International Journal of Pediatric Otorhinolaryngology 74 (2010) 398–403

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International Journal of Pediatric Otorhinolaryngology

journal homepage: www.elsevier.com/locate/ijporl

Lymphatic malformations: A proposed management algorithm

J.C. Oosthuizen *, P. Burns, J.D. Russell
Our Lady’s Hospital for Sick Children, Crumlin, Dublin, D12, Republic of Ireland

A R T I C L E I N F O A B S T R A C T

Article history: Objective: The aim of this study was to develop a management algorithm for cervicofacial lymphatic
Received 20 October 2009 malformations, based on the authors’ experience in managing these lesions as well as current literature
Received in revised form 13 January 2010 on the subject.
Accepted 15 January 2010
Study design and methods: A retrospective medical record review of all the patients treated for lymphatic
Available online 18 February 2010
malformations at our institution during a 10-year period (1998–2008) was performed. Data collected:
age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms,
Keywords:
treatment modality used, complications and results achieved.
Lymphangioma
Cystic hygroma
Results: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal
Lymphatic malformation distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first
Paediatric year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for
Management algorithm referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and
dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical
excision and laser therapy. In 5 cases (36%) a combination of these were used.
Conclusion: Historically surgical excision has been the management option of choice for lymphatic
malformations. However due to the morbidity and high complication rate associated this is increasingly
being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant
promise. Based on experience in managing these lesions as well as current literature the authors of this
paper have developed an algorithm for the management of cervicofacial lymphatic malformations.
ß 2010 Elsevier Ireland Ltd. All rights reserved.

1. Introduction
Lymphatic malformations (LMs) are rare benign tumours that
result from localized congenital malformations of the lymphatic
system. These lesions are diagnosed during infancy in the
overwhelming majority of cases and most often present as an
asymptomatic mass in the cervicofacial region [1]. The manage-
ment of LMs remains a challenge. Surgical excision has tradition-
ally been the first line of treatment however due to the close
proximity of vital structures this often leads to incomplete excision
and recurrence, or damage to vital structures e.g. cranial nerves [2].
More recently the use of OK-432 has gained popularity especially
in the management of macrocystic lesions [3]. The authors
reviewed the records of all cases treated for LMs at our institution
during a 10-year period. Whilst the majority of cases were
managed surgically, promising results were obtained with
observation alone as well as with OK-432 injection. Based on
experience gained in the management of these lesions as well as
* Corresponding author at: Tawlaught, Fenit, Co. Kerry, Ireland.
Tel.: +353 667136863.
E-mail address: C.oosth@gmail.com (J.C. Oosthuizen).
current literature on the subject the authors of this paper have
formulated a proposed management algorithm for LMs.
2. Patients and methods
After obtaining local ethics committee approval, all the patients
diagnosed and treated for LMs at our institution between 1998 and
2008 were identified. A retrospective review of these patients
medical records were performed focussing on age at diagnosis,
location of lesion, type of LM, radiologic investigation performed,
presenting symptoms, treatment modality used, complications
encountered and results achieved.
3. Results
A total of 14 patients were identified (Table 3). The diagnosis
was based on clinical examination and radiologic investigations.
Eight (57%) patients were male and the remaining six (43%) female.
Two cases (14%) were identified during the prenatal period, five
(36%) at birth and three (21%) during the first year of life. The
remainder of cases (29%) showed some variation regarding age of
presentation with a range of 3–7 years of age. There was an equal
distribution between the right and left sides. The location of these
lesions was predominantly surpahyoid (86%), with 4 cases

