Cystic hygroma (lymphangioma) is often a benign congenital malformation of the lymphatic system that occurs as a result of sequestration or obstruction of lymphatic vessels. Theselesions are usually discovered in infant or children younger than two years of age.Occurrence in adults is uncommon, and fewer than 100 cases of adult lymphangioma have been reported in the literature (1,2) . The objectives of this case report are to present theclinical history and surgical findings, review of literature and the unique problems encountered in the surgical management of this particular patient

Karunakaran, Anupama

Ramamoorthy, Muthukumar

English

Otolaryngology online journal

 
www.jorl.net 
 
 
 
Volume 2 Issue 2 2012      ISSN 2250- 0359 
 
Muthukumar Ramamoorthy *  Anupama Karunakaran* 
 
*Madras Medical College 
 
 
GIANT CYSTIC HYGROMA COLLI IN AN ADULT- A RARE CASE REPORT 
 
 
INTRODUCTION 
 
Cystic hygroma (lymphangioma) is often a benign congenital malformation of the lymphatic 
system that occurs as a result of sequestration or obstruction of  lymphatic vessels. These 
lesions  are  usually  discovered  in  infant  or  children  younger  than  two  years  of  age. 
Occurrence in adults is uncommon, and fewer than 100 cases of adult lymphangioma have 
been reported in the  literature 
(1,2) . The objectives of this case report are to present the 
clinical  history  and  surgical  findings,  review  of  literature  and  the  unique  problems 
encountered in the surgical management of this particular patient 
 
Case report 
 
       HISTORY 
  A  75  yr  old  south  Indian  male,  an  agricultural  labourer  presented  in  our  OPD  with 
complaints of swelling in the neck of 2 years duration. It was slowly increasing in size snd 
posed  a  restriction  to  neck  movements.      He had  no  complaints  regarding  his  voice  or 
deglutition.. 
He was particularly alarmed and concerned about the fact that following aspiration of the 
swelling a couple of weeks ago by a private practitioner the swelling had reappeared with 
increased vigour and attained its original size. He was not a diabetic and had no history of 
trauma or respiratory tract infection in the recent relevant past. 
 
       Physical  examination  revealed  a  diffuse,  smooth,  soft  fluctuant  non  transilluminant 
swelling measuring 20 /18 cm which occupied the whole of the anterior neck pushing the 
right  sternocleidomastoid  muscle  and  carotid  laterally.  The  swelling  was  predominantly 
present  over  the  right  side  and  encompassed  the  submandibular,  carotid,  muscular  and 
posterior triangles on the right side.Indirect laryngeal examination revealed normal mobile 
vocal cords.  
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On the left side it did not cross lateral to the sternomastoid muscle .the larynx and trachea 
were not palpable in the midline and were pushed far to the left and were palpable along the 
lateral border of the neck on the left. The left carotid was palpable in normal position and was 
not displaced. A presumptive diagnosis of a cystic hygroma was made and investigations 
were carried out. 
IMAGING     
The contrast enhanced CT of the neck revealed a non enhancing, homogeneous soft tissue 
mass in the neck extending from the submandibular region to the supraclavicular fossa. The 
airway was pushed to the left and there was no retrosternal extension of the lesion. There was 
normal enhancement of the great vessels and were normal except for the lateral displacement, 
 
  
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Aspiration revealed serosanguinous fluid with normal epithelial cells and a few inflammatory 
cells. No malignant cells were seen. 
SURGICAL TECHNIQUE. 
        This case posed unique problems because of the deviated airway. Intubation was done 
blindly over a bougie with a cuffed size 8 endotracheal tube.Atransverse skin crease incision 
was made 5 cms above the suprasternal notch. Subcutaneous tissue was dissected and the 
platysma incised. the anterior jugular veins were encountered and ligated on both sides. The 
strap muscles were found to be stretched and thinned out over the swelling. While opening 
the  strap  muscles  the  swelling  was  in  advertantly  punctured  and  about  2  litres  of 
serosanguinous fluid was let out. The cyst wall found to be thickened andcontained follicles 
on  the  inner  secretory  surface.  The  cyst  wall  was  completely  dissected  in  toto  from  the 
surrounding  structures  and  was  found  to  be  slightly  adherent  to  the  thyroid  and  cricoid 
cartilage.however a plane of cleavage allowed complete dissection from the cartilage. The 
hypoglossal nerve was encountered on the right during dissection and was preserved. There 
was no injury to any of  the neurovascular structures of the neck. The excessively stretched 
out skin was trimmed and the wound was closed in layers with a drain.  
 
There was a serous collection in the drain which continued for three days and eventually 
stopped.  Tight  compression  dressing  was    made  to  avoid  recollection.  The  patient  was  
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discharged on the 10
th day without any complications. It has been one year following surgery 
and patient has had no recurrent swelling. 
 
