Cervicofacial cystic lymphatic malformations: Does surgical excision remain the first option?

Cervicofacial cystic lymphatic malformations: Does surgical excision remain the first option?

Mohammed Hassan Osman, MDa; Ibrahim Ali Ibrahim, MDb a) Maxillofacial Unit, Assiut University Hospital, Assiut, Egypt.

b) Pediatric Surgery Unit, Assiut University Hospital, Assiut, Egypt.

Objectives:  Treatment  of cervicofacial  LMs  is challenging.    The aim of this  study is to evaluate the results of different modalities in the management ofCLMs in an attempt to develop an applicable algorithm for the management ofthese cases.

Patients and methods:  26 patients with a CLM were classified into 3 groups:

•       Group A: Patients with isolated cervical cystic malformations (18/26 patients).

•       Group B: Patients with oropharyngeal extension (7/26 patients).

•       Group C: Patients with mediastinal extension (1/26 patient). Results: Group I:

•       4 patients presented  at birth. They  were subjected  to needle aspiration  followed  by

urgent surgical excision to alleviate respiratory distress. One ofthem needed another setting. Results were regarded as excellent.

•       5th child  presented  with infection  at  age of 2 months  with  rapid enlargement  and

respiratory distress. Urgent Surgical drainage was done to alleviate respiratory distress and to control fever. The LM completely disappeared.

•       8 patients with isolated cervical LM underwent elective surgical excision, 7 of them had

excellent results. The 81h one developed injury to the marginal mandibular nerve.

•      The  remaining 5  patients underwent  injection  sclerotherapy  with  98% ethanol  with excellent results.

Group II: 7 patients were subjected to injection with 98% ethanol. 5 of them had excellent

results and 2 needed surgical intervention to the cervical part for cosmetic purpose.

Group III: One patient underwent surgical excision with excellent results.

Conclusion: Surgery remains the first option in certain circumstances, however  injection with 98% ethanol is recommended for oropharyngeal extension as surgical excision is very difficult and dangerous.

Key words: Cystic lymphatic malformation, management options, sclerotherapy. Abbreviation: Cervicofacial lymphatic malformations CLMs.

Introduction:

Lymphatic  malformations  (LMs)  are benign tumors of the lymphatic system characterized by anastomosing lymphatic channels and cystic spaces of different sizes.l They may arise anywhere in the developing lymphatic system; however, the cervicofacial lymphatic  malformations  represent  75%  of the cases Figure (1).2

LMs  constitute  6%  of all benign  lesions

of infancy and childhood3 and Sixty percent

are  present  at  birth,  and  80%  to  90%  of cases  become  symptomatic  within  the  first two   years   of   life.4    The  management   of cervicofacial   LMs  is  challenging   because of  the  close  association  with  the  adjacent vital    structures         and       poor            demarcation.5

Different lines are used to treat lymphatic malformations including surgery, aspiration drainage, laser therapy and sclerotherapy or a combination these.

Surgical excision has been considered the

Am-ShamsJSurg2014; 7(2):407-414

therapeutic gold standard for management of lymphatic  malformations;   however  surgery has several disadvantages as incomplete resection  owing  to  infiltration  of  adjacent vital structures, high recurrence rate, damage to the surrounding structures, and wound infection. Aspiration, radiation, and laser therapy have been tried but with bad results. More  recently,  percutaneous   sclerotherapy has   gained   popularity.   Several   sclerosing agents have been used including OK-432, bleomycin, fibrin glue, doxycycline,  50% dextrose, absolute alcohol and Ethibloc.5,6,7

We reported  our experience  in managing cystic lymphatic malformations of the neck with surgery, aspiration, drainage, absolute alcohol  sclerotherapy  or  combination   in  a trial  to reach to an applicable  algorithm  for the management of cervicofacial lymphatic malformations.

