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PubMed This is a summary of 13 peer-reviewed journal articles Updated
Otolaryngology · Lymphatic Malformation

Location, Staging, and Airway Risk

At a Glance

Head and neck lymphatic malformations (LMs) are classified using the de Serres staging system based on their location. Parents must closely monitor children with these LMs for airway risks like stridor or feeding issues, as these masses can swell rapidly during minor illnesses.

While lymphatic malformations can occur anywhere in the body—such as the chest, arms, or legs, where they are typically evaluated for how they affect pain or physical mobility—those located in the head and neck require special attention [1][2].

When an LM occurs in the head or neck, its specific location is often more important than its overall size [3]. Because the neck contains the “high-rent district” of the body—including the airway, major blood vessels, and nerves for swallowing—doctors use specialized staging to understand the risk and plan treatment [4][5].

The de Serres Staging System

To help families and doctors speak the same language, specialists use the de Serres staging system specifically for head and neck LMs [6]. This system is based on where the malformation sits in relation to the hyoid bone (a small bone in the upper neck) [7].

  • Stage I: Unilateral (one side) and suprahyoid (above the hyoid bone, near the jaw/mouth) [7].
  • Stage II: Unilateral (one side) and infrahyoid (below the hyoid bone, in the lower neck) [7].
  • Stage III: Unilateral (one side) and both above and below the hyoid bone [7].
  • Stage IV: Bilateral (both sides) and suprahyoid [7].
  • Stage V: Bilateral (both sides) and both above and below the hyoid bone [7].

Stages IV and V are considered the most complex because they involve both sides of the neck, which increases the likelihood of the malformation wrapping around or pressing on the airway [8][9].

Function-Threatening vs. Non-Function-Threatening

Doctors classify LMs based on whether they interfere with a child’s essential body functions:

  1. Function-Threatening: These LMs interfere with vital tasks. The most critical “function” is breathing [4]. If a malformation narrows the airway, it is considered life-threatening and requires urgent management [10][11]. Other function-threatening LMs may press on the eyes (threatening vision) or the throat (threatening the ability to swallow) [4][12].
  2. Non-Function-Threatening: These may cause swelling or change a child’s appearance, but they do not press on vital structures or interfere with daily activities like breathing or eating [4].

Monitoring the Airway

The most important thing for parents to watch for is airway compromise. Because LMs are “fluid-filled,” they can swell rapidly during a common cold or a bout of the flu [13].

Signs that an LM may be affecting the airway include:

  • Stridor: A high-pitched, “squeaky” noise when the child breathes in [10].
  • Retractions: Seeing the skin pull in sharply around the ribs or neck when the child takes a breath.
  • Feeding Difficulties: Frequent coughing, gagging, or choking while eating, which may suggest the malformation is pressing on the esophagus [5].

If your child has a head or neck LM, your care team will likely include a pediatric ENT (Ear, Nose, and Throat) specialist to ensure the airway remains safe as the child grows [4].

Proceed to Getting the Right Diagnosis.

Common questions in this guide

What is the de Serres staging system for lymphatic malformations?
The de Serres staging system classifies head and neck lymphatic malformations based on their location relative to the hyoid bone. It ranges from Stage I to Stage V, helping doctors understand the complexity and risks to vital structures like the airway.
What are the signs that a lymphatic malformation is affecting a child's airway?
Key signs of airway compromise include stridor (a high-pitched, squeaky breathing sound), retractions (skin pulling in around the ribs or neck when breathing), and feeding difficulties such as frequent choking or gagging.
Why do head and neck lymphatic malformations require special attention?
Lymphatic malformations in the head and neck are critical because they are located near the airway, major blood vessels, and nerves used for swallowing. Even a small malformation in this area can become life-threatening if it presses on the airway.
Can a cold or flu affect my child's lymphatic malformation?
Yes, because lymphatic malformations are fluid-filled, they can swell rapidly during a common cold or the flu. Parents should have an emergency plan in place in case this sudden swelling begins to affect their child's breathing.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my child's imaging, what is their de Serres stage (I-V)?
  2. 2.Is the malformation pressing on the trachea (windpipe) or the esophagus (food pipe)?
  3. 3.What specific signs of respiratory distress (like stridor or retractions) should I look for at home?
  4. 4.If my child gets a cold or fever, how quickly can the malformation swell, and what is our emergency plan?
  5. 5.Does the location of this malformation put my child's vision or speech development at risk?

