Cystic hygroma.pptx

Lecture notes for medical students

1. Tips on using my ppt. You can freely download, edit, modify and put your name etc. Don’t be concerned about number of slides. Half the slides are blanks except for the title. First show the blank slides (eg. Aetiology ) > Ask students what they already know about ethology of today's topic. > Then show next slide which enumerates aetiologies. At the end rerun the show – show blank> ask questions > show next slide. This will be an ACTIVE LEARNING SESSION x three revisions. Good for self study also. See notes for bibliography.

2. Lymphangioma & Cystic hygroma

3. Lymphangioma & Cystic hygroma Congenital malformation

4. Epidemiology

5. 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.

6. 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.

7. Pathophysiology

8. 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.

9. Presentation

10. 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.

11. 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.

12. 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

13. 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. 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.

15. 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). 

16. 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 .

17. 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.

18. Central in a discussion of CH is the understanding that it is synonymous with macrocystic lymphatic malformation and cystic lymphangioma . 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

19. Treatment

20. 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.

21. 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.

22. 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 .

23. 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).

24. 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.

25. 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.

26. 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.

27. 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.

28. Complications

29. 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.

30. Prognosis

31. 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.

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