I. Pathophysiology

  1. Congenital lymphatic malformation
    1. Lymphatic vessel fails to connect and drain
    2. Results in large multiloculated cyst
  2. Distribution
    1. Neck (60%)
    2. Other locations
      1. Chest Wall
      2. Mediastinum
      3. Axilla
    3. Rare locations
      1. Inguinal region
      2. Retroperitoneal region

II. Associated Conditions

  1. Turner's Syndrome
  2. Noonan's Syndrome
  3. Down Syndrome

III. Symptoms

  1. Initial
    1. Asymptomatic
    2. Painless lesion unless infected or bleeding
  2. Progression: Continues to increases in size
    1. 90% symptomatic by age 2 years

IV. Signs

  1. Fluctuant mass usually in neck
  2. Mass transilluminates

V. Complications

  1. Infection
  2. Bleeding
  3. Airway compromise (due to compression)

VI. Differential Diagnosis

VII. Management

  1. Deliver where NICU available if diagnosed prenatally
    1. Risk of airway compromise
  2. Surgical excision

VIII. Prognosis

  1. Recurrence rate after surgical excision: 10-15%

IX. References

Images: Related links to external sites (from Google)

Ontology: Cavernous lymphangioma (C0205828)

Definition (NCI) A lymphangioma characterized by the presence of thin-walled cavernous lymphatic spaces.
Concepts Neoplastic Process (T191)
MSH D008202
ICD10 M9172/0
SnomedCT 89056007
English Cavernous Lymphangiomas, Lymphangiomas, Cavernous, LYMPHANGIOMA, CAVERNOUS, Cavernous Lymphangioma, Cavernous lymphangioma, Cavernous lymphangioma (morphologic abnormality), cavernous; lymphangioma, lymphangioma; cavernous, Lymphangioma, Cavernous
Portuguese Linfangioma Cavernoso
Spanish Linfangioma Cavernoso, linfangioma cavernoso (anomalía morfológica), linfangioma cavernoso
French Lymphangiome caverneux
German Lymphangiom, kavernöses
Dutch caverneus; lymfangioom, lymfangioom; caverneus, Lymfangioom, caverneus

Ontology: Lymphangioma, Cystic (C0206620)

Definition (MSH) A cystic growth originating from lymphatic tissue. It is usually found in the neck, axilla, or groin.
Definition (NCI) A benign lymphatic neoplasm usually arising from the neck and characterized by cystic dilation of the lymphatic vessels.
Concepts Neoplastic Process (T191)
MSH D018191
ICD10 M9173/0
SnomedCT 423984004, 40225001, 399882002
English Hygroma cystic, Hygroma, Hygroma, Cystic, Lymphangioma, Cystic, Cystic Hygromas, Cystic Lymphangiomas, Hygromas, Hygromas, Cystic, Lymphangiomas, Cystic, Hygroma, NOS, Cystic Hygroma, Cystic Lymphangioma, Non-neoplastic hygroma (disorder), Non-neoplastic hygroma, HYGROMA CYSTIC, Colli, Cystic Hygroma, Hygroma Colli, Cystic, Cystic Hygroma Colli, CYSTIC HYGROMA, Lymphangioma, Cystic [Disease/Finding], cystic hygroma, cystic lymphangiomas, hygromas, cystic hygromas, Cystic lymphangioma, Cystic hygroma, Cystic hygroma (disorder), Cystic lymphangioma (morphologic abnormality), cystic; lymphangioma, hygroma, lymphangioma; cystic, Lymphangioma;cystic, cystic lymphangioma
Dutch cystisch hygroom, hygroom cystisch, cysteus; lymfangioom, lymfangioom; cysteus, cystisch lymfangioom, Cystisch lymfangioom, Hygroom, Hygroom, cystisch, Lymfangioom, cystisch
German zystisches Hygrom, Hygrom, zystisch, HYGROMA CYSTICUM, zystisches Lymphangiom, Hygrom, zystisches, Hygrom, Lymphangiom, zystisches
Portuguese Higroma quístico, HIGROMA QUISTICO, Linfangioma cístico, Higroma Cístico, Higroma, Linfangioma Cístico
Spanish Higroma quístico, higroma no neoplásico, higroma no neoplásico (trastorno), higroma quístico (trastorno), higroma quístico, higroma, linfangioma quístico (anomalía morfológica), linfangioma quístico, Linfangioma cístico, Higroma Quístico, Higroma, Linfangioma Quístico, Higroma Quistico, Linfangioma Quistico
Japanese 水滑性嚢腫, ノウホウセイリンパカンシュ, ノウホウセイヒグローマ, スイカツセイノウシュ, リンパ管腫-嚢胞性, 嚢腫性リンパ管腫, のう胞性リンパ管腫, ヒグローマ, 嚢胞性ヒグローマ, 嚢胞性リンパ管腫, ヒグローマ-嚢胞性
Swedish Lymfangiom, cystiskt
Czech lymfangiom cystický, Cystický lymfangiom, Hygroma cysticum, Cystický hygrom
Finnish Imusuonirakko
Russian GIGROMA, LIMFANGIOMA KISTOZNAIA, GIGROMA KISTOZNAIA, ГИГРОМА, ГИГРОМА КИСТОЗНАЯ, ЛИМФАНГИОМА КИСТОЗНАЯ
French HYGROMA KYSTIQUE, Hygrome kystique, Lymphangiome kystique, Hygroma kystique
Italian Igroma cistico, Igroma, Linfangioma cistico
Polish Wodniak torbielowaty, Hygroma, Naczyniak limfatyczny torbielowaty
Hungarian Hygroma cystikus, Cystikus lymphangioma, Cystikus hygroma