Patients and methods:

26  patients  (9  males  and   17  females) with-a cystic lymphatic malformation  of the neck were managed in the maxillofacial  and pediatric Surgical Units in Assiut University hospital, Egypt. The diagnosis was based on the clinical examination ultrasonography, CT scan and MRI for some cases. Size, nature, extension, and number of the cystic cavities were  defined.  The  patients  were  classified into 3 groups:

Group  A: Patients  with  isolated  cervical

cystic  malformations  (macrocystic  >1  em:

18/26 patients),

Group B: Patients with oropharyngeal extension (mixed: 7/26 patients) and

Group C: Patients with mediastinal extension (macrocystic: 1/26 patient).

The  age of the  patients varied  from  one

day up to 30 years.

Results:

Group I: Isolated cervical cystic lymphatic malformation.

This group had no evidence of oropharyngeal or mediastinal extension as proved clinically and by investigations. There were 18 patients who presented between birth and 7 years.

•     Four patients presented at birth with massive cervical LM and respiratory distress, rapid  intubation  and admission  in  the  ICU was done. The 4 patients underwent needle aspiration for rapid decompression  followed by urgent surgical excision to alleviate respiratory distress. 7 days later the tube was removed. One of them needed another setting after one year to complete the resection. Results were regarded as excellent Figure (2).

•       5th child presented  with infection  at

age of 2 months with rapid enlargement  and respiratory distress. Urgent surgical drainage was done to alleviate respiratory distress and to  control  fever.  The  patient  was  followed up about 6 months and the LM completely disappeared with no need for another intervention.

•       Eight  patients  with  isolated  cervical

LM  underwent  elective  surgical  excision,  7 of them had excellent results (no recurrence or complications). The 8th one developed injury to the marginal mandibular nerve and was regarded as moderate result Figure (3).

•      The    remammg    5    patients    with isolated cervical LM underwent injection sclerotherapy with 98% ethanol with excellent results   (no   recurrence   or   complications). In  our  hospital  we  use absolute  alcohol  as a sclerosing argent as it has minimal side effects, easy to purchase, and of low cost Figure (4).

Group II: Patients with oropharyngeal extension:

Seven patients presented with neck LMs with oropharyngeal extension. Surgical excision was regarded as very difficult and dangerous, so, these patients underwent injection with 98% ethanol under general anesthesia.  5  of them  had  excellent  results and 2 needed surgical intervention to the cervical part for cosmetic purpose.

Technique of sclerotherapy: The injection

of  ethanol  was  performed  under  local  or general anesthesia depending on the level of cooperation of each child.

The first step was placing the patient on CT

table in a supine position with the head rotated to the opposite side of the lesion. Under CT guidance, a direct puncture of the lesion was

Figure (1): Cystic hygroma of the neck.

Figure (2): A baby with massive cervical L.J\1: (a) Immediately after birth (b) Intra-operative

(c) After the surgical excision.

Figure (3): A child with left sided cervical LM (a) Pre-operative (b) Intra-operative (c) In the follow up period.

performed  using  a  20-gauge  needle. This was followed by aspiration of as much of the cystic fluid as possible. The volume of cystic fluid was recorded. The volume of alcohol injected vruied depending on the size of the lesion approximately equivalent to 50% of the aspirate fluid volume. After injection, another CT image slice was perfonned on the lesion. All patients were observed for 24 hours after the procedure. Clinical and imaging follow­ up by US was performed at 1, 3 months and

6 months.

Group III: Patient with mediastinal extension:

One patient presented with neck LMs with

mediastinal  extension  underwent  surgical excision with excellent results Figure (5).

Technique: The operation was performed tlu·ough a combined cervical approach  for the cervical prut and a tight anterolateral thoracotomy for the intrathoracic component.

The patient was placed in a supine position

L

Figure (4): A child with right sided cervical L.M: (a) Before sclerotherapy (b) In the follow up period.

