Presentasi Kasus Bedah Plastik

SEORANG ANAK 2 TAHUN DENGAN HEMANGIOMA REGIO BUCCAE DEXTRA

Oleh: A.D. Rahmilia Fenda Adita Tiur Estika Situmorang G0006172 G0007072 G0007023

Pembimbing : dr. Amru Sungkar, Sp.B, Sp. BP

KEPANITERAAN KLINIK ILMU BEDAH FAKULTAS KEDOKTERAN UNS / RSUD Dr. MOEWARDI SURAKARTA 2011

STATUS PASIEN

A. Anamnesa I. Identitas pasien Nama Umur Jenis Kelamin Agama Alamat No CM MRS Tanggal Pemeriksaan : An. R : 2 Tahun : Laki-laki : Islam : Jagalan RT 01/13 Jebres, Surakarta : 01 04 20 90 : 26 September 2011 : 26 September 2011

II. Keluhan Utama Benjolan di pipi kanan

III. Riwayat Penyakit Sekarang Orang tua pasien mengeluh ada benjolan pada pipi kanan pasien. Benjolan dikeluhkan sejak lahir yang semakin lama semakin membesar. Orang tua pasien mengaku kalau pasien tidak pernah menangis karena nyeri dari benjolan tersebut. Orang tua pasien mengaku sudah 2x pasien menjalani operasi pengangkatan benjolan tersebut, yaitu bulan januari 2011 dan bulan mei 2011. Sekarang benjolan tersebut timbul lagi dan semakin lama semakin membesar.

IV. Riwayat Penyakit Dahulu a. Riwayat bibir sumbing b. Riwayat penyakit darah c. Riwayat Diabetes melitus d. Riwayat alergi/asma : disangkal : disangkal : disangkal : disangkal

B. Anamnesa sistemik Mata : mata kuning (-), penglihatan kabur (-), pandangan ganda (-), berkunang-kunang (-) Telinga : darah (-), lendir (-), cairan (-), telinga berdenging (-), pendengaran berkurang (-) Mulut : darah (-), gusi berdarah (-), sariawan (-), mulut kering (-), gigi goyah (-) sulit berbicara (-) Hidung : penciuman menurun (-), darah (-), sekret (-) Sistem Respirasi : sesak nafas (-), suara sengau (-), sering tersedak (-) Sistem Kardiovaskuler Sistem Gastrointestinal : nyeri dada (-), sesak saat aktivitas (-) : mual (-), muntah (-), nyeri perut (-), diare (-) Sistem Muskuloskeletal : nyeri otot (-), nyeri sendi (-) Sistem Genitourinaria Integumen : nyeri BAK (-), kencing darah (-) : nyeri (-), lepuh (+) pada ekstremitas superior, gatal (-)

C. Pemeriksaan Fisik I. Primary Survey a. Airway b. Breathing : Bebas, cervical spine stabil : Pernapasan spontan, thoracoabdominal RR: 22x/menit c. Circulation d. Disability e. Exposure : TD: 120/80 mmHg, N: 84x/menit. : GCS: E4V5M6, lateralisasi (-) : t: 36,7 oC, jejas (+) (lihat status lokalis)

II. Secondary Survey a. Kepala b. Mata : mesocephal, jejas (-), benjolan di pipi kanan (+) : konjungtiva pucat (-/-), sklera ikterik (-/-), pupil isokor (3mm/3mm), reflek cahaya (+/+), hematom periorbita(-/-) c. Telinga : secret (-), darah (-), nyeri tekan mastoid(-), Nyeri Tragus (-) d. Hidung : bentuk simetris, nafas cuping hidung (-), secret (-), darah (-) e. Mulut : gusi berdarah (-), lidah kotor (-), jejas (-), mukosa basah (+), maxilla goyang (-), mandibula goyang (-), pelo (+) f. Leher : pembesaran tiroid (-), pembesaran limfonodi (-), Nyeri tekan (-), JVP tidak meningkat g. Thoraks : bentuk normochest, simetris, gerak pernafasan simetris, jejas (-) (lihat status localis) h. Jantung Inspeksi Palpasi Perkusi : ictus cordis tidak tampak : ictus cordis tidak teraba : batas jantung kesan tidak melebar

Auskultasi : bunyi jantung I-II intensitas normal, reguler, bising(-) i. Pulmo Inspeksi Palpasi Perkusi : pengembangan dada kanan = kiri : fremitus taktil kanan=kiri : sonor/sonor

Auskultasi : suara dasar vesikuler (+/+), suara tambahan (-/-) j. Abdomen Inspeksi : distended (-)