Questions For You

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References

References (13)
  1. 1

    Laparoscopic-assisted resection with adjunct sclerotherapy for abdominal lymphatic malformations: Long-term outcomes from a large cohort study.

    Tran TD, Pham HD, Vu HM, et al.

    Journal of pediatric surgery 2025; (61(1)):162739 doi:10.1016/j.jpedsurg.2025.162739.

    PMID: 41106469
  2. 2

    Coexistence of Acute Appendicitis and Mesenteric Cystic Lymphatic Malformation in an Adult: A Case Report and Narrative Review of Intraoperative Management Strategies.

    Barbu LA, Cercelaru L, Vîlcea ID, et al.

    Life (Basel, Switzerland) 2025; (15(9)) doi:10.3390/life15091390.

    PMID: 41010332
  3. 3

    Lymphatic malformations.

    Kulungowski AM, Patel M

    Seminars in pediatric surgery 2020; (29(5)):150971 doi:10.1016/j.sempedsurg.2020.150971.

    PMID: 33069296
  4. 4

    Lymphatic and Mixed Malformations.

    Hathaway BA, Radu S, Wilson J, Nauta AC

    Lymphatic research and biology 2021; (19(1)):41-50 doi:10.1089/lrb.2020.0100.

    PMID: 33493408
  5. 5

    Lymphatic malformations: a 20-year single institution experience.

    Kronfli AP, McLaughlin CJ, Moroco AE, Grant CN

    Pediatric surgery international 2021; (37(6)):783-790 doi:10.1007/s00383-021-04859-5.

    PMID: 33586010
  6. 6

    Active Observation as an Alternative to Invasive Treatments for Pediatric Head and Neck Lymphatic Malformations.

    Bonilla-Velez J, Whitlock KB, Ganti S, et al.

    The Laryngoscope 2021; (131(6)):1392-1397 doi:10.1002/lary.29180.

    PMID: 33107991
  7. 7

    Large Cystic Lymphangiomas of the Neck: A Surgical Challenge.

    Subramaniyan P, Mohiyuddin SMA, Jyothi DN, et al.

    Cureus 2025; (17(6)):e85542 doi:10.7759/cureus.85542.

    PMID: 40630345
  8. 8

    Thirty-day perioperative outcomes in resection of cervical lymphatic malformations.

    Khanwalkar A, Carter J, Bhushan B, et al.

    International journal of pediatric otorhinolaryngology 2018; (106()):31-34 doi:10.1016/j.ijporl.2017.12.034.

    PMID: 29447887
  9. 9

    Indications for tracheostomy in children with head and neck lymphatic malformation: analysis of a nationwide survey in Japan.

    Ueno S, Fujino A, Morikawa Y, et al.

    Surgery today 2019; (49(5)):410-419 doi:10.1007/s00595-018-1755-3.

    PMID: 30778735
  10. 10

    Anesthetic Management of a Massive Cystic Hygroma of the Neck in a Neonate.

    Puri S, Jafra A, Dogra N, et al.

    Journal of Indian Association of Pediatric Surgeons 2024; (29(2)):183-185 doi:10.4103/jiaps.jiaps_197_23.

    PMID: 38616834
  11. 11

    Airway obstruction caused by rapid enlargement of cervical lymphangioma in a five-month-old boy.

    Shimizu J, Taga T, Kishimoto T, et al.

    Clinical case reports 2016; (4(9)):896-8 doi:10.1002/ccr3.659.

    PMID: 27648270
  12. 12

    A toddler with acute, life-threatening airway obstruction due to a retropharyngeal macrocytic lymphatic malformation: successful emergency sclerotherapy.

    Cherkes M, Pondorfer-Schaefer P, Pock J, et al.

    International journal of pediatric otorhinolaryngology 2025; (195()):112433 doi:10.1016/j.ijporl.2025.112433.

    PMID: 40580782
  13. 13

    Rapid lymphatic malformation expansion: Imaging, association with asymptomatic Streptococcus infection, multidisciplinary management, and therapeutic approach.

    Healy J, Balcazar JA, Rivera L, Ruas V

    Radiology case reports 2023; (18(9)):3162-3168 doi:10.1016/j.radcr.2023.06.029.

    PMID: 37404220

This information about lymphatic malformation staging and airway risks is for educational purposes only. Always consult your pediatric ENT or care team immediately if you notice changes in your child's breathing.

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