Lymphangioma. An otolaryngologic perspective

International Journal of Pediatric OtorhinoIaryngology, 14 (19873 133-140 Elsevier

133

POR 00472

Department of Otolaryngology and Communicative Sciences, Baylor College of Medir:ine, Houston, TX 77030 (U.S.A.) (Received 8 May 1987) (Revised version received 12 August 1987) (Accepted 29 August 1987)

Key work

Lymphangioma; Congenital hamartoma

Lymphangiomas are relatively rare congenital hamartomas of the lymphatic system usually diagnosed in infancy and early ch&lhoud, Although the precise pathogenesis has net been clearly defined, abnormal development leads to formation of fluid-filled sinuses lined by vascular endothelium. In the head and neck, lymphangioma may encroach on vital structures and infiltrate surrounding tissues making complete surgical removal difficult. A numb of children (n = 137) with lymphangioma have been treated at Texas Children’s ospital from 1972-1985. In 45 (33%), the tumors were diagnosed a: birth and in an a.dditional511 (37%), by two years of age. Commonly affected areas included the head and neck (45%), trunk (26%&),and axilla (17%). Mediastinal involvement occurred in 4%. Surgical removal was the preferred treatment method and with complete removal the recurrence rate was 6%; known incomplete removal increased this rate to 35%. Although lymphangiomas are slow growing, early removal was preferable: recurrences increased as the time interval between tumor identification and surgical intervention lengthened. Size fluctuation with a history of infection, another unfavorable prognostic factor, also increased recurrence rate.

Lymphangiomas are relatively rare congenital hamartomas of the lymphatic system usually diagnosed in infancy. The first description of this tumor in the

Presented at the Sxond Annual Meeting of the American Society of Pediatric Otolaryngology, 1987. Correspondence: R.J.H. Smith, Department of Otolaryngology and Communicative Colkqge of Uedicine, One Baylor Plaza, Houston, TX 77030. U.S.A.

Sciences, Baylor

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literature is credited to Redenbacher who, in 1828, referred to a lesion as a ‘ranula congenita’ [ll]. Fifteen years later, Wernher coined the descriptive term ‘cystic hygroma’, hygroma being derived from the Greek ‘hydro’ = moist and ‘oma’ = tumor [Ie]. ~though Wernher thought the fluid-filled sacs represented new growth of benign neoplastic tissue, in 1855 Rokitansky postulated that a cystic hygroma was simply a collection of serous fl@d held in place by surrounding tissue 1151.Eight years later, Virchow published a paper in Wemher’s support and in 1872 Koester hypothesixed that hygroma arose from lymphatic tissue [14]. This hypothesis was studied extensively by Florence Sabin in her meticulous investigation of the embryologic development of the lymphatic system [12]. She concluded that the human lymphatic system originates from 5 primordial sacs: two paired jugular sacs; two paired sacral sacs; and an unpaired retroperitoneal sac. From these primitive sacs, endothelial sprouts propagate outward to form the peripheral lymphatic system. This centrifugal theory of lymphatic development gained further support from the classic work of Goetsch (61. From histologic preparations of cystic hygroma, he identified endothelial buds sprouting from the growing edge of the tumor and infiltrating surrounding tissue. He felt this represented true neoplastic growth of the lymphatic system. Several investigators have challenged this work, proposing instead a ccntripetal theory in which peripheral lymphatics develop from mesenchymal slits in sequestered portions of the primordial sacs [lo]. These sequestrations enlarge and ultimately join the venous system. Failure of this anastomosis results in formation of a cystic hygroma. This hypothesis, however, accounts for neither the- histologic findings nor the potential for rapid and extensive growth and Sabin’s centrifugal theory is the most widely accepted today. Classification of lymphangioma is confusing. Landing and Farber divide lymphangioma into broad categories on the basis of size of cystic spaces [9]. Capillary lymphangioma, or lymphangioma circumscriptum, are vesicular masses composed of capillary-sized lymphatic channels which often protrude into the epidermis. Cavernous lymphangioma contain larger dilated sinuses which invade surrounding tissue. Cystic hygromas are the largest of the cystic structures and frequently’completely surround neural and vascular structures. Because of the great overlap in these categories, it is likely that the nature of the surrounding tissue determines to some extent the physical structure of a lymphangioma and that the different types of cystic tumors are simply variants of the same pathologic process [l]. It has been noted that the smaller cystic forms tend to occur in locations where the restrictive nature of surrounding tissues limits room for expansion, such as the floor of mouth and chest wall areas. The larger variant, the cystic hygroma is found more often in areas of loose fatty tissue where expansion can easily occur. Bill and Sumner have further postulated that the periphery of the tumor may have lymphangiomatous characteristics and the main body of the mass, those of a cystic hygroma [l]. The pu@ose of this paper is to review the experience obtained at Texas ChMmn’s Hospital in the treatment of lymphangioma. Special attention is focused on those lymphangiomas occurring in the he,ad and neck. We will use the terms

