History: "diagnosed with cystic hygroma at the age of 2 months after a knot came up on the left side of his neck... referred to a surgeon. Observation is suggested.  it went down significantly in a month..  At about 1 year, another mass came up in the same site. It was determined that it was a swollen lymph node and not a recurrence of the cystic hygroma. After an ear infection at the age of 25 months it was apparent again and the surgeon recommended surgical excision.  However, after discussing this with a pediatrician, a decision was made to hold off again and see if it went down again. A CT scan had been performed. Recently after an upper respiratory infection, it came back with a vengeance.  Overnight it got about the size of 2 golf balls.  He is almost 29 months old.  Another CT scan was performed and the mass has grown considerably. The radiologist wrote that it is associated with cervical lymph adenopathy greater on the left than right and it is extending medially displacing the airway to the right and showing some mild compressive effects upon the airway. Sclerotherapy with OK-432 is suggested. My questions:  
  • The fact that the lesion "involutes" to where it is unnoticeable and the bluish-bruised discoloration it has at times seem to correlate more with the hemangioma according to an article of yours that I read.  What is your opinion?
  • Also, if hemangiomas are hereditary, my husband had one around his eye that came up in infancy and was removed.  It was the red type.  That may be irrelevant to my son though. ?
  •   Because he is having some slight difficulty swallowing, the stress on us all, and the fact that I am getting neurotic about his breathing and avoiding an emergency tracheotomy we need help and advise soon.?
  • The lesion seems to move and change and is seeming to move from under the ear to the neck region this time.  I am afraid that it may be related to dental hygiene as he also has a supernumerary tooth and we may have not been diligent enough in oral hygiene.?  
  • Should an MRI be done? should we get to the nearest vascular malformation treatment center ASAP?"

Imaging Studies: CT performed. Not shown here. 


Diagnosis and Recommendation:  

Diagnosis: The history and photos are most suggestive of macrocystic lymphatic malformation or commonly called "cystic hygroma".  However, MRI is needed to confirm the diagnosis and also the assess the extent of the lesion. There may be minimal skin discoloration with these lesions (should not be confused with venous malformations). Also, some of these lesions are combined lesions, called "lymphatic Venous Malformation". 

  •  These lesions are benign conditions; however, there may be sudden bleeding into the   lesion, which may cause significant enlargement of the lesion rapidly. This typically happens in the lesions located in the orbit, behind the eyeball. In those cases, with sudden enlargement of the lesion, it is relatively common to see permanent optic nerve damage. In the case presented here, sudden airway compromise is a possibility if there is intralesional bleeding. This is also true for sclerotherapy, since sclerotherapy generally causes some enlargement of the lesion, particularly if absolute alcohol is used.  

Imaging: MRI is the gold standard modality in vascular anomalies, particularly for lymphatic malformations. MRI is much more reliable in diagnosis and more importantly to show the extend of the lesion. It is particularly important in this patient, since there is possible airway involvement. If sclerotherapy is performed without assessing the malformation in detail, it may cause significant swelling in the area and may cause obstruction of the airway partially or completely. Therefore, almost every lymphatic malformation patient, particularly if the lesion is located in the head and neck, needs MRI.  

Treatment: To my knowledge, currently OK432 is not available. One other issue with OK-432 is that it is difficult to find a physician (or hospital) who would be interested in using this agent since it is not FDA approved. This means that most hospitals do not allow their physicians use this agent considering the fact that there is too much risk. There are other sclerosant agents available, with similar success rates. One of these agents is Doxycycline. This agent is quite safe and long term possible complications are well established. For the patient demonstrated here, alternative to sclerotherapy would a surgical excision depending on the extend of the malformation in the neck.  




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