Figure (5): Female patient 30 years with cystic IM  of the neck extended to the mediastinum: (a) The cervical part (b) CT showing the mediastinal part (c) CT of the cervical part

(d) Intraoperative photo shows thoracotomy and the mediastinal mass (e) Intraoperative photo shows the cervical mass.

and the tight side was elevated from the table by a bag below the right side of the back. Cetvical approach: through a lower collar incision, a flap was elevated with creating a plane of dissection that left the capsule of the cyst intact allowing a complete excision of the cyst, after that the right anterolateral thoracotomy was performed. The skin incision   followed  the   submammruy  fold and  extended  fi:om the  sternum  antetiorly to the midaxillruy line giving access to the

fifth intercostal space. The skin incision was cru1ied down through the subcutaneous tissue and superficial fascia to the pectoralis major muscle. Postetiorlytothe anterioraxillruy line, the digitations of the senatus antetior muscle were exposed. The superficial pectoral fascia and the pectoralis major muscle were divided with electrocaute1y. The 5th intercostal space was opened and the chest cavity was entered using a self retaining retractor allowing good exposure of the cyst preserving the capsule

LM

[

with life threatenning conditiot1

-+'/-severe deformity

urgent syrgical intervention

+/-preliminary tracheostomy

+/-preliminary aspiration

urgent syrgicalintervention

+/-preliminary tracheostomy

+/-preliminary aspirat on

Figure (6): Algorithm of treatment ofLi\1s of the head and neck.

intact and allowing complete excision of the cyst. This was followed by insertion of an intercostal tube.

Post operative  was smooth  with removal

of  the  intercostal  tube  on  the  4th  day  and chest x-ray showed complete inflation of the tight lung. Follow up showed no recunence or complications.

Discussion:

Lymphatic malformations result fi:orn abnormal development  of lymphatic  vessels or due to abnonnal  communication  between the lymphatic and venous system. It may be congenital or acquired secondruy to smgety, trauma, infections, neoplasms or chronic infl.runrnation.s All om cases have no acquired cause.

LMs  could   be  classified   according   to

the  diameter  of  the  cyst  into  rnacrocystic (>1 ern) or  rnicrocystic  (<1 ern),  but  mixed (macro- and rnicrocystic) fmms ru·e usually present  clinically,  such  as  rnacrocystic lesions in the neck with rnicrocystic lesions in  the  tongue  or  cheek.9   Also  LMs  could be classified according to extension into isolated or simple when affecting the neck or with orophruyngeal extension when extends fi:orn the neck into  the orophruynx  or  with

mediastinal extension when extends from the neck to the mediastinum.to A staging system was proposed by de Serres et al based on the location and extent of the lesions: stage  I is unilateral infrahyoid, stage II is unilateral suprahyoid, stage  III is unilateral infrahyoid and  suprahyoid,  stage  IV  is  bilateral infrahyoid, stage Vis bilateral infrahyoid and suprahyoid: generally, as the stage increases, the prognosis becomes poorer.ll

Good  evaluation   and  accmate   imaging is mandatory before any intervention to detetmine  the extension, size and to define if there is involvement  of any vital stmctmes. The US, CT and MRI ru·e all used to investigate cases with lymphatic rnalforrnation.I2

The  rnajmity  of  cases  ru·e  diagnosed  at birth (75 %,) and 90 %of the remaining cases were diagnosed by the age of two yeru·s.I3 In om  seties  3 cases  were diagnosed  at  bitth, and 75% of cases were diagnosed dming the first 7 yeru·s of life.

Most cases with lymphatic rnalfmmation presented  by  asymptomatic   mass  followed by aitway problem and dysphasia.I4 This is consistent with om seties as 75% of the cases presented as asymptomatic mass and 25% presented with huge mass that causes aitway distress.

Infection    and   hemorrhage    into   these cysts   are   quite   common   and   my   cause rapid enlargement with potential airway compromise.l4  In our series, only one case develops  infection  causing  airway  distress that needed urgent aspiration and surgical drainage. This case showed complete resolution.

Treatment   of   lymphatic    malformation is still challenging, observation, aspiration, laser, sclerotherapy  and surgery all are used solitary  or  in  combination  in treatment  of these cases.

