Case Report ORBITAL CAVERNOUS HEMANGIOMA IN AN INFANT WITH INTRACRANIAL LESIONS: A CASE REPORTEleni Evagelidou1, Elena Tsanou2*, Ioannis Asproudis3, Spiridon Gorezis4, Miltiadis Aspiotis1, Dimitrios Peschos2 and Antigoni Siamopoulou1* Corresponding author: Elena Tsanou etsanu@cc.uoi.grAuthor Affiliations1 Pediatric Clinic, University Hospital of Ioannina, Panepistimiou Avenue, Ioannina, 45 500, Greece 2 Cytology-Pathology Department, University Hospital of Ioannina, Panepistimiou Avenue, Ioannina, 45 500, Greece 3 Eye Clinic, University Hospital of Ioannina, Panepistimiou Avenue, Ioannina, 45 500, Greece 4 Epitrus Vision Center, Panepistimiou Avenue, Ioannina, 45 500, Greece For all author emails, please log on. Cases Journal 2009, 2:6912 doi:10.4076/1757-1626-2-6912The electronic version of this article is the complete one and can be found online at: http://www.casesjournal.com/content/2/1/6912

Received:26 March 2009

Accepted:16 June 2009

Published:11 September 2009

2009 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/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

AbstractIntroductionCavernous hemangiomas of the orbit are benign vascular malformations, commonly encountered in adults. Although they are infrequent in pediatric population their diagnosis and course are of a great significance, mainly because they can cause visual disturbances such as amblyopia that can ensue, and secondarily due to their cosmetic and psychological effect. Special attention is required in follow up and treatment. Additionally, a systemic evaluation is necessary in order to discover asymptomatic lesions elsewhere in the body carrying a risk of complications. Case presentationThe authors describe the clinical course, diagnosis, therapeutic approach and prognosis of an infant with an orbital cavernous hemangioma accompanying intracranial lesions. A female infant 18 months of age, presented with a mass in the left upper eyelid, causing blepharoptosis. Preoperative magnetic resonance imaging and angiography of the brain and the orbits showed a hemangioma of the left upper eyelid and intracranial lesions to the left temporal fossa and the pons. At the age of 2 years and 8 months she was admitted again due to severe eyelid swelling, intense strong pain, exophthalmos and collateral ophthalmoplegia. Two operations were performed to remove the orbit mass. Histological examination, showed characteristics of cavernous hemangioma. ConclusionThe atypical presentation of cavernous orbital hemangioma with early infantile onset, merits attention. IntroductionCavernous hemangiomas are uncommon vascular malformations located in the brain [1]. They are usually encountered in the orbit as primary tumours in adults [1,2]. Patients with orbital cavernous hemangiomas typically present in the fourth and fifth decade of life [1,2]. Lesions occurring under the age of 20 and especially in infants are rare [1]-[4]. We describe a case of a female infant with an orbital cavernous hemangioma and accompanying intracranial lesions, which was implicated with serious thrombosis, optic nerve atrophy, severe visual loss and strabismus. Case presentationA Greek female infant, 18 months of age, was referred to the pediatric clinic for evaluation of a mass in the left upper eyelid. According to the patient's perinatal and medical history, she was born after a 36-weeks twin pregnancy, following in-vitro fertilization (IVF), with a birth weight of 2.200 g, small-for-gestational age. There was no positive family history of hemangiomas. There were no indications of deficiency in her developmental status. Also there was no history of trauma, seizures or focal neurological deficits. The physical examination revealed no pathologic findings with the exception of a soft and painless mass in the left upper eyelid, causing incomplete closure of the eyelid and only mild irregular discoloration of the lid skin. An increase in tumor volume and depth of bluish hue when the child cried were noticeable. Also there was a small subcutaneous mass in the upper lip and a caf-au-lait spot with smooth margins on the right leg. No other skin spots were observed. The ophthalmological evaluation showed that both eyes were equal in size and eye motility was normal. No strabismus was recognized. Direct ophthalmoscopy showed no abnormality in either anterior segment. The fundus examination in both eyes was normal. Skiascopic examination revealed no particular refractive errors, except for a small degree of hyperopia. Subsequent examination of the skin and magnetic resonance imaging (MRI) of the brain and the orbits (with T1 and T2 weighted sequences) and magnetic resonance angiography (MRA) were performed. A lesion resembling cavernous hemangioma was found in the superior part of the left orbit (Figure 1). The lesion was located between the roof of the orbit and the superior rectus muscle. The lesion was dyed with gadolinium approximately 13 min after infusion of the agent (Figure 2). No dilatation of the collateral ophthalmic vein was observed. A similar lesion was found intracranially located in the left temporal fossa, extending to the collateral cavernous sinus up to the lateral wall of the fourth ventricle through the cerebellopontine angle. This finding resembled a cavernous angioma. No pathologic findings were detected in cerebellar and cerebral hemispheres. The lesion was also extending to the pons. A similar lesion was found in the upper lip. No signs of hemangioma or other pathologic findings were noted in the abdomen, pelvis and post-peritoneum by ultrasonography. Furthermore there was no evidence for presence of other diseases such as type-1 neurofibromatosis, Sturge-Weber syndrome, Blue rubber bleb nevus syndrome or von Hippel-Lindau syndrome.