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‘cystic hygroma’ and ‘lymphangioma’ this paI9er.

interchangeably

throughout

the remainder

of

ateiials Clinical material for drawn from tlae records Hospital. In all cases, obtained in 31 patients,

this 13-year retrospective review beginning in 1972 was of 137 patients with lymphangioma at Texas Children’s diagnoses were histologically confirmed. Follow-up was an average of 3.8 years following their initial surgcr;l,

No sexual bias was fiound in this study. In 29 children (33%), lymphangioma were diagnosed at birth and in an additional 33 (37%), by two years of age (Fig. I). The area were most commonly affected foIlowed in order of frequency head and a, and extremities (Fig. 2). the trunk Lymphangioma confined to the head most commonly involved the scalp, cheek, and submandibular areas. One patient had tongue involvement and another had extensive infiltration of the left orbit which necessitated multiple surgical procedures. In the neck, lesions were evenly divided between the anterior and posterior triangles with one child having involvement of the substance of tile thyroid gland (Figs. 3,4). Most patients (75%) presented with a slowly growing asymptomatic mass although 17% sought medical attention after a rapid increase in the size of the lymphangioma, often subsequent to infection or local trauma. A history of size fluctuation in association with an othe:tise mild upper respiratory tract infection was not unusual. In 6 patients, the lymphangioma was first noticed following a seemingly trivial event. Such was the case in one child whose lesion reportedly

Fig. 1. Age of patient at time of diagnosis.

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Hold

AXI&

ExtromltkrModlastlnuln Other

Note - Some plr. had more than one rite of involvement

Fig. 2. Sites most commonly affectedby lympbangioma.

at the site of a routine vaccination. Another child noticed an enlarging mass on her buttock after falling from her bicycle. Ninety-nine patients were initially evaluated at Texas Children’s Iiospital and of these, 95 underwent surgery. In 78 children, the excision was thought to be ‘complete’; in 17, it was ‘incomplete’. One child with extensive involvement had several staged procedures and 3 children had open drainage of the lymphangioma. Neither injection of sclerosants nor radiotherapy was used in any case in this series. Although several patients were initially observed for a period of time piior to surgery, complete spontaneous regression was not noted. With ‘complete’ excision, the recurrence rate was 6%; this increased to 35% when the excision was ‘incomplete’. Lymphangiomas involving the submandibular and floor of mouth areas were most likely to recur, followed by lymphangioma of the anterior triangle. Surgical sites with the lowest recurrence were the axilla and trunk (Fig. 5). Suppurative infections of the lymphangioma prior to surgery increased the difficulty of the operabion resulting in a 31% recurrence rate; however, this rate fell to 15% when there was no clear history of prior infection. The likelihood of recurrence also increased as the time interval from tumor identification to surgical intervention increased. There was no mortality during initial therapy although one child with extensive lymphangiomatosis eventually died secondary to complications following numerous ‘developed’

SITE - HEAD GN=15)

SITE - NECK ( _.

Oral cavity, tongue,

Anterior Triangle

- 13

lloor 01 mouth Scalp

- 5 -4

Posterior trlanqle

- 13

Face

-3

Wldesgrsad

-

3

Wldespread

-1

Thyroid

-

1

Orblt

-1

Parasplnous

-

1

Perlaurlcular

-1

Fig. 3. Arcas of involvementin the head.

Fig. 4. Areas of involvement in the neck.

