Cystic Hygroma: A presentation of three cases in the
orofacial region
Sameer M. Khateery, BDS, FAAHD, MS,
Carnal A. Swaify, BDS, MD, PhD
Oral and Maxillofacial Surgery Department, Riyadh Dentai Center, Riyadh, Saudi Arabia
Three cases of cystic hygroma treated at the Department of Oral and
Maxillofacial Surgery are presented. Methods of diagnosis and treatment are
discussed
Hygroma cysticum coli or cystic hygroma is a division of
lymphangiomas that presents mostly at birth, it is therefore a congenital
malformation of the lymphatic system.1,2 Most of
these tumors are present by the time the patient reaches the age of 3 to 5
years.2
The most common site for the tumor is the posterior
triangle of the neck and it may involve vital structures, such as the
sympathetic chain, carotid sheath content, and branches of the hypoglossal,
lingual, and the facial nerves.2 Depending
on the anatomical site, cystic hygroma may cause dysphagia or airway
obstruction and respiratory distress that necessitates immediate surgical
interference.3'4'5
The cystic hygroma in the neck is manifested as large, deep,
diffuse swelling.1,5 On palpation, it is often doughy and is
usually transilluminant. These lesions are composed of lymph containing
endothelial spaces that vary in size from capillary dimensions to cysts of
several centimeters in diameter.6 Although,
the embryologic origin is still unclear, failure of primary lymph spaces to
join the central lymphatic system (thoracic duct and right lymphatic duct) and
the venous system may be the basis for their development. Cystic hygroma is
usually multiloculated and is composed of spaces. These spaces contain straw
colored fluid which may be discolored with secondary infection of intracystic
hemorrhage.7
It may also cause obstructive sleep apnea. Such apnea is a
different sign of presentation than the ones that are commonly observed. Commonly,
the hygroma manifests itself as a uni- or bilateral swelling of the neck that
causes asymmetry.8 Although surgical excision of the tumor, is
the treatment of choice for most clinicians, local injection of bleomycin fat
emulsion could be another successful alternative.9
In this report, we are presenting three cases of cystic
hygroma that were treated since August 1990. Two of the cases were completely
excised, while the third one was partially excised. Facial nerve palsy was not
noticed in any of them postoperatively. Hospitalization period ranged from
5-17 days.
Case 1
A ten-year-old Saudi female referred to our department at
Riyadh Dental Center (RDC) with a large swelling of the right parotid and
submandibular regions. The referral diagnosis was residual buccal fat. The
swelling existed for 5 years. When the patient was 6 years old, the lesion was
diagnosed as "lipoma" and was operated by a general surgeon in order to remove
it through an intra-oral approach, but the treatment was not successful.
Examination of the patient revealed soft, diffuse, mobile
large swelling over the right parotid and submandibular regions [Fig. 1].
Transillumination test was positive. Clinical diagnosis of cystic hyg-roma was
made. CT scan showed the extent of the tumor [Fig. 2].
The patient was then admitted to RDC for surgical removal
of the lesion. Through a parotidectomy incision, about 70% of the lesion was
excised, leaving the deep (medial) portion. The facial nerve branches were
identified and preserved. The patient was hospitalized for 17 days and was
discharged in a satisfactory condition. There were no facial nerve deficit.
Unfortunately, the central part and tip of the elevated skin flap suffered a
deep second degree burn due to application of hot compress during the first two
days after surgery which required further management.
Case 2
A three-year-old Kuwaiti female presented to the Oral and
Maxillofacial Surgery Clinic, RDC with a large swelling of her left
submandibular and pre- auricular region. The swelling was asymptomatic except
for the disfigurement [Fig. 3]. It was present at birth and increased in size
subsequently. Upon examination, the swelling was soft, diffuse, about 6 cms. in
diameter. Transillumination test was positive. Aspiration of the lesion gave
clear, yellow fluid. A CT
scan was requested and showed a cystic lesion with clear boundaries. A
diagnosis of cystic hygroma was made. A decision was made to surgically remove
the tumor. The patient was admitted to RDC, and the lesion was excised completely
through a modified pre-auricular incision. The facial nerve branches were
identified and protected after exposing the trunk just after its exit from the
stylo-mastoid foramen [Figs. 4a and b]. The lesion involved the skin and was
not dissected easily. The incision was closed in layers after the insertion of
a vacuum drain.
Case 3
A thirteen-year-old Saudi boy presented to our department
complaining of large swelling over the left preauricular region. The swelling
started two years earlier after the patient received trauma to that area. A
general surgeon aspirated the lesion on two occasions after which the swelling
subsided, to regain its size within a few weeks.
Clinical examination revealed soft, diffuse swelling
involving the left parotid and sub- mandibular regions [Figs. 5a and b].
Aspiration of the lesion gave brownish fluid [Fig. 6]. CT scan was requested
and showed large cystic lesion with definite boundaries I Fig. 7].
The facial nerve was identified, its branches were
preserved and the lesion was completely excised through a parotidectomy
incision [Figs. 8a and b], and the incision was closed in layers after drain
insertion. Post-operatively, there was slight weakness of the frontal branch
which recovered completely, in four weeks [Fig. 9]. The patient has had a
satisfactory hospital course and left the center on the 5th day
post-operatively.
Surgical excision is the treatment of choice for cystic
hygromas. Cystic hygroma in the orofacial region is a difficult lesion to
excise. Although the tumor is benign, the intimate relation to the various important
anatomic structures makes its dissection a tedious task.
Involvement of the skin makes it a risk during raising the
skin flap and even after successful elevation, the sensibility of the skin is
jeopardized which may be dangerous if the patient is instructed to apply hot
compresses. Extension of the lesion and involvement of muscle and glandular
structure make complete excision sometimes impossible. When complete excision
is sought, unnecessary sacrifice of muscles will leave the patient with a
deformity which is more difficult to treat as it constitutes a deformity in the
form of a defect and a functional one in the form of loss of muscle function.
Partial excision may be the solution, even though recurrence is possible
following incomplete excision. The goal in the management of cystic hygromas
must be primarily to relieve obstruction upon vital tissues and a good cosmetic
result.
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