Presented at AW Sjahranie General Hospital under supervision of dr. Rudy Thabry SpB(K)Onk

2. BACKGROUND
• FIRST DESCRIBED BY WERNHER IN 1843
• CYSTIC HYGROMA (CH) IS A CYSTIC LYMPHATIC LESION THAT CAN AFFECT ANY
ANATOMIC SUBSITE IN THE HUMAN BODY.
• CH USUALLY AFFECTS THE HEAD AND NECK (APPROXIMATELY 75%), WITH A LEFT-SIDED
PREDILECTION.
• WITHIN THE NECK, THE POSTERIOR TRIANGLE TENDS TO BE MOST FREQUENTLY
AFFECTED.
• APPROXIMATELY 20% OF CHS OCCUR IN THE AXILLA; MORE INFREQUENT SUBSITES
INCLUDE THE MEDIASTINUM, GROIN, AND RETROPERITONEUM.
• CH IS SYNONYMOUS WITH CYSTIC LYMPHANGIOMA, WHICH IS ALSO KNOWN AS A
MACROCYSTIC LYMPHATIC MALFORMATION AND WAS FIRST DESCRIBED IN 1828 BY

3. EPIDEMIOLOGY
• THE INCIDENCE OF CH IS ESTIMATED TO BE 1 CASE PER 6,000-16,000 LIVE
BIRTHS.
• MORTALITY  AS HIGH AS 2-6% IN SOME SERIES  SECONDARY
TO PNEUMONIA, BRONCHIECTASIS, AND AIRWAY COMPROMISE  IN THE
LARGER-SIZED LESIONS.
• MORBIDITY DEPENDS ON THE ANATOMIC LOCATION OF THE CH  RELATED TO
COSMETIC DISFIGUREMENT AND IMPINGEMENT ON OTHER CRITICAL
STRUCTURES SUCH AS NERVES, VESSELS, LYMPHATICS, AND THE AIRWAY.

4. CONT’D
• NO RACIAL PREDOMINANCE
• THE SEX DISTRIBUTION IS EQUAL.
• 50-65% ARE EVIDENT AT BIRTH, WITH 80-90% OF CHS PRESENTING BY AGE 2 YEARS.
• CH CAN BE VISUALIZED USING ABDOMINAL ULTRASONOGRAPHY BY 10 WEEKS
GESTATION
• FAST-SPIN MRI CAN ALSO BE USED TO DETERMINE THE EXTENT OF FETAL CH.
• ELEVATED ALPHA FETOPROTEIN LEVELS IN AMNIOCENTESIS FLUID HAS BEEN REPORTED
IN PREGNANCIES WITH CH.

6. CAUSES
• KARYOTYPIC ABNORMALITIES ARE PRESENT IN 25-70% OF CHILDREN WITH CH.
• CH HAS BEEN NOTED TO BE MORE COMMON IN PERSONS WITH TURNER
SYNDROME, DOWN SYNDROME,KLINEFELTER SYNDROME, AND TRISOMY 18 AND
13, ALTHOUGH THESE ARE NOT CONSIDERED A CAUSE.
• IN ADDITION, SEVERAL NONCHROMOSOMAL DISORDERS, INCLUDING NOONAN
SYNDROME, FRYNS SYNDROME, MULTIPLE PTERYGIUM
SYNDROME, AND ACHONDROPLASIA, ARE ASSOCIATED WITH AN INCREASED INCIDENCE
OF CH.
• INTRAUTERINE ALCOHOL EXPOSURE HAS BEEN ASSOCIATED WITH THE DEVELOPMENT
OF LYMPHANGIOMAS.
• DISSOLUTION OF BONE CAUSED BY EITHER LYMPHANGIOMAS OR HEMANGIOMAS IS