0165-5876/$ – see front matter ß 2010 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ijporl.2010.01.013
 J.C. Oosthuizen et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 398–403
Table 1
Treatment modalities used.
Treatment modality Cases
Surgery 5 numerous theories have been proposed regarding the embryologi-
Ok-432 injection 3 cal aetiology of these lymphatic malformations. These include the
Observation 3 centrifugal and centripetal theories, the former proposed by Sabin
Ok-432 followed by surgery 2 and the latter by McClure and Huntington, as well as the combined
suffering from extensive disease encompassing both the supra and
infrahyoid areas. In 2 of these cases there was significant
mediastinal extension of the tumour. Only 1 case of exclusively
infrahyoid LM was treated at our institution during the 10-year
period. The most commonly affected area was the submandibular
region affecting 5 cases (36%). Six cases had combined macro- and
microcystic disease and 2 cases suffered from predominantly
microcystic disease.
The predominant reason for referral to our service was an
asymptomatic mass (50%), the remaining patients presented with
either airway compromise (36%) or dysphagia (14%). In all cases
the diagnosis of LM was confirmed with radiologic investigations.
Magnetic resonance imaging (MRI) was the most commonly used
modality and was employed in 71% of cases. Other investigations
included computed tomography (CT) and ultrasound (US) scan-
ning.
Treatment modalities employed consisted of observation, OK-
432 injection, surgery or a combination of these (Table 1). All cases
of microcystic disease of the oral cavity were managed with
surface potassium–titanyl-phosphate (KTP) laser therapy in order
to control symptoms. Of the seven patients treated with surgical
excision, four had been operated on at other hospitals prior to
referral to our institution. Major complications of treatment
included a single case of facial nerve palsy and another of
hypoglossal nerve palsy. Both of these cases had undergone
previous surgery and the above complications occurred during
revision surgery for recurrent disease. Minor complications
included seroma and haematoma formation in 2 separate cases.
Two cases (14%) were lost to long term follow up and were
therefore not included in the final outcome measurement.
Outcome was defined as excellent in cases with complete
resolution of the lesion and no residual cosmetic or functional
impairment. In cases with minimal residual or recurrent disease, of
little concern, the outcome was classified as good. A patient with
recurrent or persistent disease staying stagnant or showing some
degree of improvement was rated as fair. Finally patients with
severe progressive disease showing minimal or no response to
treatment were classified as having poor outcome. Favourable
outcomes were obtained in 10 cases (83%). Of these five were
classified in the ‘‘excellent’’ and the remainder in the ‘‘good’’
outcome groups. Persistent or recurrent disease was encountered
in 4 cases. The most common site of recurrent disease was the
tongue (50%).
4. Discussion
LMs once referred to as either cystic hygroma or lymphangioma
depending on cyst size is now more commonly divided into
macrocystic, microcystic or combined disease [4]. The reported
incidence of these tumours in the literature is quite variable,
ranging between 4 per 10 000 births in one study [5] and 1 per
16 000 births in another [6]. The overwhelming majority of LMs
occur in the cervical region with an increased incidence on the left
side. There is no difference in distribution between the sexes with
both equally affected. The age at diagnosis is reportedly 75% at
birth with 90% of the remaining cases diagnosed by the age of two
399
[7]. Two cases in our series (14%) were diagnosed during the
antenatal period. Five cases (36%) were diagnosed at birth and 86%
of cases were diagnosed within the first 3 years of life. To date
theory proposed by van der Jagt and Kutsuna [3,7]. Regardless of
the proposed theory the final result is failure of either the
peripheral lymphatics to flow into the jugular sacs or failure of the
jugular sacs to reunite with the venous system [7]. It is also widely
accepted that LMs can be acquired secondary to surgery, trauma,
infections, neoplasms and chronic inflammation [3,7]. The
histopathological classification of LMs as proposed by Kennedy
in 1989 includes four distinct groups [7]. It is widely accepted
however that all these lesions form part of the same disease
process [8].
The majority of cases (43%) in our series presented with an
asymptomatic mass in the cervicofacial region. The remainder
either presented due to airway concerns (36%) or dysphagia (21%).
These findings are consistent with previously reported presenting
symptoms and signs [9]. In the group that presented with airway
compromise, three patients required tracheostomies two of which
were emergent. Infective episodes as well as haemorrhage into
these cysts are quite common, often leading to rapid enlargement
of the cyst with potential airway compromise [10]. This can
occasionally, as illustrated in our case series, require an emergency
tracheostomy. Current guidelines on the management of these
episodes advocate the use of parenteral Gram positive coverage for
up to 3 weeks followed by a prolonged course of oral antibiotics
[10]. In our case series 64% of patients developed at least one such
episode whilst others suffered from recurrent infections, all of
which were successfully managed medically. A recent report by
Sires et al. [11] showed promising results with use of systemic
corticosteroids in the treatment of these episodes in ophthalmic
LMs.
In an effort to predict the prognosis of LMs several staging
systems have been proposed. Orvidas and Kasperbauer [12] used
the variables of functional impairment, cosmesis, number of
locations and age at diagnosis in order to formulate a staging
system. They demonstrated an increase in persistence as well as
complication rate with increasing stage. However the most
commonly used staging system was developed by de Serres
et al. and published in 1995 [13]. It is based on the anatomical
location of the LM and consists of five stages (Table 2). In their
paper they described a clear correlation between the stage of the
disease and the prognosis as well as associated complication rate.
Group 1 patients had a complication rate of 17% compared to 67%
in group 3 and 100% in group 5. Clearly demonstrating a
progressive increase in complication rate associated with higher
staging. In a retrospective study of 22 cases Hamoir et al. [14]
applied the proposed staging system and reported findings
consistent with those of de Serres et al. In our case series 7%
were classified in group 1, 64% in group 2 and 29% in group 3. There
were no patients in group 4 or 5. Our results reflect the findings of
de Serres as well as Hamoir et al. with a more favourable outcome
Table 2
Staging of LMs.
Stage Description
1 Unilateral infrahyoid
2 Unilateral suprahyoid
3 Unilateral infrahyoid and suprahyoid
4 Bilateral suprahyoid
5 Bilateral suprahyoid and infrahyoid
400 J.C. Oosthuizen et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 398–403

Table 3
Summary of Results.