 
 
HISTOPATHOLOGY 
Histopathologic diagnosis was cystic lymphangioma which wassupported with a thick fibrous 
wall, containing dilated blood vessels partly filled with erythrocytes andinfiltrated with 
lymphocytes 
DISCUSSION.  
Cystic hygroma or giant lymphangioma is a benign malformationof the lymphatic system.It is 
believed to arise from a congenital malformation of the lymphatic system in which a failure 
of  communication  between  the  lymphatic  and  venous  pathways  leads  to  lymph 
accumulation
1. Most cystic hygromas present in-utero or in infancy and therefore most ofthe 
literature on  management  considers  paediatric  cases.  The  effect  of  these  lesions  depends 
ontheir position and relationship to surrounding structures, Although the lesion can occur 
anywhere, the most common sites are in the posterior triangle of the neck (75%), axilla(20%), 
mediastinum (5%), groin, retroperitoneal space and pelvis
2 To date there have been fewer 
than 150 reports of adult cervicofacial cystic hygroma in the English language literature and 
the optimum management of these lesions is still a matter ofdebate.. Thirty-two patients with 
cervical  lymphangioma  were  treated  at the  Mayo  Clinic;  this  is  the  largest  series  of  the 
literature
3.  However,  rapid  enlargement  over  a  short  period  of  time  has  frequently  been 
reported
3 and major structures such as the larynx, trachea, oesophagus, brachial plexus and 
great vessels have known to be compressed or incorporated within the lesion. .. Diagnosis in 
adults is considered to present a greater challenge than in children and initial misdiagnosis, 
frequently as branchial cleft cysts
1 
 
There  are  three  histologicalsubtype.  Capillary  lymphangioma  (composed  of  small 
lymphatics),  cavernous  lymphangioma  (composed  of  larger  lymphatics),  cystic 
lymphangioma  (cystichygroma-  composed  of  large  macroscopic  lymphatic  spaces  with 
collagen and smooth muscle)
1. Cavernous lymphangioma is the most common subtype. But 
cysticlymphangioma  occurs  approximately  1  in  12000  births  and  95%  occurring  by  the 
second year of life
1.  
Cystic  hygroma  could  be  classified  into  septated  (multiloculated)  or  non-septated  single 
cavity (non-loculated). Presentation in adulthood is rare and the cause is uncertain, although  
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trauma and upper respiratory tract infection have both been suggested as possible triggers for 
onset
[5,6].In this case there was no identifiable cause and onset was sudden 
 Proposal for staging of lymphatic malformations of the head 
and neck (adapted from de Serres[8]) 
Stage Location of lesion 
I Unilateral infrahyoid 
II Unilateral suprahyoid 
III Unilateral infrahyoid and suprahyoid 
IV Bilateral suprahyoid 
V Bilateral infrahyoid and suprahyoid 
Our case belonged to stage v 
Complete  surgical  excision  has  traditionally  been  considered  the  treatment  of  choice  for 
cystic hygroma
[6]. However, several authors have suggested that sclerotherapy may be a more 
appropriate  first-line  therapy
[7].  Although  sclerotherapy  is  now  well  established  in  the 
treatment of neonatal and paediatric cystic hygromas, there have been relatively few cases 
reported of its use in adult patients. Some success has been reported in small numbers of 
adults with sclerotherapy agents such as OK-432
[7]. Caution has been urged with the use of 
agents suchas OK-432 which induce a local immune response that often results in a rapid 
temporary increasein the size of the cystic hygroma
[7]. Depending on the anatomical relations 
of the tumour, suchincreases in size may be intolerable and it has been suggested that such 
therapy  should  only  beadministered  in  specialised  facilities  due  to  the  risk  of  airway 
obstruction
[9] 
.Smith et al
.[7] compared results from their large-scale trial of OK-432 with pooled results 
fromlarge surgical case series reported in the literature and reported greater success rates and 
loweroccurrence of major complications with OK-432 sclerotherapy compared with surgical 
excision. However, their study focussed mainly on children and only one of the comparative 
surgical caseseries included adult patients. Several authors have expressed the opinion that 
surgical excision of cystic hygroma is an easier procedure in adult patients, because these 
lesions are bettercircumscribed, and as such the success rate is greater
[2,5]. 
In this case,  it was thought that the  ideal treatment would  be complete surgical  excision 
asmultiloculated cystic hygroma may not respond to sclerotherapy. Success of surgery has 
beenfound to correlate with histology, encapsulation, complete excision, anatomical location 
and  stageof  the  lesion
1.Imaging  appeared  to  show  smooth  margins,  indicating  a  lack  of 
infiltration,  which  is  a  good  prognostic  feature,  facilitating  complete  removal  and  low 
recurrence.  However,  this  was  an  extensive  stage  V  lesion  with  close  relations  to  major 
structures and therefore adifficult procedure was anticipated. It proved to be impossible to 
remove  the  cystic  hygromacompletely  without  rupture,  a  recognised  problem  as  these 
tumours usually  have a  fragile thinwall. Intra-operative rupture of the  lesion  complicates 
complete removal as it obscures the limitsof the structure
[1]. However, Riechelmann
6reported 
very low levels of recurrence (1/9 patients)following subtotal excision when small plaques of  
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tumour wall were known to be left in situ.To the best of our knowledge we were able to 
remove the cystic hygroma completely.Complications of surgery should be discussed with 
the patient before consent is obtained forsurgery, including 
-Difficult intubation 
_ Scar: there may be a long scar depending on the size of the cystic mass but this should 
usuallyimprove over time. 
_ Injury to important structures and nerves: all blood vessels and nerves located betweenthe 
mandible and the sternomastoid muscle are vulnerable to injury,specifically themandibular 
branch of the facial nerve, the spinal branch of the accessory nerve and the greaterauricular 
nerves and hypoglossal nerves. 
_  Venous  bleeding  is  a  possibility  but  injury  to  the  carotid  sheath  and  its  content  and 
someexternal carotid branches should be rare. 
_ Wound Infection. 
_ Recurrence after surgery is a possibility. 
CONCLUSION 
_ Cystic hygroma are congenital neck masses usually presenting at birth but may present at 
any age 
_ Cystic hygroma is a rare presentation in adults 
_ Malignancy should be excluded in all adult patients presenting with a cystic neck swelling 
Adult-onset cystic hygroma 
Differential diagnoses for a congenital neck mass in adults 
– Branchial cleft cyst (lateral to midline) 
– Dermoid cyst (midline) 
– Thyroglossal cyst (midline) 
– Haemangioma 
– Thymic cyst (midline/lateral) 
– Neck malignancy (midline/lateral) 
 Investigations should include: 
– Ultrasound scan 
– Fine-needle aspiration (important to exclude malignancy) 
–Computed tomography or Magnetic resonance imaging  
_ Final diagnosis depends on tissue analysis  
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Treatment options in adults include: 
– Surgery 
– Sclerotherapy 
_ In multiloculated cystic hygromas, surgery may be the preferred option but it is important 
thatsurgical complications are kept to a minimum. 
 