Auskultasi : bising usus (+) normal Perkusi : timpani

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Palpasi k. Ekstremitas

: supel, nyeri tekan (-), defance muscular (-) : akral dingin oedem -

D. Status Lokalis: Regio Buccar dextra Inspeksi Palpasi : benjolan (+) warna hitam kebiruan : nyeri tekan (-), lunak, pulsasi tidak teraba, ukuran 4x3x3cm, batas tidak tegas

E. Assesment I Soft Tissue Tumor R.Buccae (D)

F. Plan I 1. Cek DR3 dan elektrolit 2. USG Doppler

G. Pemeriksaan Penunjang 1. Pemeriksaan Laboratorium Hb AE Hct AL AT Golongan darah PT APTT Na : 11 g/dL : 6,12. 106/uL : 35,3 % : 11,3. 103/uL : 278. 103/uL :B : 13,7 dtk : 33,8 detik : 145 mmol/L
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K Ion Ca

: 4,8 mmol/L : 1,15 mmol/L

H. Assesment II Hemangioma R.Buccae (D) Post Eksisi

I. Plan II 1. Konsul bedah plastik 2. Medikasi

J. Prognosis a. Ad vitam b. Ad sanam c. Ad kosmetikum d. Ad fungsionam : dubia ad bonam : dubia ad bonam : dubia ad bonam : dubia ad bonam

Tinjauan Pustaka

Hemangioma I. Definisi Hemangioma adalah suatu tumor pembuluh darah dengan cirri proliferasi endotel yang meningkat pesat pada waktu bayi ( 1 tahun pertama), dan dapat mengalami involusi secara perlahan pada masa kanakkanak melalui proses kematian sel secara progresif atau terjadinya fibrosis (sampai usia 6-7 tahun) ( Sudjatmiko, 2010).

II.

Epidemiologi Insiden hemangioma di AS terjadi 0,54 setiap 1000 kelahiran. Insiden pada bayi kulit putih 10-12%, sedangkan pada bayi premature dengan berat badan <1000 gram insidennya sebanyak 22%. Hemangioma lebih banyak terjadi pada anak perempuan dengan perbandingan 2-5:1 ( Sudjatmiko, 2010).

III. Pathogenesis Hemangioma merupakan tumor pada infant yang sering terjadi. Hemangioma merupakan tumor jinak yang muncul sejak awal dan berproliferasi cepat selama tahun pertama kehidupan, yang bercirikan dari hiperplasi endotel dan percytic, dan diikuti dengan fase involusi yang lambat. Pola dasar dari klasifikasi histopatologi telah dibagi berdasarkan karakter imunohistokimia dari hemangioma di setiap fase (Marchuck, 2001).

Gambar 1. Hemangioma pada Regio Frontal

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Hemangioma merupakan suatu tipe angiogenesis, yaitu meningkatnya factor angiogenesis dan berkurangnya factor supresi sel-sel. Hemangioma yang berploriferasi terdiri atas kumpulan sel-sel endotel yang membelah cepat. Saat mengalami involusi, aktivitas endotel berkurang, dan sel-selnya menjadi lebih rata dan matur ( Sudjatmiko, 2010). Hemangioma kapiler terbentuk dari saluran vaskuler yang kecil dan menyempit yang dibatasi dengan satu lapis endotel dan dikelilingi jaringan pengikat. Sedangkan cavernous hemangioma terbentuk dari saluran vaskuler yang besar. Kapiler lobular hemangioma merupakan bentuk polipoid yang terbanyak dari hemangioma kapiler yang terjadi di permukaan mukosa seperti kavitas oral dan nasal, lidah, konjungtiva, duodenum atau colon ( Baharudin, 2005).

IV. Perjalanan Penyakit Hemangioma a. Hemangioma biasanya ditemukan pada 2 minggu pertama masa neonatal, tapi pada hemangioma subkutan atau visceral baru muncul pada usia 2-3 bulan. Saat kelahiran hemangioma dapat tampak sebagai bintik pucat, bercak merah, atau daerah kemerahan yang menyerupai memar. b. Fase Proliferatif: hemangioma tumbuh cepat pada 6-8 bulan pertama. Kulit menjadi menonjol dan berwarna merah muda terang. Bila letaknya lebih dalam, maka kulit hanya sedikit menonjol dan berwarna kebiruan. c. Fase Involusi: warna kulit berubah menjadi keunguan, dan tumor menjadi melunak. Involusi terjadi pada 50% anak usia 5 tahun dan 70% anak usia 7 tahun. Pada 50% anak kulit akan kembali seperti semula. Sisanya dapat meninggalkan kemerhan, keriput, daerah kekuningan yang hipoplastik, parut (bila terjadi ulserasi), atau sisa fibrosis jaringan lemak ( Sudjatmiko, 2010).