7. PATHOPHYSIOLOGY
• LYMPHANGIOMAS ARE THOUGHT TO ARISE FROM A COMBINATION OF THE FOLLOWING:
• A FAILURE OF LYMPHATICS TO CONNECT TO THE VENOUS SYSTEM,
• ABNORMAL BUDDING OF LYMPHATIC TISSUE,
• SEQUESTERED LYMPHATIC RESTS THAT RETAIN THEIR EMBRYONIC GROWTH POTENTIAL.
• THESE LYMPHATIC RESTS CAN PENETRATE ADJACENT STRUCTURES OR DISSECT ALONG
FASCIAL PLANES AND EVENTUALLY BECOME CANALIZED.
• THESE SPACES RETAIN THEIR SECRETIONS AND DEVELOP CYSTIC COMPONENTS
BECAUSE OF THE LACK OF A VENOUS OUTFLOW TRACT.
• THE NATURE OF THE SURROUNDING TISSUE DETERMINES WHETHER THE
LYMPHANGIOMA IS CAPILLARY, CAVERNOUS, OR CYSTIC.

8. CONT’D
• CHS TEND TO FORM IN LOOSE AREOLAR TISSUE, WHEREAS CAPILLARY AND CAVERNOUS
FORMS OF LYMPHANGIOMAS TEND TO FORM IN MUSCLE.
• STUDIES USING CELL PROLIFERATION MARKERS  LYMPHANGIOMA ENLARGEMENT IS
RELATED MORE TO ENGORGEMENT THAN TO ACTUAL CELL PROLIFERATION.
• MOLECULAR STUDIES  VASCULAR ENDOTHELIAL GROWTH FACTOR C (VEGF-C) AND ITS
RECEPTORS MAY PLAY AN IMPORTANT ROLE IN THE DEVELOPMENT OF LYMPHATIC
MALFORMATIONS .
• LYMPHANGIOMAS CAN BE ACQUIRED  TRAUMA (INCLUDING
SURGERY), INFLAMMATION, OR OBSTRUCTION OF A LYMPHATIC DRAINAGE PATHWAY.

9. PRESENTATION
• HISTORY
• SIGNS AND SYMPTOMS VARY DEPENDING ON THE LESION'S LOCATION.
• THE MICROCYSTIC FORM TENDS TO PREDOMINATE OVER CH IN THE ORAL CAVITY AND
OROPHARYNX.
• MICROCYSTIC LYMPHANGIOMAS COMMONLY APPEAR AS CLUSTERS OF CLEAR, BLACK, OR RED
VESICLES ON THE BUCCAL MUCOSA OR TONGUE.
• CHS TEND TO PREDOMINATE BELOW THE MYLOHYOID MUSCLE AND CAN INVOLVE BOTH THE
ANTERIOR AND POSTERIOR TRIANGLES OF THE NECK.
• TYPICALLY LARGE AND THICK WALLED CYSTS AND HAVE LITTLE INVOLVEMENT OF
SURROUNDING TISSUE.

10. CONT’D
• HISTORY:
• THE OVERLYING SKIN CAN TAKE ON A BLUISH HUE OR MAY APPEAR NORMAL.
• CHS OFTEN PRESENT AFTER A SUDDEN INCREASE IN SIZE SECONDARY TO INFECTION OR
INTRALESIONAL BLEEDING.
• SPONTANEOUS DECOMPRESSION OR SHRINKAGE IS UNCOMMON
• RARELY, CHILDREN WITH CH DISPLAY SYMPTOMS OF NEWLY ONSET OBSTRUCTIVE SLEEP
APNEA SYNDROME (OSAS).
• THIS SITUATION MAY INVOLVE CHILDREN WITH CH OR OTHER SPACE-OCCUPYING LESIONS OF
THE SUPRAGLOTTIS OR PARAGLOTTIC REGION.