Figure 1. Cavernous hemangioma of the left upper eyelid on a sagittal T1 weighted MRI.

Figure 2. Cavernous hemangioma of the left upper eyelid in the same patient, on an axial T2- weighted MRI, after intravenous injection of Gadolinium.

By 23 months of age, our patient required hospitalization due to increasing size of the orbital hemangioma causing severe reduction of the palpebral fissure. A second MRI of the brain and orbits revealed increasing in size of the hemangioma in its lateral palpebral part, especially in the part localized outside the orbit. There were no alterations in the intracranial lesions. At the age of 2 years and 8 months, our patient was admitted again due to sudden and severe left upper eyelid swelling, intense pain and exophthalmos in the left eye. Clinical examination revealed alterations of ocular motility and pupil dilation, while imaging suggested the presence of intraorbital hemorrhage. The patient was rushed to surgery on order to decompensate the orbit and protect the optic nerve. An anterior surgical approach was performed through the superior orbital rim allowing entrance to the roof of the orbit and removal of a dark red mass. On histological examination, the lesion proved to be a cavernous hemangioma (Figure 3). Large anastomosing vascular spaces were observed filled with blood and separated by fibrous stroma.

Figure 3. Large vascular spaces lined by endothelial cells. Note the red blood cells at the periphery of the lumen.The excision proved to be incomplete and the postoperative course was not uneventful. The patient presented with consistent eyelid swelling, exophthalmos and collateral ophthalmoplegia. A second operation was done in order to remove the remnants of the cavernous hemangioma, twenty days postoperatively. Two years after the operations, the patient shows mild palpebral ptosis, visual acuity of 1/20- 1/10 (left eye) and 10/10 (right eye) in the Snellen optotype, total atrophy of the left optic nerve and small angle divergent strabismus. DiscussionCavernous hemangiomas are non-infiltrative, low-flow hamartomas, usually observed in adults and thought to be benign [5]. In the orbit, they represent 80% of adult angiomatous malformations, accounting for 9.5% to 15% of primary orbital expanding masses [1,5]. Orbital cavernomas have been reported to appear with symptoms at an average age of 42 years, ranging from 18 to 67 years [6]. Intracranial and orbital cavernous hemangiomas in children, especially in infants, are rare [4]. The prevalence of cerebral cavernous malformations in children is estimated to be between 0.37% and 0.53% [7]. Multiple lesions in a single orbital cavity or simultaneously occurring in both orbits have been reported, while a familial form of orbital cavernous angiomas have also been described [6,8]. The incidence of familial cerebral cases has been estimated to be close to 20% in the literature, and the pattern of inheritance is consistent with an autosomal dominant mode with incomplete clinical penetration and possible de novo mutation [7]. In our patient there was a combination of early age of onset (infancy), cavernous hemangiomas in orbit and lip, cerebral cavernoma with lesions in left temporal fossa, left cavernous sinus and pons, and asymptomatic clinical profile in relation to cerebral angiomas. The examinations for hemangiomas elsewhere in the body and the familial history of clinical noticeable hemangiomas were negative. The pathogenesis of orbital cavernous angiomas is uncertain [1]. Gardner has postulated that cavernous hemangiomas grow slowly possibly from a pre-existing vascular hamartomas by intracapillary endothelial hyperplasia and they are relatively isolated from the systemic circulation [9]. Harris and Jackobiec suggested that these angiomas are acquired lesions that probably begin as capillary-type proliferation, becoming cavernous spaces through a progressive ectasia [1,5]. The natural history of orbital cavernous hemangiomas may be quite variable. It may be that of benign tumours causing progressive clinical manifestations from mass effect due to their slow growth or sometimes they may not grow after a certain age or remain asymptomatic [2,5,11]. The reported stimulation in their growth during pregnancy seems to suggest some hormonal influence on their natural evolution [12]. Children with an infantile hemangioma in the eyelid and orbit are at risk of a variety of ocular problems including functional amblyopia, strabismus, proptosis and optic nerve compression [13,14]. The mal-positioned lids may lead to the development of amblyopia from partial or complete occlusion of the visual axis. The tumor may also interfere with normal visual function by leading to astigmatism from pressing on the eye and altering the curvature of the cornea. Myopia and strabismus may secondarily relate to these hemangiomas as well [1,13]-[14]. On occasion, symptoms of orbital pain, eyelid swelling, diplopia and amaurosis can occur. Patients may refer orbital pain or headache, and choroidal folds or retinal striae, as well as optic disc edema may also be seen [1][6]. Orbital cavernous hemangiomas have no tendency to cause hemorrhages, and acute clinical onset is rare [1]. In our case, the patient ended two years after the operation to show small