V.

Manajemen Meski umumnya mengalami involusi spontan, tindakan operatif dilakukan segera dan secara agresif pada keadaan sebagai berikut: a. Obstruksi jalan napas b. Gagal jantung c. Ulserasi dan perdarahan yang tidak terkendali d. Infeksi berulang yang sulit dikendalikan e. Trombositopenia f. Obstruksi struktur vital g. Gangguan pertumbuhan tulang h. Lesi kecil ( sehingga mudah diangkat tanpa risiki kosmetik maupun fungsional) i. Nyeri

Manajemen Hemangioma a. Terapi bedah

Gambar 2. Excisi Hemangioma

b.Penanganan perdarahan dan ulserasi c. Mengatasi komplikasi d.Terapi non bedah : kortikosteroid, interferon alfa, laser, kemoterapi, pressure therapy, thermal therapy, radiasi, embolisasi, skleroterapi.
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e. Observasi secara berkala untuk memantau perjalanan penyakit. ( Sudjatmiko, 2010)

VI. Prognosis Perbaikannya tergantung kecepata mendiagnosa dan ketepatan

mengangkatnya secara operatif. Pasien harus ditindaklanjuti selama beberapa tahun untuk evaluasi.

VII. Komplikasi a. Problem psikososial pada keluarganya b. Gangguan penglihatan (ambliopia atau strabismus) bila terlambat dioperasi c. Perdarahan d. Perubahan bentuk organ misalnya bibir dan palpebra ( Sudjatmiko, 2010)

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Daftar Pustaka

Baharudin A., et al. 2005. Capillary hemangioma of the lateral nasal wall in a patient who has acute lymphoblastic leukemia in remission. The International Medical Journal. 2 Desember 2005: (Vol 4 Page 49-50).

Ersoz F, et.al. 2010. Torsion of a giant pedunculated liver hemangioma mimicking acute appendicitis: a case report. World Journal of Emergency Surgery. 18 Januari 2010. Marchuck, Douglas A. 2001. Pathogenesis of Hemangioma. American Society of Clinical Investigation. 15 Maret 2001: 107(6):665-666.

Sudjatmiko, Gentur. 2010. Ilmu Bedah Plasttik Rekonstruksi. Yayasan Khazanah Kebajikan.

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Pathogenesis of hemangioma
Douglas A. Marchuk Department of Genetics, Box 3175, Duke University Medical Center, Durham, North Carolina 27710, USA. Phone: (919) 684-3290; Fax: (919) 681-9193; E-mail: march004@mc.duke.edu. Published March 15, 2001 Hemangioma is the most common tumor of any kind seen in infancy. It is also, perhaps, the least understood. This stems in part from a long history of confusing nomenclature for vascular anomalies that employed classification schemes based on superficial descriptions of the lesions. The first major step toward clarifying this nomenclatural morass was made by Mulliken and colleagues (1, 2), who employed a biological classification scheme based on the differing clinical courses and endothelial proliferative activity of hemangiomas versus malformations. Hemangiomas, often called infantile or juvenile hemangiomas for clarity, are benign tumors that exhibit an early and rapid proliferation phase during the first year of life characterized by endothelial and pericytic hyperplasia, followed by a slower but steady involution phase that may last for years. This basic scheme of histopathological classification has been refined by immunohistochemical characterization of hemangioma in each of the phases (3). Nonetheless, the pathogenesis of hemangioma is still not understood. Although growth factors and hormonal and mechanical influences have been postulated to affect the abnormal proliferation of endothelial cells in hemangioma, the primary, causative defect in hemangiogenesis remains unknown and no genetic alteration has been implicated. This dearth of molecular details is striking, considering the growing list of germline mutations in genes causing specific inherited syndromes involving vascular malformations, including hereditary hemorrhagic telangiectasia (4, 5), cutaneous venous malformations (6), cerebral cavernous malformations (7, 8), and hyperkeratotic cutaneous capillary-venous malformation (9). Are we able to investigate hemangioma in a similar manner? Cheung and colleagues (10) compared the concordance of hemangioma in monozygotic versus dizygotic twins and found no evidence of a strong predisposing inherited component. However, Blei and colleagues described six rare families segregating hemangiomas and/or vascular malformations as an autosomal dominant trait with incomplete penetrance (11). This suggested a predisposing mutation in these families segregating the trait. Genetic involvement was bolstered with the genetic mapping of a locus on chromosome 5q for hemangioma/malformation development in these particular families (12), but the gene responsible has yet to be identified. In
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addition, it is not certain that this gene plays any role in the more common sporadic hemangiomas. Into this gene-deficient story comes the paper of Boye and colleagues (13) in this issue of the JCI, showing that endothelial cells derived from hemangioma are clonal in origin and demonstrating that they arise from a common precursor cell. Significantly, fibroblast-like cells isolated from these tumors are not clonal, as determined using a well-established assay based on X-chromosome inactivation in females.

