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Cystic hygroma

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

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Cystic hygroma

  1. 1. 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
  2. 2. 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.
  3. 3. 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.
  4. 4. 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
  5. 5. 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.
  6. 6. 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.
  7. 7. 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.
  8. 8. 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.
  9. 9. 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
  10. 10. 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.
  11. 11. 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
  12. 12. 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.
  13. 13. 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).
  14. 14. 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.
  15. 15. 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.
  16. 16. 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.
  17. 17. 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
  18. 18. 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)
  19. 19. 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.
  20. 20. 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.
  21. 21. 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.
  22. 22. 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).
  23. 23. 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.
  24. 24. 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.
  25. 25. 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.
  26. 26. 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.
  27. 27. 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.
  28. 28. 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.
  29. 29. THANK YOU
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Presented at AW Sjahranie General Hospital under supervision of dr. Rudy Thabry SpB(K)Onk

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