angle divergent strabismus and atrophy of the left optic nerve. This atrophy may be the result of the operative procedure or result of the compression of the optic nerve or its vascular supply by the tumor. The diagnosis and follow up imaging of cavernous angiomas (intracranial and orbital cavernous angiomas) are best provided by MRI [7]. In our pediatric patient, MRI scanning including T1-, T2-contrast-enchanced and T1-, T2-weighted sequences were done. Also MRA was performed in order to assess the anatomical rapport between cavernomas and the normal arterial vessels. Cavernous angiomas are well known to be angiographically silent, perhaps due either to the small caliber of the feeding arteries and their slow circulation causing dilution of contrast medium, to extensive thrombosis or to a combination of both [4]. Various methods have been used to treat infantile hemangiomas of the eyelid and orbit. According to Herter et al. [6], operative treatment is recommended particularly in patients with true visual loss secondary to optic nerve compression, severe bulbar displacement and constant growth tendency. The choice of management in cases of cerebral cavernous angiomas must take into account both the possible natural evolution of the lesions and the risk of surgery. Therapeutic strategies for cerebral cavernomas take into account age, sex, location of the lesions, the efficacy of medical treatment for patient's secondary epilepsy and risk factors for severe potentially life-threatening hemorrhage [7]. In children, surgery is clearly indicated in case of acute hemorrhage or focal neurological deficit [7]. It is especially recommended for infratentorial lesions, even if they are clinically silent, due to their high risk of bleeding. However, the surgical indication must be always discussed for each case individually ConclusionCavernous hemangiomas of the orbit are benign vascular malformations, commonly encountered in adults. Although they are infrequent in pediatric population their diagnosis and course are of a great significance, mainly because they can cause visual disturbances such as amblyopia that can ensue, and secondarily due to their cosmetic and psychological effect. Special attention is required in follow up and treatment. Additionally, a systemic evaluation is necessary in order to discover asymptomatic lesions elsewhere in the body carrying a risk of complications. AbbreviationsIVF: in-vitro fertilization; MRA: magnetic resonance angiography; MRI: magnetic resonance imaging. ConsentWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editor in chief of this journal. Competing interestsThe authors declare that they have no competing interests.Authors' contributions"EE was a major contributor in writing the paper; ET performed the histologic examination; IA analysed and interpreted the visual data of the patient; SG contributed to final revision of the paper; MA provided the final approval for the version to be published, DP provided the histological images and AS concepted and designed the study". References1. Acciarri N, Giulioni M, Padovani R, Gaist G, Pozzati E, Acciarri R: Orbital cavernous angiomas: surgical experience on a series of 13 cases. J Neurosurg Sci 1995, 39:203-209. PubMedAbstract 2. Orcuitt JC, Wulc AE, Mills RP, Smith CH: Asymptomatic orbital cavernous hemangiomas. Ophthalmology 1991, 98:1257-1260. PubMedAbstract 3. Castilo BV, Kaufman L: Pediatric tumors of the eye and orbit. Pediatr Clin N Am 2003, 50:149-172. PubMedAbstract | PublisherFullText 4. Yamasaki T, Handa H, Yamashita J, Paine J, Tashiro Y, Uno A, Ishikawa M, Asato R: Intracranial and orbital cavernous angiomas. A review of 30 cases. J Neurosurg 1986, 64:197-208. PubMedAbstract | PublisherFullText 5. Harris G, Jakobiec F: Cavernous hemangioma of the orbit. J Neurosurg 1979, 51:219-228. PubMedAbstract | PublisherFullText 6. Herter T, Bennefeld H, Brandt M: Orbital cavernous hemangiomas. Neurosurg Rev 1988, 11:143-147. PubMedAbstract | PublisherFullText 7. Mottolose C, Hermier M, Stan H, Jouvet A, Saint-Pierre G, Froment J-C, Bret P, Lapras C: Central nervous system cavernomas in the pediatric age group. Neurosurg Rev 2001, 24:55-71. PubMedAbstract | PublisherFullText 8. Sarraf D, Payne A, Kitchen N, Sehmi K, Downes S, Bird A: Familial cavernous hemangioma. Arch Ophthalmol 2000, 118:969-973. PubMedAbstract | PublisherFullText 9. Gardner A: Cavernous hemangioma of the orbit. A consideration of its origin and development. Orbit 1988, 3:149-156. | 10. Pozzati E, Giuliani G, Nuzzo G: The growth of cerebral cavernous angiomas. Neurosurgery 1989, 25:92-97. PubMedAbstract | PublisherFullText 11. Henderson JW, Farrow GM, Garrity JA: Clinical course of an incompletely removed cavernous hemangioma of the orbit. Ophthalmology 1990, 97:625-628. PubMedAbstract | PublisherFullText 12. Zaubermann LI, Feinsold M: Orbital hemangioma growth during pregnancy. Acta Ophthalmol 1979, 48:929-933. PubMedAbstract | PublisherFullText 13. Stigmar G, Crawford J, Ward C, Thomson H: Ophthalmic sequelae of infantile hemangiomas of the eyelids and orbit. Am J Ophthalmol 1978, 85:806-813. PubMedAbstract | PublisherFullText 14. Nicholson DH, Green WR: Pediatric Ophthalmology. 3rd edition. Edited by Nelson LBCalhoun JHHarley RD. WB Saunders Co; 1991:382-420.

