A venous anomaly of the neck

A venous anomaly of the neck

ELSEVIER International Journal of Pediatric Otorhinolaryngology 42 (1997) 81-89 Case report A venous anomaly of the neck 6. TaSkin Yiicel *, Erkhan...

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ELSEVIER

International Journal of Pediatric Otorhinolaryngology 42 (1997) 81-89

Case report

A venous anomaly of the neck 6. TaSkin Yiicel *, Erkhan GenC, Sefa Kaya Department Hacettepe

of Otorhinolaryngology-Head University Faculty of Medicine,

and Neck Surgery, Ankara, Turkey

Received 8 March 1997; received in revised form 4 August 1997; accepted 6 August 1997

Abstract Vascular anomalies localize mostly in the head and neck region. Besides the complications they present,when they are left untouched, they are cosmeticallyugly. A vascular lesionin

any part of the body of a child is always bothersometo the parentsand may be a problem for the family. The confusionabout classificationof theselesionsand the delay in seeingthe physician may adversely affect the child also. There has been a debate concerning the classificationof vascular malformations, their clinical presentation, and their treatment modalities. In this paper we reviewed the classificationas accepted in the literature and presenteda caseof hugevenousanomaly in the neck region with its angiographicand MRI views and discussedthe possiblecomplicationsand modesof treatment. 0 1997 Elsevier ScienceIreland Ltd. Keywords:

Vascular malformation; Venous anomaly; Angiography; Magnetic resonance

imaging

* Corresponding author. Present address: Hosdere Cad. No 49/13, 06540 Y. Ayranq Ankara, Turkey. 0165-5876/97/$17.00 0 1997 Elsevier Science Ireland Ltd. All rights reserved. PI1 SO165-5876(97)00112-2

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1. Introduction Vascular anomalies are peculiar to the human since the early ages. Anyone who had such lesions on their body were accused of being witches and others accepted them as the mark of evil. As Watson and McCarthy [13] suggested during the 194Os, vascular malformations prefer to be localized at the head and neck region. Although this place makes up only 14% of the total body surface area, 50% of the vascular anomalies are seen in this region. The aesthetical, anatomic, and as Mallen et al. [6] reported, the hemodynamical burden of this situation to the body is another important point that should be faced when one deals with them. Angiomatous diseases affect up to 3% of the children [9], although pure venous anomalies are rarely seen when compared to other vascular anomalies of the body. The importance of venous anomalies are better understood when one considers that half of these lesions have been located on the head and neck region, sometimes compromising the air passages and the deglutition.

Fig. 1. Right lateral view of the lesion.

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Fig. 2. Frontal view of the mass while the neck is extended.

Fig. 3. The Tl weighted MRI scan of the lesion showing the mass that fills the right side of the neck, from the mandible and the parotid region, down the supraclavicular fossa.

Vascular anomalies may cause thrombocytopenia, pulmonary hypertension, congestive heart failure, respiratory failure, the bleeding they caused is often profuse, and also may cause considerable damage to the vital organs either by compression or by necrosis [14]. In 1982, Mulliken and Glowacki [8] made a basic classification of the vascular anomalies. According to this classification, a vascular malformation was divided into two large categories which included a wide spectrum of anomalies on each side. On one hand, there are hemangiomas which exhibit a history of presence that is

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Fig. 4. The T2 weighted MRI scan of the lesion revealing the posterior notf : the hypointensity of the lesion suggesting the vascular component.

section of the mass. The right internal Fig. 5. The angiographic and the oblique views of the venous phase of the procedure fron n the external jugular vein.

carotid revealed

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of the vascular

artery catheterization the venous anomaly

anomaly,

performed originated

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Fig. 6. The two heads of the mass are clearly seen in this venous phase angiographic section. They are probably interconnected with the tiny vascular channels. Note the anomaly sits just on the clavicula and very close to the lung.

barely noticeable at birth but shows rapid growth and slow involution in time. They were characterized by hypercellularity during the proliferative phase; fibrosis and diminished cellularity during the involution phase. On the other hand, there are vascular malformations which are present at birth and grow commensurately with the child and were characterized by a normal rate of endothelial cell turnover. This classification was also supported and clinically adapted by Finn et al. [3]. The vascular malformation group had four basic subgroups according to Kaban et al. [5]. They are: capillary, venous, lymphatic and arterial anomalies. Venous anomalies are one type of vascular malformations, but they may be present in combination with the other listed anomalies [5]. The case that is presented here is a large venous anomaly located on the neck of a child.

