MR Conte Fig. 7.12 4a Lf upper eyelid focal besangioma causing stgmativn. (b) Let upper eyed hemangioma The hil pprcola wih complet Wewl axe chemction TIntraconal IH that involves the posterior orbit could result in proptosis, displacement of the lobe, or visual axis obstruction (24, 25). Another relatively common complication of IH is lacrimal duct ebstructioa because of pees sure in the region of the lacrimal duct, esulting in increased tearing and recurrent conjunctivitis. subglotis, and laryngeal localization represents 1L5 % of congenital lesions of the larynx [29] (Fig. 7.134, 0). ‘Most of the subglottic hemangiomas become ‘symptomatic during the frst year of life. They are located in the posterior of lateral subglotic space, and besides hoarseness, dyspmes, and ‘feeding difficulties, they produce atypical grad- uly worsening croupous stridor as the mainTable 71 Hemangioma highaise locations ‘Location Complication Eyelid Functiveal imparmene amblyopia Ear Functoeal impairment ea ret Functional mpuirmcelaimeay involvement Fehon and nasal tip Diaigurement eed et eceeeeinast Ueemton [Large segmental facial and scalp aren HACE syndrrmeviscealbernangfomas [Lange segmental urbosacal nes ‘complications such as ulceration, bleeding, risk {or permanent disfigurement, compromised organ function, vision and airway obstruction, of high- ‘ouput cardiac failure. In these eases, quick active and aggressive therapeutic intervention is manda- tory (7,8, 18] (Table 7.1). Ulceration ‘This occurs during the proliferation pase in 15 % of TH G-4 moms of age) however it could occur in pecursoe lesions prior tothe development of TH. ‘The most commonly affected areas include the anogenital area, head, and neck, particularly ‘he Lips and pesioeal and interuiginous areas: nev- ertheless TH on any site could be ulcerated (Fig. 7.11). The ulcer could persist for several weeks or months and result in pain, bleeding, infection, and sometimes permanent scarring [10]. The causes of ulceration are understood the factors that may play a role are friction, macera- tion, or both. Ulceration is mae frequent ia ‘mixed or supecficial and larger segmental heman- ‘giomas than in the Focal subtype [13-21] Visual Compromise Pesioybital hemangiomas most often involve the upper Lid, bu the lower lid and retrobulhar space are also common. We can define the anatomic location ofthe ocular hemangiomas as preseptl, cextraconal, and intracoaal [22,23]. lhe lesions that affoct the Visual axis may cause amblyopia, and that is the complication of most concer (Fig. 7.128, 0). Spial dysrahinm, anogenital genital sberlin Fig. 2.11 Ulcer hemangioens in diaper sea -Amblyopia result from different smechanisrns 1. Anisometropia (there is a refractive differ. ‘nce between the eyes): In the case of heman- siomas, astigmatism is usually secondary to pressure from the tumor on the globe, which causes an imegular corneal curvature, 2. Strabismic amblyopia can occur ifthe heman- siomas affect the mavement af extrocular muscles. 3. Deprivation amblyopia rising from part oF complete eyelid elorure is particulaly derastat- ingift occurs during the frst few mands of Is, ‘when itcan cause profound visual impaitment, mayFig. 7.7 Facil segmental emangioma assocised with HACE syadrome (he four fil egrets ave parally involved “Indeterminate hemangiomas” were those that ‘wore not readily classified as citer localized or seg- ‘mental, and “multifocal hemangiomas” wore defined ‘as mowe than ten cutaneous hemangiomas [6] ‘Although approximately 60 % of hemangio- ‘mas occur oa the head and neck, they can also ‘eccur in any region. of the body on the trunk (25 9), extremities, and genitals (15 *€) (7. 