Cell-autonomous hemangiomas

defects

in

The data of Boye et al. (13) support the hypothesis that the primary defect is intrinsic to endothelial cells, rather than other cell types constituting the hemangioma. A competing hypothesis, holding that proliferation of endothelial cells is primarily a response to factors secreted by neighboring cells, can therefore be ruled out. Although stromal cells (14) and the overlying epidermis of hemangiomas (15) exhibit altered properties, including aberrant expression of angiogenic factors, these endothelial cells would not be expected to develop as clones of a single founder cell if they were merely responding to an externally derived angiogenic signal. The clonality data are intriguing in one other aspect as well. When Williams et al. (16) injected mouse endothelioma cells expressing the polyoma middle-T oncogene into adult mice, they observed that the resulting tumors resemble benign hemangiomas, but they showed that the endothelial cells within these tumors are primarily host-derived, apparently recruited by the injected oncogenic cells. The results obtained by Boye et al. for hemangioma in infants (13) show no evidence of additional endothelial recruitment by the original endothelial precursor cells of the tumor. If additional endothelial cells are indeed recruited into the tumor, it appears that they retain their nonproliferative phenotype. It would be interesting to determine whether the endothelial cells directly obtained from the tumor, prior to selection via growth in vitro, also retain their clonality. Boye and colleagues (13) show that the endothelial cells they isolated from hemangiomas exhibit enhanced proliferation and migration, in keeping with the rapid growth of the vascular lesion in the neonate. However, there was one surprise: In the presence of the angiogenic inhibitor endostatin, migration of these cells was not inhibited but rather stimulated, suggesting a radically altered cellular phenotype. One possible explanation, favored by the authors, is that a precursor endothelial cell had undergone a mutation in a gene regulating angiogenesis, resulting in clonal expansion. Evidence for somatic mutations in hemangioma comes from Berg and colleagues, who showed that loss of heterozygosity (LOH)
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in hemangioma tissue is prevalent on chromosome 5q (17), suggesting that lossof-function mutations contributes to hemangioma development. Research over the past decade has shown that angiogenesis is tightly regulated by a balance of promoting and inhibitory factors. One way to shift the balance toward aberrant proliferation would be to inactivate one of these inhibitory factors. Thus, the LOH on chromosome 5q suggests that an inhibitory factor maps to this chromosomal region, which, when mutated, promotes hemangioma development. An equally likely possibility is a somatic mutation leading to constitutive activation of an angiogenesis-promoting gene. A number of candidate genes come to mind, some of which are suggested by Boye et al. (13). These genes can be sequenced in the isolated endothelial cells to identify somatic mutations responsible for hemangiogenesis.

Developmental hemangiomas

origins

of

An alternative interpretation of the present data comes from work of North and colleagues, who have documented the expression of placental vascular epitopes in hemangiomas. Hemangiomas display high levels of immunostaining for the GLUT1 glucose transporter (18), a surface protein that is highly expressed in most embryonic and fetal endothelial cells but is lost in most tissues except at the blood-tissue barriers, including microvessels in the central nervous system and the placenta. North et al. have expanded on this initial finding to show that other antigens associated with placental vessels, including FcRII, Lewis Y antigen, and merosin, are also expressed in hemangioma (19). The similarities in gene expression between hemangiomal and placental vessels might be explained by a somatic mutation in a regulatory gene that directs hemangiomal endothelial cells toward a placental phenotype, but North and colleagues also suggest an alternative theory (19). Embolic placental endothelial cells could reach fetal tissues from chorionic villi through right-to-left shunts characteristic of the normal fetal circulation. If the embolus contained a single endothelial cell or only a small number of endothelial cells, this would also be compatible with the clonality results obtained by Boye and colleagues (13). Because the placental endothelial cells involved might well be of fetal rather than maternal origin, these models are not easily distinguished, short of identifying somatic mutations in genes that can be subsequently shown to induce hemangioma formation. Nevertheless, the placental origin theory is attractive, because it would explain the exclusively perinatal or congenital presentation of hemangiomas. The otherwise puzzling observation that chorionic villus sampling increases the risk of hemangiomas (20, 21) further supports this model, since the local placental injury caused in this procedure might increase shedding of endothelial cells from chorionic villi into the fetal circulation.
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Hemangiomas pose other perplexing questions that will only be answered as the events that initiate hemangiogenesis are elucidated. For example, the strong gender predilection of hemangioma toward female over male infants (3:1 or more) suggests hormonal effects in hemangiogenesis. In addition, the anatomical predilection for the head and neck region of juvenile hemangioma must be explained. Perhaps most intriguing from a therapeutic standpoint is the spontaneous involution of the lesion. This distinguishing characteristic has been shown to be due in part to apoptosis of the endothelial cells (22), but the trigger for this process remains unknown. Can this apoptotic program be switched on earlier and be accelerated? Whatever mechanisms are identified in hemangiogenesis, we have come a long way from the descriptive phase of hemangioma research. The intriguing data of Boye and colleagues (13) suggest one mechanism for hemangioma formation and bring the field a step closer to understanding the molecular etiology of this common tumor.