Laporan Kasus HEMANGIOMA KAVERNOSA ORBITAL PADA BAYI DENGAN LESI INTRAKRANIAL: LAPORAN KASUSEleni Evagelidou 1 , Elena Tsanou 2 *, Ioannis Asproudis 3 , Spiridon Gorezis 4 , Miltiadis Aspiotis 1 , Dimitrios Peschos 2 dan Antigoni Siamopoulou 1* Penulis Sesuai: Elena Tsanou etsanu@cc.uoi.grPenulis Afiliasi 1 Pediatric Clinic, Rumah Sakit Universitas Ioannina, Panepistimiou Avenue, Ioannina, 45 500, Yunani 2 Sitologi-Departemen Patologi, University Hospital of Ioannina, Panepistimiou Avenue, Ioannina, 45 500, Yunani 3 Klinik Mata, Rumah Sakit Universitas Ioannina, Panepistimiou Avenue, Ioannina, 45 500, Yunani 4 Epitrus Visi Pusat, Panepistimiou Avenue, Ioannina, 45 500, Yunani Untuk semua email penulis, silakan masuk . Kasus Journal 2009, 2: 6912 doi: 10,4076 / 1757-1626-2-6912 Versi elektronik dari artikel ini adalah salah satu yang lengkap dan dapat ditemukan secara online di: http://www.casesjournal.com/content/2/1/6912