8] ‘Most present as solitary cutancous andlor subcu- taneous lesions (80-90 §%), and a significant per- centage of patients (15 5¢) have multiple lesions 1,8) Gig. 7) Hemangiomas, whether small and inngevos or Jase and deforming but ae united by 2 predictable iological behavior characterized By theee distinct sages: a rapidly proliferating, high-flow stage (8-12 ‘monihs), followed by & prolonged involuting stage (1-12 years), and finally by a variably prominent end-stage wih Fibrofatty residuum. [3, 5,511] ‘However others leave only residual telangicc- tasias Fig. 7.9) ‘Moat hemangioma growth occurs in the frst 5-9 months, at which point 80 % of te final size thas often been reached [1] ‘The deep and mixed IH with segmental or indeterminate distibution differ in their growth Patter [12]. These TH sometimes appear Later land continue to grow for a longer period than superficial hemangiomas (sometimes ihe pro- onged grovrth has been observed until 2 years of age). Cervicofaeial TH and thase involving the parotid gland are a typical example of TH with this growth patie. This group is known as Fig. 7.8. Multifocal hemangiomas in 3 pien with vs- cel imolerment hemangiomas with prolonged growth phase [12, 15] Fig. 7.103, b. Another subgroup of IH exhibits a patter of ‘minimal growth, and they presented a reticular or {clangioctatic appearance with a proliferative com- ponent predominating inthe periphery consisting Of papules a few millimeters in diameter. They lectively are located in the trunk and limbs and. lumbosacral area [144-16] (Fig. 72). The begin- ning of involution is even mare difficult to predict ‘but usually manifested by changes in color from. bright red toa paler red or gray, with @ whitish diseoloration that occurs always in the center of the Demangioms and extends cenuifugally. Deep. lesions become Less blue and less warm, TH iavo- utes over different periods of time, some ina few ‘years, while others can take several years. ‘TH during the peoliferstive phase is compared. of well-defined masses of capillaries lined byIR Coie Fig. 7.2 Hemangioma with misma or arested promt, Fig. 74 Deep hemangiomas Fig. 75 Mined hemangioma Fig. 76 Segmental hemangioma shape, have large size and involve an anatomic region, and can be reticular or telangicetutic or ‘lat chemry-red patches (6-8] (Fig. 7.0) Four segments or patterns of involvement ccccur in facial area regions. Segmeat | involved the Tateral forehead and temporal and laters] frontal scalp. Segments 2 and 3 correspond tothe maxillary and mandibular, and Segment 4 involved the nose, philtrum, and medial frontal scalp. These segmental hemangiomas tend to be greater thun Som in diameter and mast often span on side of the fave and asck (Fig. 7.7). “Localized hemangiomas” were detined as those hemangiomas that seem to grow from a single focal point or were localized to an area without any apparent linewr or developmental configuration. They are usually aval or round,aw (of RICH clfered from NICH and common infan- tile hemangioma, but some overlap was nated ‘among the thee lesions. RICH is composed of, smullto-large lobules of capillaries with moder ately plump endothelial cells and pericytes; the Joules are surrounded by abundant fibrous ts se, One-half of the specimens have a central lnvoluting zone characterized by lobular loss, fibrous tissue, and draining channels that are often large and abnormal ‘Additionally hemangiomas have unique vascu lar phenotype demoastntd by glucose tanspomter |L(GLUF-) staining marker. Since is fs deseripe tion by PE. North in 2000, its use has become ‘widely spead by clinicians and researchers inthe field of vascular anomulies. Endothoial cells in RICH and other vascular tumors malformations donot express glucose transporter protein [1]. Unfortunately and despite cardinal advances in the diagnosis of hemangiomas, the use of a ‘wrong nomenclature femains as the most fe- quent origin of incomect diagnosis and inappro- priate treatments. References 1, Hand IL, iden L 2000) Nascar ita of infancy: reolving nesolagic sanfusion, Am ¥ Med Genet 10-257 260 Chiles KO, Passo Frieden! (2002) Hemangiamas of infancy: clinical harctesics, mospbalogic sub Iypet and irelaionip tn ace, ys ‘rh Derma 138 1567-1578, Bea LM, EnjlrasO, Mullen (1996) Cangenital hemangioma evidence of acsksted.iovaltinn Peder 2809-35 ‘4 1 JC Lape Guteres Haggsuen AN, Drolet BA, BatelgaE, Chain SL Garon MC, Hla KA, Lucky AM, Mancini Al “ear DW, Newell B, Nopper AJ, Frieden U (2006) Prospective tidy of afte hemangioma: clinical haracertice predicting complications aed tel ‘ment Peis 118(91982 837 Pheden UHaggnears AN, Drolet BA, Mancini A Fiedler Sf, Boon 1, Chamis SU, Bassiga E Garton MC, Nopper Al. Siegel DH Maes EW. Gourd BS, Dechoi? 