Case A.A.D. Site Side Sex Presenting Type Intervention Size (cm) Complications Outcome Recurrent/
complaint persistent

1 8 months SM, T/M R M Swallowing Mic KTP 45 Nil G P

2 Birth F, AT, PT, T/M L M Airway Mic OK-432, S, KTP – Facial n palsy P P&R
3 Birth F, AT, T/M R F Airway Comb S, KTP 88 Hypoglossal n palsy; F –
haematoma
4 6 years F L M Swallowing Comb O – Nil E –
5 6 weeks SM R F Airway Mac S 10  8 Nil E –
6 Prenatal SM L M Swallowing Comb S – Nil E –
7 3 years AT, M L M Cosmetic Mac S 96 Nil G –
8 7 years F R F Cosmetic Mac OK-432 – Nil G P
9 Birth SM L F Airway Comb O – Nil G P
10 3 years SM L M Cosmetic Mac O 34 Nil E –
11 8 months P R M Cosmetic Comb O, OK-432 4  0.7 Nil G P
12 Prenatal AT, PT, M R F Airway Mac S 10  10 Seroma E –
13 Birth PT L F Cosmetic Mac OK-432, S 5  3.5 Nil LF –
14 Birth F R M Cosmetic Comb OK-432 10  7.8 Nil LF –

A.A.D. = age at diagnosis, SM = submandibular, T/M = tongue/mucosa, F = face, AT = anterior triangle, P = parotid, M = mediastinal extension, PT = posterior triangle,
Comb = combined disease, Mac = macrocystic, Mic = microcystic, O = observation, KTP = KTP laser, S = surgery, G = good, P = poor, F = fair, LF = lost to follow up.