11. CONT’D
• HISTORY
• SUPRAHYOID LYMPHANGIOMAS TEND TO CAUSE MORE BREATHING DIFFICULTIES
THAN INFRAHYOID LESIONS.
• POTENTIALLY LIFE-THREATENING AIRWAY COMPROMISE THAT MANIFESTS AS NOISY
BREATHING (STRIDOR) AND CYANOSIS IS A POSSIBLE SYMPTOM OF
LYMPHANGIOMAS.
• FEEDING DIFFICULTIES, AS WELL AS FAILURE TO THRIVE,  POTENTIAL
LYMPHANGIOMA.
• THIS IS ESPECIALLY TRUE WHEN THE LESION AFFECTS STRUCTURES OF THE
UPPER AERODIGESTIVE TRACT

12. CONT’D
• PHYSICAL FINDINGS MAY INCLUDE THE FOLLOWING:
• CHS ARE TYPICALLY SOFT, PAINLESS, COMPRESSIBLE (DOUGHY) MASSES.
• A CH TYPICALLY TRANSILLUMINATES.
• CLOSELY EVALUATE FOR TRACHEAL DEVIATION OR OTHER EVIDENCE OF IMPENDING AIRWAY
OBSTRUCTION.
• CLOSELY INSPECT THE TONGUE, ORAL CAVITY, HYPOPHARYNX, AND LARYNX BECAUSE ANY
INVOLVEMENT MAY LEAD TO AIRWAY OBSTRUCTION.

14. WORKUP
• LABORATORY STUDIES
• STUDIES HAVE SUGGESTED THAT FLUORESCENT IN SITU
HYBRIDIZATION (FISH) CAN BE USED TO EVALUATE FOR
CYSTIC HYGROMA (CH) IN PRENATAL CHROMOSOMAL
ANALYSIS. CHROMOSOMES 13, 18, 21, X, AND Y ARE
SPECIFICALLY MENTIONED

15. CONT’D
• IMAGING STUDIES
• MRI, CT SCANNING, AND ULTRASONOGRAPHY ARE ALL HELPFUL IN DELINEATING THE
NATURE OF A CYSTIC NECK MASS.
• CT SCANNING AND MRI REVEAL RING-LIKE MARGIN ENHANCEMENT WITH SHARP
DEMARCATION OF CYSTIC AREAS.
• THE CYSTIC AREAS TEND TO APPEAR CIRCUMSCRIBED AND DISCRETE.
• A POORLY DEFINED ISODENSE MASS THAT OBSCURES MUSCLE AND FATTY PLANES IS
MORE CONSISTENT WITH A MICROCYSTIC LYMPHATIC MALFORMATION THAN A CH.

16. CONT’D
• IMAGING STUDY
• MRI:
• MRI IS THE CONSENSUS STUDY OF CHOICE. IT PROVIDES THE BEST SOFT TISSUE DETAIL AND CAN
DELINEATE THE RELATIONSHIP OF THE LESION TO UNDERLYING STRUCTURES.
• CONTRAST CAN BE USED TO DIFFERENTIATE HEMANGIOMAS FROM LYMPHANGIOMAS. ON MRI, CHS APPEAR
HYPERINTENSE ON T2-WEIGHTED IMAGES AND HYPOINTENSE ON T1-WEIGHTED IMAGES.
• CT SCANNING:
• CT SCANNING IS FASTER AND MAY BE MORE READILY AVAILABLE THAN MRI.
• CT SCANNING CARRIES THE RISK OF RADIATION EXPOSURE, AND DETAIL IS LOST IF THE CH IS SURROUNDED
BY TISSUE OF SIMILAR ATTENUATION.
• CONTRAST HELPS TO ENHANCE CYST WALL VISUALIZATION AND THE RELATIONSHIP TO SURROUNDING
BLOOD VESSELS. ON CT SCANS, CHS APPEAR ISODENSE TO CEREBROSPINAL FLUID (CSF).