3, Nori PE, Excly ND (QO) Infante Remangamas: cute’ baomlodgs, fone dreatane.Prceadings of research work ‘Sop on inate Bemangiomas, Apel 79, 2005, Bathends Mayland. USA. Pediat? Dermatol 2215; ae eden 1 Hajoas Q, Eserly N 2003) Vascular ‘chm ind other sbncrmalies of blood wesel ‘nd ymin. ly Schachner LA, Monee RC (ois Podtne. Dentatalogy. 3rd Ed. London. Mosby pp 13 861 Buravs PE (1998) Diagnostic maging inthe alton of vascular Heeas, Demat Cin 16 ‘Boca LM. Fishman SI Land DF, Mlk 1 (1995) Congest Shrvarorne masqueraiog av cangestal hemangioma: poet of tro cases J Pei Sse 30913761361 Maton ME Rabin) Fiend 2018) Sscrocovey geal ‘eramma masquerading 2x congenital bemangcma Padiat Dermatol 1112-118 Phelan Bl, Shab KN, Tayi JA, Rubin AL (2013) Congenialmytbrsma maueratings nue ‘Sate emangioa ins osonate, Pte Dental 30(6 2248-2289 Faden I, Roper M, Garzan MC (2108) Canons masquerading as infnute tmemangioms: pat | ‘Australas J Dermat 802)77-97 Feder I, Ropes Ml, Garson MC (2103) Canine ‘masquerading as infnite emangioms: part 2 ‘Aastalae J Demat SQ3}153- 168 Noni PE, Winer My Mize A, Mien MC Jr (Gio) GLUTI's newly discovered immuno Shemical marker for joveile hemangioma, Ham Patt 3-11-228. Diagnosis of Hemengiomes Fig ‘can assess thelow of hemangiomas, characterized bby a shunt patern with decreased arterial ness tance and increased venous velocity. In deep cculaneous oF visceral hemangiomas, contrast- enhanced MRI demonstrates the extent oF the lesion and helps differentiation between herman ‘slomas and other disorders presenting with sim lar findings on US examination, Hemangiomas hhave atypical solid appearance with intermediate intensity on a Tl-weighted spin-echo image, ‘which is more intense compared with venous of lymphatic malformations. During the prolifer tive suge, hemangiomas show a relatively los! intensity ina T2weighted spin-echo image, ‘while in the imvolution phase, they have a very low intensity. Contrastenfuanced Tieweighted MRI shows moderate intensity with prominent ‘low voids during the proliferative stage because of the high flaw at this stage. In contrast, hema ‘Blomas show low ingeasity during involution as 4 result ofthe low flow at that stage, The appropi ate diagnostic tests for congenital hemangiomas are ultrasonography with Doppler (the lesions are uniformly hypoechoic, mostly confined to the subcutaneous fat and diffusely vascular, being twaversed by multiple tublar vascular channels) ‘and magnetic resonance imaging (RICH bus areas of inhomogeneity and larger flow voids). Angiography is only indicated if embolization ® 3 Non-iwlucing a paral invoking congenial herangioms has to be performed (lnge and imegular feeding arteries in disorganized pattems, arterial anew sys, diet arteriovenous shunts, and ineavas cular thrombi are common features in RICH and are rarely sen in infantile hemangiomas) (7) ‘The type of lesion can usually be determined based on the medical history and clinical exam nation, Imaging is mostly useful for confirming the clinical diagnosis, estimating the extent ofthe lesion and determining the feasibility f surgical resection, ‘The differential diagnosis alsa includes angio sarcoma, glioma (Fig. 8.2), congenttal Sbeasar- coma, infantile myofbramatosis, kaposiform hemangioendothelioms, pyogenic granuloma, {eratoma, tufted angioma, or rhabdomyosarcoma, ‘Frequently. an ultrasound examinatioa of a grow ing deep vascularized tumor in a 2-month-old patient concludes thatthe final diagnosis would probably be “bemungioma,” considering “ean ‘enital Sbrosarcoma” as an alternative option, In those instances, both physicians and. parents consider a biapay ax necessary fora stfer accu rate final diagnosis [8-12] ‘Skin biopsy can be helpful in distinguishing ‘unusual or atypical hemangiomas from ther vas cular lesions. Specimens may be evalusted by ‘routine histological examination and immunohis: ‘ochemical analysis. The histological appearance” 6 oper Gutieree Fig. 8:1 Congeital orbital shabdomyosarcoma (a) and leukemia (h) previously misdiagnosed at hemangiomas, ‘escived an unuccesefal course of peopranalal lg. .2 Ulasound and MRI sndngs were unable 10 detente tbe diagnosis of glioma venus