References
1. Mulliken, J, Glowacki, J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982. 69:412-420. View this article via: PubMed 2. Mulliken, J., and Young, A.E. 1988. Vascular birthmarks: hemangiomas and vascular malformations. W.B. Saunders Co. Philadelphia, Pennsylvania, USA. 2437. View this article via: PubMed 3. Takahashi, K, et al. Cellular markers that distinguish the phases of hemangioma during infancy and childhood. J Clin Invest 1994. 93:23572364. View this article via: JCI.org PubMed CrossRef 4. McAllister, KA, et al. Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet 1994. 8:345-351. View this article via: PubMed CrossRef 5. Johnson, DW, et al. Mutations in the activin receptor-like kinase 1 gene in hereditary hemorrhagic telangiectasia type 2. Nat Genet 1996. 13:189-195.
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View this article via: PubMed CrossRef 6. Vikkula, M, et al. Vascular dysmorphogenesis caused by an activating mutation in the receptor tyrosine kinase TIE-2. Cell 1996. 87:1181-1190. View this article via: PubMed CrossRef 7. Laberge-le Couteulx, S, et al. Truncating mutations in CCM1, encoding KRIT1, cause hereditary cavernous angiomas. Nat Genet 1999. 23:189193. View this article via: PubMed CrossRef 8. Sahoo, T, et al. KRIT1, a Krev1/rapla binding protein, is mutated in cerebral cavernous malformations (CCM1). Hum Mol Genet 1999. 8:2325-2334. View this article via: PubMed CrossRef 9. Eerola, I, et al. KRIT1 is mutated in hyperkeratotic cutaneous capillaryvenous malformation associated with cerebral capillary malformation. Hum Mol Genet 2000. 9:1351-1355. View this article via: PubMed CrossRef 10. Cheung, DS, Warman, ML, Mulliken, JB. Hemangioma in twins. Ann Plast Surg 1997. 38:269-274. View this article via: PubMed 11. Blei, F, Walter, J, Orlow, SJ, Marchuk, DA. Familial segregation of hemangiomas and vascular malformations as an autosomal dominant trait: a rare genetic disorder. Arch Dermatol 1998. 134:718-722. View this article via: PubMed CrossRef 12. Walter, JW, et al. Genetic mapping of a novel familial form of infantile hemangioma. Am J Med Genet 1999. 82:77-83. View this article via: PubMed CrossRef 13. Boye, E, et al. Clonality and altered behavior of endothelial cells from hemangiomas. J Clin Invest 2001. 107:745-752. View this article via: JCI.org PubMed CrossRef
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14. Berard, M, et al. Vascular endothelial growth factor confers a growth advantage in vitro and in vivo to stromal cells cultured from neonatal hemangiomas. Am J Pathol 1997. 150:1315-1326. View this article via: PubMed 15. Bielenberg, DR, et al. Progressive growth of infantile cutaneous hemangiomas is directly correlated with hyperplasia and angiogenesis of adjacent epidermis and inversely correlated with expression of the endogenous angiogenesis inhibitor, IFN-beta. Int J Oncol 1999. 14:401408. View this article via: PubMed 16. Williams, RL, et al. Endothelioma cells expressing the polyoma middle T oncogene induce hemangiomas by host cell recruitment. Cell 1989. 57:1053-1063. View this article via: PubMed CrossRef 17. Berg, J.N., et al. 2001. Evidence for loss of heterozygosity of 5q in sporadic haemangiomas: are somatic mutations involved in haemangioma formation? J. Clin. Pathol. In press. View this article via: PubMed 18. North, PE, Waner, M, Mizeracki, A, Mihm (Jr), MC. GLUT1, a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol 2000. 31:11-22. View this article via: PubMed CrossRef 19. North, P.E., et al. 2001. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch. Dermatol. In press. View this article via: PubMed 20. Kaplan, P, et al. Malformations and minor anomalies in children whose mothers had prenatal diagnosis: comparison between CVS and amniocentesis. Am J Med Genet 1990. 37:366-370. View this article via: PubMed CrossRef 21. Burton, BK, Schulz, CJ, Angle, B, Burd, LI. An increased incidence of haemangiomas in infants born following chorionic villus sampling (CVS). Prenat Diagn 1995. 15:209-214.
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View this article via: PubMed CrossRef 22. Razon, MJ, Kraling, BM, Mulliken, JB, Bischoff, J. Increased apoptosis coincides with onset of involution in infantile hemangioma. Microcirculation 1998. 5:189-195. View this article via: PubMed CrossRef This article Copyright 2001, American Society for Clinical Investigation Copying, redistribution, and other usage policies

