doi: 10.15562/bmj.v5i3.346 A CASE REPORT

researchers reported that the incidences of rhab- flow which then causes heart failure and hydrops. domyoma are 60%-86%;6,7 1.17%;8 36%-42%;2,9 and Cardiac dysrhythmias are often found due to 60%.10 pressure on the conduction system.2 The presence The etiology is unknown. of cardiac dysrhythmias and nonimmune fetal tumor, which is known as , is a benign hydrops indicates a poor prognosis.1 The causes of tumor of the heart which contains death in fetal cardiac rhabdomyoma are ventric- immature myocytes.1 Rhabdomyoma comes from ular blood flow obstruction, cardiac dysrhyth- the embryonal myoblast in which an abnormal mias intrauterine myocard infarction, and loss of tissue proliferation happens and produces encap- myocardial function due to the pressure of the large sulated single or multiple solid mass, with varying mass.13 Various studies showed that rhabdomyoma sizes.2 is a slow growing intrauterine tumor with little or This tumor could grow anywhere in the no growth after birth. This tumor tends to regress cardiac muscle, and the most common site is spontaneously after birth.1,2,10 in the ventricle, rather than at heart valves, Management of fetal cardiac rhabdomyoma and usually protrudes inside the heart cavity. depends on the severity of the complications Macroscopically, it is seen as a yellow solid tumor which arise. It is very important to do a series of and round-oval shape. Specific microscopic find- ultrasound examinations to evaluate and identify ing is spider cell, which is a large and clear cell signs or symptoms of congestive heart failure and with glycogen contain cytoplasm that spreads to dysrhythmias. Delivery should be done in tertiary the plasma membrane.2 health center where a pediatric cardiologist is Cardiac rhabdomyomas are also known as available. Conservative management is provided myocardial . There is a strong correla- for asymptomatic patients and surgery is prepared tion between cardiac rhabdomyoma and tuberous for patient with hemodynamic instability.2 Special sclerosis, where the incidence rate of tuberous attention should be given to the central nervous sclerosis is 50-86% in patients with cardiac rhab- system, to find brain ventricle dilatation, and domyoma.2,10,11 Tuberous sclerosis is inherited in an kidneys dysplasia.2,10 Ruling out tuberous sclerosis autosomal dominant pattern with varying expres- is often very challenging because brain manifesta- sions and high penetration. The gene related to tions are not sufficiently monitored with ultrasound tuberous sclerosis is found on chromosome number only.14 Detailed family history especially mental 9. The clinical manifestations are skin lesions (depig- retardation and epilepsy should be explored, and mentation, sebacea adenoma, shagreen patches), tests should be performed on both parents to see cerebral abnormalities (calcifications and periven- the existence of clinical sign of tuberous sclerosis.2,15 tricular nodules, seizures, and cerebral atrophy), Postnatal management includes serial ultra- and phacoma of the retina.2 Dermatologic lesions, sound for monitoring signs of congestive heart uniform hypomelanotic macules, usually present at failure or cardiac dysrhythmias. If asymptomatic, birth. Diagnosis is based on skin biopsy or Wood it is advisable to treat conservatively. However, if Light examination. No correlation between cardiac a cardiac outflow obstruction, persistent arrhyth- rhabdomyoma with other genetical abnormalities, mias, heart failure, or cardiogenic emboli appear, a except with tuberous sclerosis.2 resection surgery should be considered.1 The differential diagnoses are: rhabdomyoma, teratoma, , myxoma, and hemangioma. REFERENCES Sonographic this tumor appears as single or multi- 1. Dalvi R, Vernekar J, Godinho S, Kartha R. Cardiac rhabdo- ple, homogeneous echogenic mass within the - antenatal diagnosis. Indian J Radiol Imaging [serial myocardium. Teratoma appears as a complex mass, online]. 2002 [cited 18th Dec 2004];12:339-40. Available which covers the pericardium, and usually related from: http://www.ijri.org/text.asp?2002/12/3/339/28475. 2. Meyer WJ, Gauthier DW, Font G. Cardiac Rhabdomyoma. with pericardial effusion. Sometimes double cham- The fetus net (serial online) Last update 16 September bered right ventricle is seen. 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6. Allan L, Hornberger LK, Sharland GK (eds). Fetal car- 12. Meyer WJ, Gauthier DW, Font G. Cardiac Rhabdomyoma. diac tumours. Textbook of Fetal Cardiology. London: The fetus net [serial online] Last update 19 January 1991 Greenwich Medical; 2000. [cited 18th Dec 2004]. Available from: URL: https://www. 7. Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr sonoworld.com/fetus/page.aspx?id=49 Cardiol. 2004; 25: 252–73. http://dx.doi.org/10.1007/ 13. Geva T, Santini F, Pear W, Driscoll RG, Praagh RV. s00246-003-0590-4. Cardiac Rhabdomyoma. Rare Cause of Fetal Death. 8. Holley DG, Martin GR, Brenner JI. Diagnosis and manage- Chest. 1991;99:139-42. http://dx.doi.org/10.1378/ ment of fetal cardiac tumors: a multicentre experience and chest.99.1.139 review of published report. J Am Coll Cardiol. 1995; 26:516- 14. Tercanli S, Gembruch U, Holzgreve W. Nonimmune 20. http://dx.doi.org/10.1016/0735-1097(95)80031-B Hydrops Fetalis: Diagnosis and Management. In Callen 9. Pipiton S, Mongiovi M, GrilloR, Gaglian S, Sperandeo V. ed. Ultrasonography in Obstetrics and Gynecology. 4th ed. Cardiac rhabdomyoma in intrauterine life: Clinical fea- Tokyo: Saunders; 2000. p 557. ture and natural history: A case series and review of pub- 15. Davies MJ. Tumor of the heart and pericardium. The lished reports. Ital Heart J [Internet]. 2002 [cited 18th Dec pathology of the heart. London: Blackwell; 1975. p 2004];3(1):48-52. Available from: http://europepmc.org/ 423-40. abstract/med/11899590 10. Merz E. Ultrasound in Obstetrics and Gynecology. Second ed. New York: Thieme; 2005. 11. Bader RS, Chitayat D, Kelly E. Fetal rhabdomyoma: pre- natal diagnosis, clinical outcome, and incidence of associ- ated tuberous sclerosis complex. J Pediatr. 2003;143:620-4. https://dx.doi.org/10.1067/S0022-3476(03)00494-3 This work is licensed under a Creative Commons Attribution

546 Published by DiscoverSys | Bali Med J 2016; 5 (3): 543-546 | doi: 10.15562/bmj.v5i3.346