Diterima: 26 Maret 2009

Diterima: 16 Juni 2009

Diterbitkan: 11 Sep 2009

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Abstrak Pengenalan Hemangioma kavernosa orbit yang malformasi vaskular jinak, biasa ditemui pada orang dewasa. Meskipun mereka jarang pada populasi pediatrik diagnosis dan tentu saja mereka dari sangat penting, terutama karena mereka dapat menyebabkan gangguan penglihatan seperti amblyopia yang dapat terjadi, dan yang kedua karena efek kosmetik dan psikologis mereka. Perhatian khusus diperlukan dalam tindak lanjut dan pengobatan. Selain itu, evaluasi sistemik diperlukan untuk menemukan lesi asimtomatik tempat lain dalam tubuh membawa risiko komplikasi. Presentasi Kasus Para penulis menjelaskan saja, diagnosis, pendekatan terapi klinis dan prognosis bayi dengan hemangioma kavernosa lesi intrakranial yang menyertainya orbital. Seorang bayi perempuan usia 18 bulan, disajikan dengan massa di kelopak mata bagian atas kiri, menyebabkan blepharoptosis. Pra operasi MRI dan angiografi otak dan orbit menunjukkan hemangioma dari kelopak mata atas kiri dan lesi intrakranial ke fossa temporalis kiri dan pons. Pada usia 2 tahun dan 8 bulan dia dirawat lagi karena pembengkakan parah kelopak mata, rasa sakit yang kuat intens, exophthalmos dan jaminan oftalmoplegia. Dua operasi dilakukan untuk menghilangkan massa orbit. Pemeriksaan histologis, menunjukkan karakteristik hemangioma kavernosa. Kesimpulan Presentasi atipikal hemangioma kavernosa orbital dengan onset infantile awal, perhatian manfaat. Pengenalan Hemangioma kavernosa yang malformasi vaskular jarang berada di otak [ 1 ]. Mereka biasanya dihadapi dalam orbit sebagai tumor primer pada orang dewasa [ 1 , 2 ]. Pasien dengan hemangioma kavernosa orbital biasanya hadir dalam dekade keempat dan kelima dari kehidupan [ 1 , 2 ]. Lesi yang terjadi di bawah usia 20 - dan terutama pada bayi jarang terjadi [ 1 ] - [ 4 ]. Kami menggambarkan kasus bayi perempuan dengan hemangioma kavernosa orbital dan lesi intrakranial yang menyertainya, yang terlibat dengan trombosis serius, optik atrofi saraf, kehilangan penglihatan berat dan strabismus. Presentasi Kasus Seorang bayi perempuan Yunani, usia 18 bulan, dirujuk ke klinik pediatrik untuk evaluasi massa di kelopak mata atas kiri. Menurut sejarah perinatal dan medis pasien, dia lahir setelah kehamilan kembar 36-minggu, setelah fertilisasi in-vitro (IVF), dengan berat lahir dari 2.200 g, kecil-untuk-kehamilan usia. Tidak ada riwayat keluarga yang positif dari hemangioma. Tidak ada indikasi kekurangan dalam status perkembangan nya. Juga tidak ada riwayat trauma, kejang atau defisit neurologis fokal. Pemeriksaan fisik menunjukkan tidak ada temuan patologis dengan pengecualian massa lembut dan tidak menyakitkan di kelopak mata bagian atas kiri, menyebabkan penutupan lengkap dari kelopak mata dan hanya perubahan warna yang tidak teratur ringan kulit kelopak. Peningkatan volume tumor dan kedalaman warna kebiruan ketika anak menangis yang terlihat. Juga ada massa subkutan kecil di bibir atas dan caf-au-lait spot dengan margin halus di kaki kanan. Tidak ada bintik-bintik kulit lainnya yang diamati. Evaluasi oftalmologi menunjukkan bahwa kedua mata yang sama dalam ukuran dan motilitas mata normal. Tidak ada strabismus diakui. Oftalmoskopi langsung menunjukkan tidak ada kelainan baik segmen anterior. Pemeriksaan fundus di kedua mata normal. Pemeriksaan Skiascopic mengungkapkan tidak ada kesalahan bias tertentu, kecuali untuk tingkat kecil hyperopia. Pemeriksaan berikutnya dari kulit dan magnetic resonance imaging (MRI) otak dan orbit (dengan T1 dan T2 urutan tertimbang) dan magnetic resonance angiography (MRA) dilakukan. Sebuah lesi menyerupai hemangioma kavernosa ditemukan di bagian superior dari orbit kiri (Gambar 1 ). Lesi terletak di antara atap orbit dan otot rektus superior. Lesi ini diwarnai dengan gadolinium sekitar 13 menit setelah infus agen (Gambar 2 ). Tidak ada dilatasi vena mata agunan diamati. Sebuah lesi serupa ditemukan intracranially terletak di fossa temporal kiri, memperluas ke sinus kavernosus agunan hingga dinding lateral ventrikel keempat melalui sudut cerebellopontine. Temuan ini menyerupai angioma kavernosa. Tidak ada temuan patologis terdeteksi di belahan serebelum dan otak. Lesi juga memperluas ke pons. Sebuah lesi serupa ditemukan di bibir atas. Tidak ada tanda-tanda hemangioma atau temuan patologis lainnya yang dicatat dalam perut, panggul dan pasca-peritoneum dengan ultrasonografi. Selain itu tidak ada bukti untuk adanya penyakit lain seperti jenis-1 neurofibromatosis, sindrom Sturge-Weber, Biru karet sindrom nevus bleb atau sindrom von Hippel-Lindau.