as well as lower complication rates observed in lower stage
disease.
The diagnosis of LMs usually depends on physical examination
and a range of different imaging modalities (Fig. 5). Clinically these
lesions have been described as soft; doughy masses which
transiluminate [8]. In our case series nearly all the patients had
a MRI scan performed in order to delineate the extent of the
disease. Other imaging options include US and CT scanning. Each of
these modalities has distinct advantages as well as disadvantages.
US is very useful in evaluating superficial lesions [15] and in the
guidance of OK-432 injection therapy (see below). It is limited
however in the evaluation of deeper structures in the neck and
mediastinum [15]. Sonographically these lesions are multilocular
cystic masses with septa of variable thickness [16]. MRI is regarded
as the modality of choice in order to evaluate the involvement of
neighbouring structures and to effectively plan any surgical
intervention [3,8]. Some of the benefits of MRI include superior
multiplanar capabilities, no ionizing radiation and lack of bony
artefact [15]. The typical appearance of LMs on MRI scanning
includes low signal intensity on T1-weighted images, high signal
intensity and multiple cysts with well demarcated margins on T2-
weighted images [15,17]. Massive osteolysis, skeletal distortion
and hypertrophy have been reported secondary to cervicofacial
LMs [9]. If this is suspected CT scanning is the investigation of
choice due to its excellent delineation of bony structures.
The use of routine ultrasound evaluation of the fetus during the
antenatal period has become commonplace throughout the world.
This has led to an increased proportion of LMs being diagnosed
during the antenatal period. A high incidence of associated
chromosomal abnormalities has been noted in prenatally diag-
nosed LMs and therefore amniocentesis is advised in cases
presenting with polyhydramnios, fetal hydrops and fetal LMs
[10]. Once the suspicion of an obstructing neck mass is raised on US
further imaging is essential in order to evaluate the extent of
airway obstruction. In their report of 31 cases Bouchard et al.
employed ultrafast MRI imaging with Half-Fournier single shot
turbo spin echo sequence [18]. If this confirms airway compromise
an ex utero intrapartum treatment procedure (EXIT) should be
planned.
The senior author of this report has a special interest in
paediatric airway management and has been involved in EXIT
procedures in the past. Other members of the multidisciplinary
team include anaesthesiologists, obstetricians, neonatologists and
paediatric surgeons. The procedure is performed under general
anaesthetic. A hysterotomy is performed and the fetal head and
shoulders are delivered. In certain instances decompression of the
cystic mass can be performed under ultrasound guidance prior to
hysterotomy. Placental separation is prevented and adequate
uteroplacental blood flow is maintained by ensuring uterine
relaxation, normal mean arterial pressure and by maximizing
uterine volume. Once the head and shoulders are delivered the
fetus is monitored throughout the procedure with pulse oximetry
and echocardiography. The airway is then secured. This is achieved
via endotracheal intubation following direct laryngoscopy or rigid
bronchoscopy. If this fails reverse endotracheal intubation is the
next described option. This is achieved by isolating the trachea and
performing a tracheostomy where after a fine feeding tube is
passed back through the trachea and into the oral cavity. An
endotracheal tube can then be advanced over the feeding tube into
the trachea allowing closure of the tracheostomy. If this is not a
feasible option a formal tracheostomy is performed. Once the
airway is secured manual ventilation is started and surfactant
administered. Finally the cord is clamped only after the insertion of
an umbilical arterial line and upon achieving good oxygen
saturation [18].
The treatment of LMs still poses a formidable challenge to those
confronted with managing this rare entity. Observation, aspiration,
surgery and sclerotherapy are just some of the treatment
modalities described. Some authors view spontaneous regression
of LMs as unlikely and rare [5,14]. The weight of evidence does
however support spontaneous regression of LMs [7–10,19], some
describing rates of up to 15% [3]. Because of these findings some
physicians opt to manage asymptomatic lesions conservatively for
up to 24 months [8,10]. Extreme vigilance is required in managing
these lesions conservatively as sudden expansion of these lesions
can occur as described above. In a recent study Perkins et al. [19]
identified certain radiologic characteristics that indicate a high
likelihood of spontaneous regression. These features included LMs
with predominantly macrocystic tissue, less than five intracystic
septations and limited extent. These are however lesions that
would typically respond well to other forms of management as
well.
Simple aspiration of LMs whilst once a recognised treatment
option and still favoured by some [9], is now rarely used due to
rapid recurrence, infection and haemorrhage [3]. However needle
decompression may be beneficial in instances where there is rapid
expansion of the lesion with concomitant airway compromise
[3,10].
Sclerotherapy on the other hand has long been proposed as an
alternative to surgery in an effort to avoid its hazardous
complications. Some of the substances used in practice included
bleomycin, ethibloc, tetracycline, dextrose and sodium morrhuate
 J.C. Oosthuizen et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 398–403
[1]. These agents have largely been abandoned due to the fact that
they have the potential to cause scarring and contraction of
surrounding tissues, making subsequent surgery more difficult
[1,20]. One of the more successful of these was bleomycin,
however due to concerns regarding pulmonary fibrosis it is no
longer in use [20]. In 1987 Ogita et al. [21] described the use of OK-
432 as sclerosing agent in the management of LMs and it has since
attracted considerable attention. OK-432 (Picibanil, Chugai Phar-
maceutical Co., Tokyo) is a lyophilized biologic preparation of
group A Streptococcus pyogenes cells treated with benzylpenicillin
and is therefore contraindicated in patients with a penicillin
allergy [20]. There have been numerous reports on the successful
use of OK-432 since then [1,2,20,22,23] and is increasingly being
proposed as a first line treatment option in LMs. Compared to other
sclerosants OK-432 has the advantage that following administra-
tion there is no perilesional fibrosis and therefore subsequent
surgery is not made more difficult or hazardous [1,23,24].
Macrocystic lesions respond much more favourably than micro-
cystic lesions to the injection of OK-432. In a multicentre
prospective trial, Giguere et al. demonstrated a success rate of
66% which soars to 86% if microcystic cases are excluded [2].
Percutaneous administration of OK-432 can lead to a variety of
adverse reactions which include erythema, discomfort at the
injection site, pyrexia and swelling [2]. Despite being rare there
have been reports of rapid expansion in cyst size leading to airway
compromise, requiring surgical intervention in order to secure the
airway [2,20]. The authors of this report therefore feel that it might
be prudent to secure the airway, by means of a tracheostomy, prior
to OK-432 injection where there is the potential for airway
compromise. The use of doxycycline as a sclerosant in LMs has
previously been reported [25,26]. These reports were however
limited to use in macrocystic and combined cases. In a recently
published paper, Shiels et al. were the first to report successful
ablation of microcysts using ultrasound guided cyst drainage and
injection with high dose doxycycline [27]. They attribute the high
success rate to complete cyst aspiration as opposed to simple
needle injection and the high dose of doxycycline used (20 mg/
mL). Despite being a single, retrospective report their findings are
very promising and certainly warrant further investigation. In our
case series five patients (71%) were treated with OK-432 injection
under direct ultrasound guidance. Unfortunately two of these
patients were lost to follow up. Two of the remaining three patients
(66%) had a successful result whilst the remaining patient, whom
suffered from predominantly microcystic disease, showed no
response whatsoever.
Fig. 1. Management of antenatally diagnosed LM.
401
Microcystic disease of the oral cavity is notoriously difficult to
treat with a large percentage of cases showing persistent or
recurrent disease. Traditionally these lesions have been managed
surgically with conservative resection in order to avoid the
morbidity associated with complete excision; this however leads
to high recurrence rates and the need for repeated procedures [28].
In our case series 21% of cases suffered from microcystic disease of
the oral cavity. All of these cases were principally treated with
surface KTP laser therapy to control symptoms. The use of laser
therapy in the management of these lesions has been previously
documented [29]. Laser treatment offers the following advantages
over surgery: less postoperative oedema, less tissue trauma and
less blood loss [3]. Favourable results were obtained with this
modality; however recurrent disease was encountered in 66% of
cases treated for LMs of the oral cavity. Another described
treatment option for microcystic disease of the oral cavity is
radiofrequency ablation therapy [28,23,30,31] which has recently
shown promising results.
Traditionally surgical excision (Figs. 3 and 4) of LMs has been
the mainstay of treatment [12,32]. The complete surgical excision
of these lesions offers excellent results. However due to the close
proximity of vital structures this can be very challenging and leads
to partial resection in approximately 60% of cases [9]. Recurrence
rates of partially or incompletely resected lesions are reported to
be as high as 50–100% of cases [3]. Surgery is associated with
significant morbidity and some of the recognised complications
include muscle weakness, seroma formation, infection e.g.
mediastinitis, chylothorax, Frey and Horner’s syndrome, injury
to the cervical oesophagus and cranial nerve damage [3,7,9].
Cranial nerve injury can occur in up to a third of LMs treated
surgically with the facial nerve most frequently involved [4].
Half of the patients in our case series were treated surgically
with only one patient in this group lost to long term follow up.
Some of these had undergone surgery prior to being referred to our
institution. In the majority (66%) of these cases a favourable
outcome was achieved. Two patients developed cranial nerve
palsies, one facial and the other hypoglossal nerve. The first patient
had been operated previously at another institution and the
hypoglossal nerve palsy was present at the time of referral to our
institution. Both of these patients had extensive cervicofacial
disease encompassing both the infra and suprahyoid regions (stage
3) and in both cases the nerve damage occurred during revision
surgery.
Based on experience in managing these tumours as well as
current literature the authors of this report have developed a
402 J.C. Oosthuizen et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 398–403