2. Case report

A 13-year-old boy was admitted to our clinic with a complaint of a mass with discoloration on his neck. This mass was observed on his neck when the child was born and it grew slowly with the child, causing him no harm. The patient suffered from no respiratory distress or difficulty in swallowing and there was also no drainage, bleeding or pain on palpation (Figs. 1 and 2). Physical examination revealed a mass which was soft in consistency with a few tiny nodules in it. The mass had borders from the trapezius muscle at the lateral,

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and rising to the submandibular space on the right side of the neck, and it passed through the midline to the other side of the neck. It extended to the anterior border of the opposite sternocleidomastoid muscle medially, up to the cheek superiorly and filled the supraclavicular fossa inferiorly. It had a bluish-purple color and was filled with a Valsalva maneuver. He was able to open his mouth properly and there was no pain on the mandible by palpation. The oral examination revealed that the tonsillar pillars on both sides, the posterior oropharyngeal wall, and soft palate had bluish-purple looking vascu-

Fig. 7. The lateral views of the same lesion after catheterization showing the phleboliths in the vascular structure.

of the right internal carotid artery

Fig. 8. The well defined border of the venous anomaly H + E, 115X.

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Fig. 9. The vascular lakes with endothelial borders and phleboliths in it, H + E, 115X

lar lesions reaching up to 4 mm in the maximal dimension. This mass was not occluding the laryngeal inlet and the vocal cords were visible and had full abduction capacity. The hematologic profile of the patient was stable, including the PT, PTT and thrombin time, the bleeding and coagulation times. The thrombocytes were sufficient in number and function also. The screening by conventional radiographs for a skeletal anomaly revealed no additional lesion. MRI and angiography of the neck was performed (Figs. 3 and 4) revealing a venous anomaly occupying the right side of the neck, filling the supraclavicular fossa and reaching up to the mandibula. The mass was thought to be consisting of two main parts. The two parts were continuous with the small vascular channels between them as seen on the angiographic section of the lesion in the antero-posterior views (Fig. 5). As it was filling in the venous phase of the angiographic evaluations of the lesion, the anomaly was mostly originating from the external jugular vein and there was phleboliths in it (Figs. 6 and 7). The child underwent an operation for exploration of the lesion. The mass was found to be mainly a venous anomaly originating from the external jugular vein. The anomaly occupied the whole right side of the neck and it was excised completely. The pathology result supported our diagnosis and the microscopic examination of the lesion revealed that it was composed of vascular lakes with well defined endothelial borders and some had phelebolits in them (Figs. 8 and 9).

3. Discussion

A considerable number of neck masses during infancy and childhood are due to vascular anomalies. As it is in this case, the families think that such lesions are not important and generally they do not consult a doctor until there is a complaint.