Torsion of a giant pedunculated liver hemangioma mimicking acute appendicitis: a case report
Feyzullah Ersoz1*, Ozhan Ozcan1, Ahmet B Toros2, Serdar Culcu1, Hasan Bektas1, Serkan Sari1, Esra Pasaoglu3 and Soykan Arikan1

* Corresponding author: Feyzullah Ersoz feyzullahersoz@yahoo.com

Author Affiliations
1

Istanbul Education and Research Hospital, Department of General Surgery, Istanbul, Turkey
2

Istanbul Education and Research Hospital, Department of Gastroenterology, Istanbul, Turkey

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Istanbul Education and Research Hospital, Department of Pathology, Istanbul, Turkey

For all author emails, please log on. World Journal of Emergency Surgery 2010, 5:2 doi:10.1186/1749-7922-5-2

The electronic version of this article is the complete one and can be found online at: http://www.wjes.org/content/5/1/2

Received: 16 November 2009 Accepted: 18 January 2010 Published:18 January 2010

2010 Ersoz et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract
Hemangiomas are the most common benign neoplasms affecting the liver. They occur at all ages. Most cases are asymptomatic and do not require any treatment. Rarely, hemangiomas can be pedunculated. f they undergo torsion and infarction, they become symptomatic. Herein; we report the case of a 31 year old male presenting with features of acute appendicitis: continuous right iliac fossa pain, rebound, guarding tenderness at McBurney' s point, nausea, anorexia, shifted white blood cell count and a Mantrels score of 6. At laparotomy a normal appendix was observed and a torsioned pedinculated liver hemangioma turned out to be the cause.

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ntroduction
Hemangiomas are the most common benign neoplasms affecting the liver with an incidence of 0.4-20% in autopsy series [1]. Women are affected more often than men. The female-to-male ratio is 5:1 to 6:1. They occur at all ages. Most cases are asymptomatic and do not require any treatment. Pedunculated haemangiomas are extremely rare, with only a few cases reported in the literature [2]. Herein; we report the case of a torsioned giant pedunculated liver haemangioma that mimicked acute appendicitis.

Case Presentation
A 31 year old man admitted to our emergency department with a 2 day history of right iliac fossa pain which he described as continuous. He also had anorexia, nausea. On physical examination, his pulse rate was 96 beats/min, his body temperature was 37.1C. His abdomen was markedly tender at the right iliac fossa with guarding and rebound tenderness at McBurney's point. The rest of the systemic examination was normal and the Mantrels score of the patient was 6. Laboratory data was as follows; hemoglobin 15.8 g/dl, total leukocyte count 9700/mm3, with 75% polymorphonuclear leukocytes, 37% lymphocytes, 3,2% monocytes, and 1% eosinophils; erythrocyte sedimentation rate was 2 mm for 1 h. Liver function tests, serum electrolytes, and creatinine were all within normal ranges. His bowel movements were regular on oscultation. Per rectum examination was normal. The abdominal X-ray was normal and because of the manifest clinical picture, abdominal ultrasound was not performed. Under the light of medical history and signs on abdominal examination, the patient was diagnosed as having acute appendicitis with a Mantrels score of 6 and was taken to theatre for appendectomy. At operation a normal appendix was found. At further exploration, a large soft reddish mass was palpated near the caecum. Macroscopically, the mass measured 10 12 15 cm. It was connected to the right inferior margin of the liver with a thin pedincule. It had undergone a 360 clockwise torsion on its pedincule. The mass was easily detorsioned and resected (Fig 1 and 2). Appendectomy was also performed using the routine method. Histologic assessment confirmed a cavernous hemangioma. The mass had multiple vascular spaces and fibrosis and was unusual for that there was a considerable amount of adipocytes intermingling within the tumor (Fig 3). The patient's recovery was uneventful, and he was discharged on the 2nd postoperative day.

Figure 1. Pedinculated hemangioma on the operation table; black arrow points the pedincule.

Figure 2. Resected hemangioma; arrows point the pedincule.

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Figure 3. Histopathologically the lesion composed of large vessels with cystically dilated lumina and thin walls. Lumen of blood vessels is filled with erythrocytes.(H+E).