Gambar 1. kavernosa hemangioma dari kelopak mata atas kiri pada T1 sagital -weighted MRI.

Gambar 2. kavernosa hemangioma dari kelopak mata atas kiri pada pasien yang sama, pada aksial T2- tertimbang MRI, setelah injeksi intravena Gadolinium.

Dengan 23 bulan usia, pasien kami diperlukan rawat inap karena meningkatnya ukuran hemangioma orbital menyebabkan penurunan berat dari fisura palpebra. Sebuah MRI kedua otak dan orbit mengungkapkan peningkatan ukuran hemangioma di bagian palpebra lateral, terutama di bagian lokal luar orbit. Tidak ada perubahan dalam lesi intrakranial. Pada usia 2 tahun dan 8 bulan, pasien kami dirawat lagi karena tiba-tiba dan parah kiri bengkak atas kelopak mata, rasa sakit dan exophthalmos di mata kiri. Pemeriksaan klinis menunjukkan perubahan motilitas okular dan pelebaran pupil, sementara pencitraan menunjukkan adanya perdarahan intraorbital. Pasien dilarikan ke operasi pada rangka dekompensasi orbit dan melindungi saraf optik. Pendekatan bedah anterior dilakukan melalui rim orbital superior memungkinkan masuk ke atap orbit dan penghapusan massa merah gelap. Pada pemeriksaan histologis, lesi terbukti menjadi hemangioma kavernosa (Gambar 3 ). Ruang vaskular anastomosing besar diamati penuh dengan darah dan dipisahkan oleh stroma fibrosa.