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The vascular anomalies of the infancy and childhood period are classified into two large groups as hemangiomas and vascular anomalies, the hemangiomas being the most common tumor of infancy [g]. Being a subgroup of the vascular malformations, the venous anomalies are rarely seen in this period of life. According to vascular embryology, these lesions are thought to be originating from the persistent angioblastic tissue and normally this kind of tissue reorganizes or regresses as the child grows [ 13,11,4]. So in fact, vascular anomalies are problems of the morphogenesis [7]. Venous anomalies, since they grow very slowly and proportionately with the child, and because of the hope of self involution of the mass by the family, left the lesion hidden from medical attention till the teens. Only the parents of patients with respiratory distress or severe pain or difficulty in swallowing or having cardiovascular symptoms seek medical help. Sometimes a suspicious mother recognizing the phleboliths in the mass, or a family member being worried about it complains to the physician about the lesion. As is pointed out by some authors, vascular anomalies have a 35% chance of having accompanying skeletal anomalies with them [5,1]. In our case we were not able to show any accompanying skeletal anomaly. The vascular malformations and as a subgroup of the venous anomalies, appear different on angiographic studies. As Burrows et al. [2] pointed out, in contrast to the hemangiomas, the venous anomalies consists of collections of abnormal vessels without a ‘parenchymal’ mass in their angiographic appereance. In our case, also, there were opacifications in the lesion that are suggestive of the phleboliths as seen on the angiographs of the patient but there was no parenchymatous appearance. Sometimes a consumption coagulopathy that may be cause of a profuse bleeding during the operation is reported and warned of by the literature [5]. This is basically due to the localized clotting and consumption of the coagulation factors within the malformation. Generally preoperative use of heparin and sometimes epsilon aminocaproic acid is advised in such cases. Since the hematologic profile of our patient was very stable in this aspect, we did not use them, and the bleeding during the operation was insignificant and we observed no problem postoperatively either. The treatment of childhood angiomatous diseases consists of some conservative and cosmetically non detrimental methods and the surgical excision of the lesion. Among the conservative methods, such as the use of interferon alfa-2a subcutaneously [14], the selective embolization of the anomaly [lo], and injecting a sclerozing agent into the lesion [12] are described. All of the previous methods partially involute but do not totally fade the lesion away. Some of them tend to decrease bleeding during surgery. Although, we believe that in resectable masses, surgery should always be preferred to conservative methods. Total resection of the lesion means a cure for the patient and the family as long as this resection does not develop any handicaps such as cranial nerve palsies, major vessel trauma, and the functional loss of the organs.

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References [I] J.B. Boyd, J.B. Mulliken, L.B. Kaban, J. Upton, J.E. Murray, Skeletal changes associated with vascular malformations, Plast. Reconstr. Surg. 74 (1984) 7899795. [2] P.E. Burrows, J.B. Mulliken, K.E. Fellows, R.D. Strand, Childhood hemangiomas and vascular malformations: angiographic differentiation, Am. J. Radiol. 141 (1983) 4833488. [3] M.C. Finn, J. Glowacki, J.B. Mulliken, Congenital vascular lesions: clinical application of new classification, J. Pediatr. Surg. 18 (1983) 894. [4] G. de Takats, Vascular anomalies of the extremities, Surg. Gynecol. Obstet. 55 (1932) 227. [5] L.B. Kaban, J.B. Mulliken, Vascular anomalies of the maxillofacial region, J. Oral. Maxillofac. Surg. 44 (1986) 203-213. [6] E. Malan, A. Azzolini, Congenital arteriovenous malformations of the face and scalp, J. Cardiovast. Surg. 9 (1968) 109. [7] E. Malan (Ed.), Vascular Malformations (Angiodysplasia), Carla Erba Foundation, Milan, 1974. [8] J.B. Mulliken, J. Glowacki, Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics, Plast. Reconstr. Surg. 69 (1982) 412. [9] J.B. Mulliken, A.E. Young, Vascular Birthmarks: Hemangiomas and Malformations, Saunders, PA, 1988, pp. 1-483. [lo] J. Natali, J.J. Merland, Superselective arteriography and therapeutic embolization for vascular malformations (angiodysplasia), J. Cardiovasc. Surg. 17 (1976) 465. [II] V.A. Ribert, Ueber bau wachsthum und genese der angiome, nebst bemerkungen iiber cystenbildung, Arch Pathol. Anat. 151 (1898) 381. [l2] M.C. Riche, E. Hadjean, P. Tran-Bay-Huy, et al., The treatment of capillary-venous malformations using a new fibrosing agent, Plast. Reconstr. Surg. 71 (1983) 607. [13] W.L. Watson, W.D. McCarthy, Blood and lymph vessel tumors: a report of 1056 cases, Surg. Gynecol. Obstet. 71 (1940) 569. [14] C.W. White, S.J. Wolf, D.N. Korones, H.M. Sondheimer, M.F. Tosi, A. Yu, Treatment of childhood angiomatous diseases with recombinant interferon alpha-2a, J. Pediatr. 118 (1991) 59.