bemangions, [Btcisonal Binpy lea onal agnosis of manor ‘would allow differentiation, In the newborn, lesions may not be evident or may present 8 4 ‘ed, white tclangiectaic, or blue pate. & periph eral rim of pallor due to vasaconstriction may be seen at this stage. As the tumor prolifecates, the lesion may take on the more clastic appearunee, ‘which depends on the depth of lesion and stage of evolution [6 Tncases in which clinical examination lone is inconclusive, other diagnoatic madalities such as Uluasonography and magnetic resonance imag- ing (MRI) may be utilized. Ukrasound DopplerDiagnosis of Hemangiomas Juan Carlos Lopez Gutierrez Up to 90 4% of infantile hemangiomas (1H) involving the epidermis can be easily diagnosed by their typical appearance, while in 10 % ofthe ceaes, deep locaton, abnormal growth pater or visceral involvement makes differential diagno ss necessary ‘Vascular tumors and highly vascularized solid tumors can mimic one another, Mesenchymal hamartomas of the liver of intraconal rhabdb. ‘myosarcomas of the orbit usually proliferate in the first 3 months of life as hemangiomas do, Experienced and a well-trained mulidiscplinary ‘eam are te key to avoid misdiagnosis with even ‘wally fatal consequences (Fig. 8.1). ‘Diagnosis of hemangiomas is based frst on the medical history. Two questions are of pars ‘mount importance: Was the lesion present at birth? Did proportional ‘or dispeopottional growth of the lesion after bint occur? ‘A careful history that includes age of onset, color, and Ioeatio, along with physical examina ‘don findings, is generally suliient in detenmin ing the pathological condition. The presence of the lesion at birth supports the diagnosis of vas cular malformation or congenital hemangioma. Infantile hemangiomas are charucterized by [LC Lapse Gira MDB PRD Deparment of Pine Surgery, \Vacalar Anomalies Cee. 1LsPaz Chale’ s Hp, Madi, Spain im quent balp@ sled nadine postnatal rapid proliferation phase inthe first 6-9 months of life, followed by a slaw involution phase, and in many cases complete repression. Congenital hemangiomas fully developed before inh present as erythematous warm vascular plaques or nodules with arim of pallor and donot undergo postnatal growth. Congenital hemangio- mas are divided into two categories ~ rapidly involuting congenital Remangiomas (RICH) and ‘non-involuting congenital hemangiomas (NICED. ‘As the name implies, RICH lesions begin toinvo- lute almost immediately after birth and in many cases fully involute by 1 year of age. A NICH ‘may partaly involuteo¢ soften, but full resolu tion does not occur (Fig. §.2), Despite RICH involute much faster than common hemangio- mas, some patients may develop significant health problems (eg. eardia failure or bleeding ue to arteriovenous shunting (1-3). After phy cal examination hemangiomas should be classi fied as superficial, deep. or mixed type, according ‘o-their location in the skin and subcutaneous ti sue and focal or segmental acvording to theic extension, Superficial infantile hemangiomas ‘may be mistaken for capillary malformations (port-wine stains). Deep infantile hemangiomas typically present asa luctuan, compressible blu [ah mass and may retemble lymphatic, venous, ot ‘mixed (venous and lymphatic) malformations (1, 5]. Congenital hemangiomas may be confused ‘Wilh venous malformations or mixed malforma- dons. RICH may not be distinguished trom a NICH st bith and only clinical observation 4 Mota ctal (ae), Homangiomar and Vncula Malformations: Am Aa of Diapnoris and Teament, TF or ‘Dol 10.1007178-88-470-5473-2 8, © Singer Nrlag Hala 2002,Lette aa aes Mase Yagi Gon br Visca Maifrmaies _DeprteatVscua Surgery Deparnent of Varela Strsey Cartage Mianse (Mts) Chote anne Hamas fab “ner Doct Cains Ve) tals Dik A. Loose ‘Vascular Milformations Facharailnck Hair arbore Gemaay Ish 978.88-470-5672-5 ISBN 976.88.170.5673.2 (eBook) DOL 1o.L007/978.88-470-56732 Springer Milano Heidelberg New York Boneh London trary Congres Cotrol Number: 2014959882 ‘This woek abet Wo eoprEM. Allies ar reserved by he bs, wher he wocior far of the tata eared, spel the rigs ftslon, epating, wuse of torsos reoion, brnsctngrpucon sn mcr amy sr pa an tension information sirage ad