Gambar 3. ruang vaskular besar dilapisi oleh sel endotel. Perhatikan sel darah merah di pinggiran lumen.Eksisi terbukti menjadi tidak lengkap dan tentu saja pasca operasi tidak lancar. Pasien disajikan dengan pembengkakan kelopak mata yang konsisten, exophthalmos dan jaminan oftalmoplegia. Operasi kedua dilakukan untuk menghilangkan sisa-sisa hemangioma kavernosa, dua puluh hari pasca operasi. Dua tahun setelah operasi, pasien menunjukkan ptosis palpebral ringan, ketajaman visual dari 1 / 20- 1/10 (mata kiri) dan 10/10 (mata kanan) di optotype Snellen, jumlah atrofi saraf optik kiri dan sudut kecil strabismus divergen. Diskusi Hemangioma kavernosa yang non-infiltratif, hamartomas aliran rendah, biasanya diamati pada orang dewasa dan dianggap jinak [ 5 ]. Dalam orbit, mereka mewakili 80% dari malformasi angiomatous dewasa, akuntansi untuk 9,5% sampai 15% dari massa utama memperluas orbital [ 1 , 5 ]. Cavernomas Orbital telah dilaporkan muncul dengan gejala pada usia rata-rata 42 tahun, mulai 18-67 tahun [ 6 ]. Hemangioma kavernosa intrakranial dan orbital pada anak-anak, terutama pada bayi, jarang terjadi [ 4 ]. Prevalensi malformasi gua otak pada anak-anak diperkirakan antara 0,37% dan 0,53% [ 7 ]. Beberapa lesi pada rongga orbital tunggal atau bersamaan terjadi di kedua orbit telah dilaporkan, sementara bentuk familial dari angioma kavernosa orbital juga telah dijelaskan [ 6 , 8 ]. Insiden kasus cerebral familial telah diperkirakan mendekati 20% dalam literatur, dan pola pewarisan konsisten dengan modus dominan autosomal dengan penetrasi klinis tidak lengkap dan mungkin mutasi de novo [ 7 ]. Pada pasien kami ada kombinasi dari usia dini onset (bayi), hemangioma kavernosa di orbit dan bibir, cavernoma otak dengan lesi di fossa meninggalkan temporal, meninggalkan sinus kavernosa dan pons, dan profil klinis asimtomatik dalam kaitannya dengan angioma serebral. Pemeriksaan untuk hemangioma di tempat lain di tubuh dan sejarah keluarga dari hemangioma terlihat klinis negatif. Patogenesis angioma kavernosa orbital tidak pasti [ 1 ]. Gardner telah mendalilkan bahwa hemangioma kavernosa tumbuh lambat mungkin dari hamartomas vaskular yang sudah ada dengan hiperplasia endotel intracapillary dan mereka relatif terisolasi dari sirkulasi sistemik [ 9 ]. Harris dan Jackobiec menyarankan bahwa angioma ini diperoleh lesi yang mungkin mulai sebagai kapiler-jenis proliferasi, menjadi ruang gua melalui ectasia progresif [ 1 , 5 ]. Sejarah alam hemangioma kavernosa orbital mungkin cukup bervariasi. Mungkin tumor jinak menyebabkan manifestasi klinis yang progresif dari efek massa karena pertumbuhan yang lambat atau kadang-kadang mereka mungkin tidak tumbuh setelah usia tertentu atau tetap asimtomatik [ 2 , 5 , 11 ]. Stimulasi yang dilaporkan dalam pertumbuhan mereka selama kehamilan tampaknya menunjukkan pengaruh hormonal pada evolusi alami mereka [ 12 ]. Anak-anak dengan hemangioma kekanak-kanakan dalam kelopak mata dan orbit beresiko berbagai masalah mata termasuk amblyopia fungsional, strabismus, proptosis dan kompresi saraf optik [ 13 , 14 ]. Tutup mal-diposisikan dapat menyebabkan perkembangan amblyopia dari oklusi parsial atau lengkap dari sumbu visual. Tumor juga dapat mengganggu fungsi visual normal dengan mengarah ke Silindris dari menekan pada mata dan mengubah kelengkungan kornea. Miopia dan strabismus mungkin sekunder berhubungan dengan hemangioma ini juga [ 1 , 13 ] - [ 14 ]. Pada kesempatan, gejala nyeri orbital, pembengkakan