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PJetk

Herd

Trunk

‘Ant20

Lxtrsmttler

Recurrences

Fig. 5. Involvement and recurrences by site.

surgical procedures. There were occasional transient facial nerve palsies all of which resolved within a few weeks of surgery. ost-operative infections responde to antibiotics. We obtained follow-up information on 31 patients. The average ti surgery was 3.8 years and ranged from 4 months to 17 years. Twenty-six patients had no recurrence and in the 5 with residual lymphangioma, 3 felt that the tumor was insignificant and did not wish further sur$cal intervention.

iscussio Lymphangiomas show neither racial nor sexual predilection and are most often diagnosed in the pediatric population. In this series, 33% were diagnosed at birth and 70% by two years of age. Other authors have reported these figures to be higher, 50-65% and 90%, respectively [8]. Discovery in adolescence or adulthood is reported but is distinctly uncommon. Associated anomalies inclttde Turner’s syndrome, congenital heart disease, cleft lip, spina bifida, and mental retardation. Patients most commonly present with a slowly growing, asymptomatic, fluctuant, soft-tissue mass. Frequently, rapid enlargement occurs following an upper respiraess resolves. tory infection; however, the enlargement usually diminishes as the Occasionally, large lymphangiomas compromise respiration and deglutition in the neonate necessitating emergent surgical decompression of the mass. Lymphangiomas were located in the head and neck in 45% of the children we studied, again a figure that is lower than that reported by others (65-75%:) 1131. Lymphangioma involving the head occurred most commonly in the submandibular or floor of mouth regions. Affected infants presented with macroglossia or dysphagia and complete tumo;- removal was exceedingly difficult. Another site of oral involvement was the buccal mucosa. Capillary ly,mphangioma in this location responded well to laser marsupialization (Pig. 63. Instances of cheek, orbit, and eyelid lymphangioma also occurred.

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Fig. 6. Left buccal lymphangioma prior to laser marsupialization.

In the neck, although the posterior triangle has been. reported as the most frequently involved site, we found involvement of the anterior triangle to be equally as common. In the anterior triangle, respiration and deglutition may be comprised making prompt surgical intervention imperative [2]. Involvement of the epiglottis or extension into the mediastinum compounds operative difficulty and may make complete removal impossible (Fig. 7). Since cystic hygromas comprise less than 5% of congenital neck masses, they must be differentiated from other soft tissue tumors such as brachial cleft cysts, thyroglossal duct cysts, lipomas, hemangiomas, and dermoid tumors. Unfortunately, neither a CT scan nor an ultrasound is pathognomonic; however, if these types of imaging show a multiloculated, cystic structure which by physical examination is fluctuant and transilluminates, the diagnosis of a cystic hygroma is more certain. As alluded to, complete tumor extirpation is the most desirable form of treatment; other approaches are chiefly of historical interest. Because infection occasionally is accompanied by partial or complete regression, infectious agents at one time were intentionally introduced into lymphangioma. Often this was by means of a seton which was inserted through portions of lymphangioma and left in place until induration and inflammation resulted. Sclerosing agents such as boiling water, sodi:m morrhuate, hypotonic saline, and bleomycin also have been injected into c2ats to cause.fibrosis. Possible damage to adjacent vital structures and the danger of uncontrolled and unpredictable scarring make these practices unwise.

Fig. 7. Largecervkal lymphangiomacausbg upper aertipestive compromke.

Direct aspiration to reduce size may serve as a temporizing measure until definitive surgery can be planned. Some authors even argue that adequate time must be allotted for spontaneous resolution [3]. Grabb et al. have reported complete resolution in 7 of I7 untreated patients and partial resolution in an additional 5 [7]. However, most authors agree at while a cystic hygroma may undergo apparent regression in size over a finite period of time, eventually all recur and enlarge. The most widely accepted treatment remains surgical removal [4,5]. Lymphangiomas judged to be completely removed at the time of surgery have significantly lower recurrence rates than those which are incompletely removed. Wowever, because this is not a malignant tumor, neural or vascular structures should not be sacrificed. If the hygroma cannot be removed completely without undue damage to the surrounding tissues, remaining cysts should be unroofed to promote atrophy and scarring of residual tissue. Some surgeons have recommended that the wound bed be packed with iodoform gauze to induce fibrosis in the remaining cysts. In very young patients with large infiltrating tumors, multiple staged procedures may be more feasible than a single radical extirpation. Recurrences are generally apparent within l-2 years. The incidence of infection, hemorrhage, and other complications increases with time, often making subsequent complete excision difficult or impossible. It also seems reasonable to suspect that more extensive tissue invasion may take place with prolonged delay. Therefore, we recommend surgical excision of most lymphangiomas as soon as is safely possible. In cases of laryng,eal or oral lymphangioma, staged laapr marsupialization of the cysts is the procedure of choice.

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