Spontaneous regression may occur in 1.6-

16% of the cases.l5 Perkins et al described certain radiologic features that indicate a high likelihood of spontaneous regression. These features include LM with predominance of macrocystic tissues, less than 5 intra cystic septations,   and  limited  extent.l6   However in our series no case showes spontaneous regression and this agrees with others.l7

Simple   aspiration   is   rarely   used nowadays due to rapid recurrence, infection and hemorrhage.l5 However; needle decompression may be beneficial in some instances where there is rapid expansion, with airway distress.l8 In our-series aspiration was done in 4 cases presented at birth with huge LM causing airway troubles as a preliminary solution  before urgent surgery to relieve airway problems.

Surgical excision was regarded as the main stay of treatment.l9 Due to the infiltrative nature of the disease and close proximity to vital  structure,  complete  surgical  resection is usually challenging and leads to partial resection  in  60%  of  cases.20 Surgery  may be associated with significant morbidity including seroma, infection, nerve injuries especially the facial nerve. In our series 14 cases were treated surgically,

•      8  of  them  were  treated  electively; these patients have isolated neck LMS, with no respiratory distress or life threatening conditions. One of these cases developed marginal nerve injury; one needed another sitting to complete the resection, and 6 cases had excellent results.

•       5     cases     needed     urgent     surgical

exc1s1on to  alleviate  air  way  distress,  4 of these   cases  presented   at  birth  with  huge cystic hygroma compromising the airway, so, needle decompression were done followed by urgent surgical resection. One of these 4 cases needed a second stage to complete resection. The 5th case underwent urgent surgical drainage  due  to  development   of  infection with airway distress.

•       The     14th  case  underwent   surgical

excision due to extension of the malformation into the mediastinum that makes other modalities difficult to be used. The result was excellent.

Sclerotherapy  has  been  proposed  as  an

alternative to surgery in a trial to find a safe management modality without the hazardous complications of surgery. Several sclerosing agents were tried, including bleomycin, dextrose, ok- 432, Fibrin glue, ethiblock and Sterile ethanol (98%). ok- 432 is a lyophilized product of a mixed culture of group A streptococcus   pyogenis   and   penicillin   G. It is very  effective  as a sclerosing  agent  in LMs, however it is difficult to purchase in developing countries and contraindicated  in patients with penicillin allergy. 20  Bleomycin is  an  antibiotic  with  some  antitumoral activity; it has good results up to 88%21_ However  it  is  no  longer  in  use  due  to  its serious complications  as pulmonary fibrosis, other  agents  as  fibrin  glue  are  expensive, and others as ethiblock may cause allergic reaction.  We choose  98% ethanol  as it was reported  to be an effective sclerosing  agent with minimal  side effects, easy to purchase and low cost.

In our series  we used  98% ethanol  as a

sclerosing agent in 12 cases.

•      5  of  them   presented   with  isolated cervical  cystic hygroma  injected under local anesthesia.  Follow  up  showed  excellent result.

•       The  other  7  patients   presented   by

cervical  cystic  hygroma  and oropharyngeal extension  without  respiratory  distress,  these patients were injected by 98% ethanol under general  anesthesia  and the tube was left for a  variable  time  until  the  edema  subsided to  avoid  post  operative  airway  problems.

Follow up showed excellent results with  only one case who needed surgical intervention for the cervical part for cosmetic purposes.

Conclusion :

A  number of  treatment methods are available for  cervicofacial lymphatic malformations. Due  to  its infiltrative nature, complete  surgical  excision  is  challenging, so sclerotherapy gained popularity in the management of  LM  with  good  results. However, surgery  remains  the   first   option in (1) cases with huge LM that cause life threatening condition or  airway distress, (2) cases  with  rapid  enlargement or infection, (3) cases with residual disease after sclerotherapy and (4) cases  with  mediastinal extension.

An  algorithm of treatment of  LMs  of the head  and  neck  was illustrated in Figure (6).

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