18. CONT’D
• IMAGING STUDY
• ULTRASONOGRAPHY: THIS IS THE LEAST INVASIVE STUDY.
• IT IS VERY USEFUL IN DEMONSTRATING THE RELATIONSHIP OF CH TO THE
SURROUNDING STRUCTURES.
• ULTRASONOGRAPHY HAS LIMITED ABILITY IN ASSESSING MEDIASTINAL AND
RETROPHARYNGEAL STRUCTURES.
• IT CAN BE USED TO DETECT CH IN UTERO.
• ECHOGRAPHIC VISUALIZATION OF MULTIPLE SEPTAE IN FETAL CH HAS BEEN
POSTULATED TO BE A POOR PROGNOSTIC INDICATOR.

19. CONT’D
• IMAGING STUDY
• PLAIN RADIOGRAPHY:
• WITH ANY LARGE MASS OF THE HEAD AND NECK, AIRWAY RADIOGRAPHY CAN BE
HELPFUL IN DELINEATING POSSIBLE AIRWAY COMPROMISE.
• PLAIN RADIOGRAPHY IS A REASONABLE INITIAL IMAGING MODALITY IN THE EVALUATION
OF A NECK MASS WITH A POTENTIAL AIRWAY MANIFESTATION.
• LYMPHOSCINTIGRAPHY: A CASE REPORT HIGHLIGHTED THE ABILITY TO
VISUALIZE CH USING LYMPHOSCINTIGRAPHY.

20. STAGING
• CLASSIFICATION HAS BEEN MARRED BY A HISTORICAL LACK OF CONFORMITY.
• IN 1877, THE FIRST SYSTEM WAS PROPOSED BY WEGENER.
• IN 1982, MULLIKEN AND GLOWACKI PRESENTED A CELL-BASED CLASSIFICATION THAT IS
CURRENTLY USED BY MANY AUTHORS.
• THEIR SYSTEM STRATIFIES LESIONS INTO HEMANGIOMAS OR VASCULAR
MALFORMATIONS.
• CHS FALL INTO THE LATTER CATEGORY. THE WORLD HEALTH ORGANIZATION (WHO)
RECOGNIZES 3 TYPES OF LYMPHANGIOMAS: CAPILLARY, CAVERNOUS, AND CYSTIC.

21. CONT’D
• CENTRAL IN A DISCUSSION OF CH IS THE UNDERSTANDING THAT IT IS
SYNONYMOUS WITH MACROCYSTIC LYMPHATIC MALFORMATION AND CYSTIC
LYMPHANGIOMA.
• GIGUERE ET AL HAVE PROPOSED CATEGORIZATION OF LYMPHANGIOMAS
BASED ON THE SIZE OF THE CYSTIC COMPONENT, AS FOLLOWS:
• MACROCYSTIC - CYSTIC SPACES AT LEAST 2 CM
• MICROCYSTIC - SPACES LESS THAN 2 CM
• MIXED LESIONS

22. CONT’D
• DE SERRES ET AL HAVE PROPOSED THE FOLLOWING SYSTEM FOR STAGING
OF CH OF THE HEAD AND NECK:
• STAGE I - UNILATERAL INFRAHYOID (17% COMPLICATION RATE)
• STAGE II - UNILATERAL SUPRAHYOID (41% COMPLICATION RATE)
• STAGE III - UNILATERAL AND BOTH INFRAHYOID AND SUPRAHYOID (67%
COMPLICATION RATE)
• STAGE IV - BILATERAL SUPRAHYOID (80% COMPLICATION RATE)
• STAGE V - BILATERAL INFRAHYOID AND SUPRAHYOID (100% COMPLICATION
RATE)

23. TREATMENT
• MEDICAL CARE
• ALTHOUGH SOME AUTHORS HAVE REPORTED WATCHFUL WAITING OF CYSTIC HYGROMA
(CH), IT SHOULD BE CONSIDERED ONLY IN PATIENTS WHO ARE ASYMPTOMATIC.
• THE MEDICAL TREATMENT OF CH CONSISTS OF THE ADMINISTRATION OF SCLEROSING
AGENTS.
• SCLEROSING AGENTS INCLUDE:
• OK-432 (AN INACTIVE STRAIN OF GROUP A STREPTOCOCCUS PYOGENES),
• BLEOMYCIN,
• PURE ETHANOL,
• SODIUM TETRADECYL SULFATE,
• DOXYCYCLINE.