kelopak mata, diplopia dan amaurosis dapat terjadi. Pasien dapat merujuk nyeri orbital atau sakit kepala, dan lipatan choroidal atau striae retina, serta optik edema disk juga dapat dilihat [ 1 ] [ 6 ]. Hemangioma kavernosa Orbital tidak memiliki kecenderungan untuk menyebabkan perdarahan, dan onset klinis akut jarang [ 1 ]. Dalam kasus kami, pasien berakhir dua tahun setelah operasi menunjukkan sudut kecil strabismus divergen dan atrofi saraf optik kiri. Atrofi ini mungkin hasil dari prosedur operasi atau hasil dari kompresi saraf optik atau pasokan vaskular yang oleh tumor. Diagnosis dan tindak lanjut pencitraan angioma kavernosa (intrakranial dan angioma kavernosa orbital) yang terbaik disediakan oleh MRI [ 7 ]. Pada pasien anak kami, MRI scanning termasuk T1, T2-kontras-enchanced dan T1, urutan T2-tertimbang dilakukan. Juga MRA dilakukan untuk menilai hubungan antara anatomi cavernomas dan pembuluh arteri yang normal. Angioma kavernosa dikenal sebagai angiografi diam, mungkin karena baik untuk kaliber kecil dari arteri makan dan sirkulasi lambat menyebabkan dilusi mereka media kontras, trombosis yang luas atau kombinasi keduanya [ 4 ]. Berbagai metode telah digunakan untuk mengobati hemangioma infantil dari kelopak mata dan orbit. Menurut Herter et al. [ 6 ], pengobatan operatif dianjurkan terutama pada pasien dengan hilangnya penglihatan benar sekunder untuk kompresi saraf optik, perpindahan bulbar parah dan kecenderungan pertumbuhan konstan. Pilihan manajemen dalam kasus angioma kavernosa otak harus memperhitungkan evolusi alami kemungkinan lesi dan risiko operasi. Strategi terapi untuk cavernomas otak memperhitungkan usia, jenis kelamin, lokasi lesi, kemanjuran pengobatan untuk epilepsi dan faktor risiko sekunder pasien untuk parah perdarahan berpotensi mengancam nyawa [ 7 ]. Pada anak-anak, operasi jelas ditunjukkan dalam kasus perdarahan akut atau defisit neurologis fokal [ 7 ]. Hal ini terutama direkomendasikan untuk lesi infratentorial, bahkan jika mereka secara klinis diam, karena risiko tinggi perdarahan. Namun, indikasi bedah harus selalu dibahas untuk setiap kasus individual Kesimpulan Hemangioma kavernosa orbit yang malformasi vaskular jinak, biasa ditemui pada orang dewasa. Meskipun mereka jarang pada populasi pediatrik diagnosis dan tentu saja mereka dari sangat penting, terutama karena mereka dapat menyebabkan gangguan penglihatan seperti amblyopia yang dapat terjadi, dan yang kedua karena efek kosmetik dan psikologis mereka. Perhatian khusus diperlukan dalam tindak lanjut dan pengobatan. Selain itu, evaluasi sistemik diperlukan untuk menemukan lesi asimtomatik tempat lain dalam tubuh membawa risiko komplikasi. Singkatan IVF: fertilisasi in-vitro; MRA: magnetic resonance angiography; MRI: MRI. Persetujuan Informed consent tertulis diperoleh dari pasien untuk publikasi dari laporan kasus ini dan gambar yang menyertainya. Salinan persetujuan tertulis tersedia untuk ditinjau oleh redaksi jurnal ini. Bersaing kepentingan Para penulis menyatakan bahwa mereka tidak memiliki kepentingan bersaing. Kontribusi penulis ' "EE adalah penyumbang utama dalam menulis kertas; ET dilakukan pemeriksaan histologis, IA dianalisis dan diinterpretasikan data visual pasien; SG berkontribusi revisi akhir dari kertas; MA memberikan persetujuan akhir untuk versi yang akan diterbitkan, DP memberikan gambar histologis dan AS terkonsep dan dirancang studi ".

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