Discussion
Cavernous hemangioma is the most common benign tumor of the liver. They are probably of congenital origin and have no potential for malignant transformation. Most are diagnosed incidentally and are asymptomatic. Hemangiomas are usually found at the right lobe of the liver in a subcapsular or marginal location. Most hemangiomas are diagnosed incidentally and are small and asymptomatic. Their size usually remains stable and can vary from a few milimetres to more than 20 cm. Lesions larger than 4 cm have been defined as giant hemangiomas [3]. Giant hemangiomas may cause abdominal discomfort, swelling, abdominal pain, icterus and thrombocytopenia [4]. Very rarely, spontaneous rupture with intraabdominal hemorrhage may create acute abdominal symptoms, which may also occur after rupture due to blunt abdominal trauma. Surgery is the treatment of choice, especially for giant, symptomatic hemangiomas with uncertain diagnosis. Rarely, hemangiomas can be pedunculated [5]. At ultrasound, the origin of the lesion may be difficult to recognize. The lesion can be attached to the liver with a thin pedicle, which is nearly undetectable at imaging. If they undergo torsion due to their long, mobile pedincule and get infarcted, they may become symptomatic. Pain is the most frequent symptom and most likely occurs from infarction or pressure on surrounding tissues. They can seldom cause pressure symptoms or get ruptured. Definite diagnosis should be made to distinguish it from other causes of acute abdominal pain. To the best of our knowledge, this present case is the first example of a torsioned pedunculated hemangioma in the literature, mimicking acute appendicitis with a Mantrels score of 6 [6]. In conclusion, this case report impresses that; even incidentally detected pedunculated hemangiomas must be managed by surgery for their tendency to get torsioned. In addition; the surgeon must look for different ethiologies when a normal appendix is found during operation.

Competing interests
The authors declare that they have no competing interests.

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Authors' contributions
All the authors participated in the admission and the care of this patient, the conception, the design, data collection and interpretation, manuscript preparation and literature search. All authors have read and approved the final manuscript

Consent
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Acknowledgements
No person and/or instution supported to this manuscript

References
1. Karhunen PJ: Benign hepatic tumours and tumour like conditions in men. J Clin Pathol 1986, 39:183-188. PubMed Abstract | Publisher Full Text | PubMed Central Full Text Return to text 2. Vivarelli M, Gazzotti F, D'Alessandro L, Pinna AD: Emergency presentation of a giant pedunculated liver haemangioma. Dig Liver Dis 2009. doi:10.1016/j.dld.2008.12.09

PubMed Abstract | Publisher Full Text

Return to text 3. Adam YG, Huvos AG, Fortner JG: Giant hemangiomas of the liver.

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49 The International Medical Journal Vol. 4 No 2 Dec 2005

CASE REPORT

Capillary hemangioma of the lateral nasal wall in a patient who has acute lymphoblastic leukemia in remission

A. Baharudina , R. Rosdi a , SAH Suzinaa and E. Omar b a Dept of ORL-HNS, School of Medical Sciences, Universiti Sains Malaysia b Dept of Pathology, School of Medical Sciences, Universiti Sains Malaysia

ABSTRACT

Haemangiomas are benign tumours composed of blood vessels; they are probably developmental 24

rather than neoplastic in origin. Capillary hemangioma arising from the nasal cavity is rarely encountered in our practice and rarely reported in the literature. We report a case of an 11-year-old Malay boy with capillary haemangioma of the lateral nasal wall who presented with epistaxis and persistent nasal blockage. He had chemotherapy at the age of 6 years old for acute lymphoblastic leukaemia and was in remission. We believe unnecessary investigations could be avoided if its diagnosis is considered especially in children. We also discuss the different approaches of treatment for this condition.

Keywords: Capillary hemangioma, epistaxis, lateral nasal wall, acute lymphoblastic leukaemia, remission. INTRODUCTION

Hemangioma occurs in 1 out of 100 Caucasian births, with about one half occurring in the head and neck (1). The term hemangioma is a general term covering a variety of conditions that only bear little or no relationship between them except for the vascular lesions involved. Hemangiomas are benign neoplasms of vascular origin with endothelial proliferation.

These lesions had originally received pathological diagnoses of cellular, capillary or juvenile

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hemangioma, reflecting various terminologies used between 1980 and 1997. However, low-magnification views revealed clear distinguishing features for these lesions (2). In most publications, the nose has been reported as an unusual location for this lesion.