Fig. 2. Proposed management of LMs.

management algorithm for paediatric cervicofacial LMs. The aim of
this algorithm is to provide definitive management in the majority
of cases prior to school going age. As already mentioned above,
surgery was the principal management option in the past, however
its complication and recurrence rate are unacceptably high in what
is essentially a benign condition. The spontaneous regression of
LMs whilst once described as unlikely has since been well
documented and therefore in asymptomatic lesions the authors
of this report would advocate expectant management initially.
Should this fail OK-432 injection therapy offers an excellent
alternative to surgery due to its high success rate, especially in
macrocystic disease, as well as the lack of perilesional fibrosis
which does not compromise future surgery if required. Sclerother-
apy of microcystic disease with high dose doxycyline is a
promising development and is something that might be consid-
ered as an adjunct to OK-432 in future. Finally should sclerothera-
py fail the degree of cosmetic and functional impairment should be
thoroughly assessed and weighed against the potential risks of
surgery. It is however of utmost importance that each case be
assessed on an individual basis in order to achieve the best possible
outcome and in certain instances this might necessitate deviation
from the guidelines above.
5. Conclusion
LMs are essentially benign tumours and the management of
these should reflect this. Even though surgery might have been the
principal management option in the past, its complication rate is
unacceptably high in the management of a benign condition. This
combined with the prospect of new innovative management

Fig. 3. Cystic hygroma in situ. Fig. 4. Isolated facial nerve during surgical excision.
 J.C. Oosthuizen et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 398–403
Fig. 5. Lymphatic malformation with mediastinal extension.
options that have been proven to be effective, have led the authors
of this report to formulate a proposed algorithm (Figs. 1 and 2) for
the management of LMs.
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