real, cone apo carpe! stare erty sma sim methogy row own cera Jewel Empl om ss Spl erin are bie aces inconecion wri ely al ei spp geile othe purpose of being ated and seu oe aoe system, or {Gelue ae by the puch ocx werk: Dapcasen of is public o ts eo feted ly nde he provisions o he Copyright Law of the Pater acto, ts fren ie, an permis fo use met always toe fom Springer, Peston for {etna be baa hough RiyhL a the Copy Ceaace emer Vans re le te peneston dors ripe Copy La ‘woe of genre! dsegme ins rote ames ena sevice maki ths btatioe es nt imp ever im aac oa specie semen, ist Sch raze te ‘Sm foe ree pote Lv ad eps sd href ee or ene we [Wile he she nd iaforrtion ois bok re belived oe te od scart dt of puicatiog, na thc anos eds sot pearance any Weal responsi foray erro oemson tat may te eie The poise males 9 wari. ges OF Sid wire te mate ented hee rina. cde par Sonesta of ringer SslenesBannes Mein snesRaul Mattassi * Dirk A. Loose Massimo Vaghi Editors Hemangiomas and Vascular Malformations An Atlas of Diagnosis and Treatment Second Edition Foreword by J.Leonel Villavicencio: Q SpringerRaul Mattassi - Dirk A. Loose Massimo Vaghi Fditors BUELL UTES POMEL el eo WEISS IM CELT AYR Foreword by PRM EN eaulels) ra Springer% NTERIAN cBSEMATAN DIREKTORATJENDERAL BERANDA —INFORMASI PUBLIK PELAVANAN KESEHATAN © 10249 Tim Promkes RSST - RSUP dr. Soerad Titonegoro Klaten Penyakit lipoma merupakan suatu penyakit yang ditandai dengan benjolon berisi lapisan lemak yong secara bertahop menumpuk di bawah Kult, yang mana benjolan ini berada di ‘antora Kult dan lapisan otot. Penyakit ini kerap muncul di leher, punggung, bahy, lengan, hingga poho. Pada umuminya, benjolon lemak atau liooma ini bisa dibilang memiliki pertumbuhan yang lambat di antara kulit dan lapisan otot, Apabila ditekan menggunakan jovi secara perlahan, maka lipoma okan terasalunak dan mudah digoyangkon, Lipoma ini temyata juga termasuk tumor jinak yang sering kali terjadi pada orang dewasa. lipoma juga tidak menyebabkan rasa sakit saat ditekan. Lipoma lebin sering dialami oleh ‘orang yong berusia 40-60 tahun atau paruh bayo, dan lebih banyak terjadi pada pria dibandingkan wanita, Sebagian pasien bisa memilki lebin dari satu lipoma di tubuhnya. Lipoma tidak memerlukan perawatan khusus Karena tidak berbahaya dan tidak bersifat ganas. Akan tetapi, operasi pengangkatan lipoma bisa dilakukan jika tumor jinak ini tumbuh besar dan mulai menimbulkan rasa saliENTER ESEAKTAN DIREKTORAT JENDERAL BERANDA — INFORMASIPUBLIKY PROF PELAYANAN KESEHATAN Gla perma Upoma bisa muncul di bagian tuouh mana pur, namun umumnya benjolan muncul di ‘ored punggung , poh, Ieher, engan, perut, atow bohu. Terkodang, lipoma pun dapat ‘Mmuneul di Kepala atou belakang kepala . Benjolan yong muncul memiliki citt-ciri sebagai beriut: ? Dopat tumbuh menjadi lebih besor, dari sebesar Kelereng hingga sebesar bola pingpong, ? Pertumbuhan benjolan sangat lambat. ? Terasa lemibek dengan konsistensi sepert lemak daging sop ? Mudah untuk digoyangkan. Benjolan dapat menimbulkan rasa sok ka ukurannya sematin besar don menean sarat disekitarnyo. Kapan Horus ke Dokter Benjolan di permukaon tubuh belum tentu lipoma, bisa soja Kista otou bahkan tumor (ganas {kanker ) yong dapat berakbat fatal bila tidak segera ctongani. Anda aisarankan ‘untuk memeriksokan dir ke dotter bla menemukan benjolan di area tubuh mana pun dan ‘opa pun karokteristknya, baik yang beru‘uran kecl atau besar, lunak atau keras, dapat ligerakkan atou tidak, sera terasa sot atau tidak. Danuahah nanaPPh "ed el zs uC aS aces prey DAFTAR ISI KATA PENGANTAR EDISI KEDUA KATA PENGANTAR. DAETAR PENYUSUN.Yaad ATLAS Penyakit Kulit Kelamin U... HEMANGIOMA BATASAN. Hemangioma merupakan tumor jinak yang terjadi akibat gangguan pada pperkembangan dan pembentukan pembuluh darah pada semua organ misalkan lit, GEJALA KUNIS 1. Hemangioma kapiler 2. Hemangioma Kavernosum 3. Hemangioma Campuran DIAGNOSIS BANDING 1. tipoma 2. Limfangioma 3. Neurofibroma PENATALAKSANAAN 1. Pembedahan 2. Radiasi 3. Bedah laser Regio ekstremitas superior dan fasialis, tampak tumor berwarna merah keunguan, batas jelas, lanak pada perabaan. Pada pemeriksaan histopatolog! tampak pleksus vesikuler ‘pada jaringan subkutaneus.