CASE REPORT

An 11 year old Malay boy was seen in the Otorhinolaryngology-Head and Neck Surgery (ORL-HNS) clinic, Hospital Universiti Sains Malaysia (HUSM) in November 2003 with a history of persistent left nasal blockage for 2 months duration and associated with daily episodes of epistaxis with clots and fresh blood and most of the time preceded by a history of nose picking. He has no other nasal symptoms.

He had been given chemotherapy at the age of 6 years old after being diagnosed with acute lymphoblastic leukemia and was followed up regularly in the pediatric clinic for his condition. Examination showed a young boy with a normal vital signs and no pallor. Nasal examination using a 0 degree nasal endoscope showed a small mass arising from the left lateral wall with blood clot surrounding the mass. Other ENT examinations were unremarkable. The lymph nodes, liver and spleen were not palpable. X-ray of the paranasal sinuses didnt reveal any abnormalities.

After discussing with the parents and with their consent, excisional biopsy of the mass was done

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under local anaesthetic. A good view was obtained using a 0 nasal endoscope attached to the Olympus video system. Proper hemostasis was achieved after packing the excised site with merosel nasal pack for 30 minutes.

He was followed up after 2 weeks and there was no more history of nasal bleeding or blockage. A repeat nasal examination revealed a normal mucosa with no blood clots or mass seen. 50 Histopathological examination of the specimen demonstrated features of capillary hemangioma. A review after 1 year showed no recurrence and he has no more epistaxis. He was also in remission for his acute lymphoblastic leukemia.

DISCUSSION

Capillary hemangioma is made up of small, narrow vascular channels lined by a single flat layer of endothelium and is surrounded by variable amounts of fibrous connective tissue which conform to the calibre of the normal capillaries. In contrast to cavernous hemangiomas that are formed by large cavernous vascular channels. The malformation results from a proliferation of arterial and venous vessels of various sizes with fistula formation between them. Lobular capillary hemangioma (LCH) is a common polypoid form of capillary hemangioma that occurs on mucosal surfaces such as the

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oral and nasal cavity, the tongue, the conjunctiva, the duodenum or the colon. The most common presenting symptom of LCH is bleeding and/or obstructive symptoms in the case of LCH of the nasal cavity3 .

LCH is a rapidly growing benign lesion of obscure origin. The disease has been described under a variety of synonyms including pyogenic granuloma, telangiectatic granuloma, granuloma pedunculatum or infected granuloma. The most frequent term applied in the literature is `pyogenic granuloma'. The pathogenesis of LCH is uncertain. In early descriptions, as the name pyogenic granuloma implies, it was regarded as a non-specific response to pyogenic organisms. The lesion is normally rapidly growing, painless, and single with a tendency to bleed when traumatized. Often the mass is ulcerated and covered with white to yellow exudate. The size of the lesion usually correlates with the duration and may range from few millimeters to several centimetres 4 .

Epistaxis is a common symptom and recurrent nasal clarification of the

bleeding requires precise

cause and exclusion diagnostics prior to therapy planning. It is important here to distinguish between locally induced bland epistaxis and symptomatic epistaxis(5) .

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Although there are various ways to treat and remove this lesion, the treatment of choice of LCH is still conservative local excisional biopsy4 . Local cautery of the base is advocated for haemostasis, as well as to decrease the capacity for recurrence. Electrocoagulation, cryotherapy and laser therapy have also been reported in the successful management of these lesions. Nd:YAG laser has the well-recognized advantages of laser in removing vascular lesions (precision, haemostasis, reduced oedema and inflammation and wound sterilization).

The clinical course of LCH is usually benign following local excision of the lesion, although severe bleeding can occur and recurrences have been reported4 .

REFERENCES

1. Anthony H, Ali R, Jeffrey PL, et al. 1999. Management of congenital subglottic hemangioma. The Journal of Otolaryngology. 28: 223-8. 2. Paula EN, Milton W, Charles AJ, hemangioma: A et al. 2001. Congenital nonprogressive

distinct clinicopathologic entity unlike infantile hemangioma. Archieves of Dermatology. 137: 1607-21. 3. Sarosh I, Thomas B, Madeleine P, hemangioma: A et al. 2003. Tracheal lobular capillary

rare cause of recurrent hemoptysis. Chest. 123: 2148-9. 4. Yousry ES, Awad AS. 1997. Lobular capillary hemangioma (pyogenic granuloma) of the 29

nose. The Journal of Laryngology and Otology. 111:941-6. 5. Marietta H, Jurgen UGH, Ewa R, et al. 2002. Endoscopically controlled laser therapy of recurrent epistaxis with the 940 nm diode laser. Medical Laser Application. 17: 231-42.

Corresponding Author: Dr Baharudin Abdullah, Department of Otolaryngology - Head and Neck Surgery (ORL-HNS), School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian 16150, Kelantan, Malaysia.

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