 
REFERENCES 
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Khan  and  F.  MihaimeedGrand  Rounds  Vol  11  pages  5–11Specialities:  General 
SurgeryArticle Type: Case ReportDOI: 10.1102/1470-5206.2011.0002 
2 . Woods D, Young JE, Filice R, Dobranowski J. Late-onset cystichygromas: the role of CT. 
Can AssocRadiol J 1989; 40:159-161. 
3 de CassoMoxo C, Lewis NJ, Rapado F. Lymphangioma presenting as a neck mass in the 
adult.Int J ClinPract 2001; 55: 337–8. PMid:11452685. 
4Schefter RP, Olsen KD, Gaffey TA. Cervical lymphangioma in the adult. Otolaryngol Head 
NeckSurg 1985; 93: 65–69. PMid:3920626. 
5. Antoniades  K, Kiziridou A, Psimopoulou M. Traumatic cervical cystic  hygroma. Int J 
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6.  Riechelmann  H,  Muehlfay  G,  Keck  T,  Mattfeldt  T,  Rettinger  G.  Total,  subtotal,  and 
partialsurgical removal of cervicofaciallymphangiomas. Arch Otolaryngol Head Neck Surg 
1999;125: 643–8. PMid:10367920. 
7.  Smith  MC,  Zimmerman  MB,  Burke  DK,  et  al.  Efficacy  and  safety  of  OK-432 
immunotherapyof  lymphatic  malformations.  Laryngoscope  2009;  119:  107–15. 
doi:10.1002/lary.20041.PMid:19117316 
8. Baer S, Davis J. Cystic hygroma presenting in adulthood. J LaryngolOtol 1989; 103: 976-
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9. Schefter RP, Olsen KD, Gaffey TA. Cervical lymphangioma in theadult. Otolaryngol Head 
Neck Surg 1985; 93: 65-69. 
10. Aneeshkumar MK, Kale S, Kabbani M, David VC. Cysticlymphangioma in adults: can 
trauma be the trigger? Eur ArchOtorhinolaryngol 2005; 262: 335-337. 
 

1 Adult-onset cystic hygroma: a case reportand review of

Cervical lymphangioma in theadult. Otolaryngol Head Neck Surg

Cystic hygroma presenting in adulthood.

Cysticlymphangioma in adults: can trauma be the trigger? Eur ArchOtorhinolaryngol

Efficacy and safety of OK-432 immunotherapyof lymphatic malformations. Laryngoscope

subtotal, and partialsurgical removal of cervicofaciallymphangiomas. Arch Otolaryngol Head Neck Surg 1999;125:

Traumatic cervical cystic hygroma.

GIANT CYSTIC HYGROMA COLLI IN AN ADULT- A RARE CASE REPORT

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GIANT CYSTIC HYGROMA COLLI IN AN ADULT- A RARE CASE REPORT

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