24. CONT’D
• MEDICAL TREATMENT
• AN INFECTED CH SHOULD BE TREATED WITH INTRAVENOUS ANTIBIOTICS, AND
DEFINITIVE SURGERY SHOULD BE PERFORMED ONCE THE INFECTION HAS
RESOLVED.
• INCISION AND DRAINAGE OR ASPIRATION RESULTS IN ONLY TEMPORARY
SHRINKAGE, AND SUBSEQUENT FIBROSIS CAN FURTHER COMPLICATE THE
RESECTION.
• RADIOTHERAPY HAS NOT BEEN DEMONSTRATED TO BE EFFECTIVE.
• THE PREFERRED TREATMENT OF ALL CH IS SURGICAL RESECTION. ONLY
RESECTION CAN TRULY OFFER THE POTENTIAL FOR CURE.

25. CONT’D
• SURGICAL CARE
• THE MAINSTAY OF TREATMENT IS SURGICAL EXCISION
• BOTH THE OPERATING TEAM AND THE FAMILY OF THE PATIENT SHOULD GO FORWARD WITH
THE KNOWLEDGE THAT CH IS A BENIGN LESION.
• IF ACUTE INFECTION OCCURS PRIOR TO RESECTION, SURGERY SHOULD BE DELAYED AT LEAST
3 MONTHS.
• THE SURGICAL TEAM SHOULD ATTEMPT TO COMPLETELY REMOVE THE LYMPHANGIOMA OR TO
REMOVE AS MUCH AS POSSIBLE, SPARING ALL VITAL NEUROVASCULAR STRUCTURES.
• COMPLETE EXCISION HAS BEEN ESTIMATED TO BE POSSIBLE IN ROUGHLY 40% OF CASES.
• CHS ARE IDEALLY REMOVED IN ONE PROCEDURE BECAUSE SECONDARY EXCISIONS ARE
COMPLICATED BY FIBROSIS AND DISTORTED ANATOMICAL LANDMARKS.

28. CONT’D
• MICROCYSTIC LESIONS ARE MUCH MORE DIFFICULT TO REMOVE BECAUSE OF THEIR
INTIMATE ASSOCIATION WITH NEARBY TISSUES.
• LASER THERAPY IS A RECENT ADVANCEMENT IN THE TREATMENT OF MICROCYSTIC
LESIONS.
• THE EXCEPTIONS TO EXCISION AT THE TIME OF DIAGNOSIS ARE:
• PREMATURE INFANTS
• SMALL IN SIZE
• INVOLVEMENT OF CRUCIAL NEUROVASCULAR STRUCTURES THAT ARE SMALL AND DIFFICULT
TO IDENTIFY (EG, FACIAL NERVE).

29. CONT’D
• IF NO AIRWAY OBSTRUCTION IS PRESENT, SURGERY CAN BE DELAYED UNTIL
THE CHILD IS AGED 2 YEARS OR OLDER, ESPECIALLY WHEN OPERATING
AROUND THE FACIAL NERVE IN THE PAROTID AREA.
• SIGNS OF AIRWAY OBSTRUCTION REQUIRE SURGICAL EVALUATION AT THE
TIME OF DIAGNOSIS.
• IN EMERGENCY SITUATIONS, ASPIRATION WITH AN 18-GAUGE OR 20-GAUGE
NEEDLE MAY OBVIATE THE NEED FOR AN EMERGENCY TRACHEOSTOMY.

30. CONT’D
• ALTHOUGH TRADITIONAL WISDOM HAS DICTATED NOT ASPIRATING LYMPHATIC
MALFORMATIONS, A STUDY BY BUREZQ ET AL DOCUMENTED SUCCESS WITH SERIAL
ASPIRATION OF CH.
• IN THEIR SERIES, 14 PATIENTS WERE TREATED WITH ASPIRATION ALONE (3 NEEDED
MULTIPLE ASPIRATIONS), WITH A MEAN FOLLOW-UP OF 5.75 YEARS.
• NO FAILURES WERE REPORTED. THIS TECHNIQUE MAY HOLD PROMISE FOR THE FUTURE
MANAGEMENT OF CH.
• OTHER AUTHORS CONTEND ASPIRATION HAS NO ROLE AND BELIEVE THAT ASPIRATION
IS OFTEN FOLLOWED BY RECURRENCE, HEMORRHAGE, OR INFECTION.
• RADIOFREQUENCY ABLATION HAS BEEN ADVOCATED FOR USE WITH INTRAORAL
LYMPHATIC MALFORMATIONS, ESPECIALLY MICROCYSTIC LESIONS.

31. CONT’D
• MAGNETIC RESONANCE–CONTROLLED LASER-INDUCED INTERSTITIAL
THERMOTHERAPY IS A NOVEL THERAPY THAT HAS BEEN PROPOSED FOR
TREATMENT OF LYMPHANGIOMAS.
• CH CAN PRESENT ON ROUTINE PRENATAL ULTRASONOGRAPHY AS A LARGE
OBSTRUCTING AIRWAY MASS, AS CAN OTHER PATHOLOGIC CONDITIONS SUCH
AS A TERATOMA OR RHABDOMYOSARCOMA.
• IF SUCH A MASS IS VISIBLE ON ULTRASONOGRAPHY, MRI SHOULD BE
PERFORMED TO FURTHER DELINEATE THE MASS.

32. CONT’D
• IN THESE CASES, A MULTISPECIALTY TEAM INCLUDING A HIGH-RISK
OBSTETRICIAN, PEDIATRIC OTOLARYNGOLOGIST, PEDIATRIC SURGEON, AND
NEONATOLOGIST SHOULD BE PRESENT AT THE EX UTERO INTRAPARTUM
TREATMENT (EXIT) PROCEDURE.
• A PLANNED CESAREAN DELIVERY IS PERFORMED, AND INTUBATION OR
TRACHEOSTOMY IS USED TO ESTABLISH AN AIRWAY.
• EXTRACORPORAL MEMBRANE OXYGENATION (ECMO) SHOULD ALSO BE
AVAILABLE. EXCISION OF THE CH IS DELAYED UNTIL THE CHILD IS STABLE.

33. COMPLICATIONS
• COMPLICATIONS INCLUDE AIRWAY OBSTRUCTION, HEMORRHAGE, INFECTION, AND
DEFORMATION OF SURROUNDING BONY STRUCTURES OR TEETH IF LEFT UNTREATED.
• COMPLICATIONS FROM THE SURGICAL EXCISION OF A CYSTIC HYGROMA (CH) ARE
MYRIAD AND ARE RELATED TO THE LOCATION AND STRUCTURES ADJACENT TO THE
MASS:
• DAMAGE TO A NEUROVASCULAR STRUCTURE (INCLUDING CRANIAL NERVES),
• CHYLOUS FISTULA,
• CHYLOTHORAX,
• HEMORRHAGE,
• RECURRENCE.
• MOST RECURRENCES OCCUR WITHIN THE FIRST YEAR BUT HAVE BEEN REPORTED TO
OCCUR AS LONG AS 10 YEARS AFTER EXCISION.

34. PROGNOSIS
• UNLIKE IN HEMANGIOMAS, SPONTANEOUS RESOLUTION OF CH IS UNCOMMON.
• RECURRENCE IS RARE WHEN ALL GROSS DISEASE IS REMOVED.
• IF RESIDUAL TISSUE IS LEFT BEHIND, THE EXPECTED RECURRENCE RATE IS
APPROXIMATELY 15%.
• IN PRENATAL CH, DIAGNOSIS AFTER 30 WEEKS' GESTATION IS CONSIDERED A
POSITIVE PROGNOSTICATOR.

35. THANK YOU