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Cystic Hygroma 

Related Terms: Lymphatic dysplasia, cystic swelling, hydroma, cervical hygroma, hygroma colli cysticum, hygroma of the neck, hygroma axillare, hygroma of the axilla, lymphedema, encephaloceles, meningomyelocele, lymphangioma

Classification:  

Lymphatic Malformation, associated with chromosomal anomalies such as monosomy x (50%), trisomy 21(10%-15%) and noonan syndrome. Incidence of occurrence is approximately 1 in 6000 pregnancies.

Cystic hygromas are now more commonly referred to as lymphangiomas or lymphatic malformations. Cystic Hygromas are common with lymphedema and lymphatic disorders.

Description:  

Soft  lymph filled masses within a thin-walled "sac"  generally appearing on the neck (75%), axilla (20%), or trunk or extremity (5%). They usually present a swollen bulges underneath the skin.  While they generally present at birth, children up to three years old may experience them. These masses are  always non-malignant and may present singularly or in multiples. Depending on the size of the cyst, the "bulge" can either appear with normal skin coloration or can appear with bruise discoloration or of redness.

They are different and non-related to three other forms of "sac-like" growths or growths associated with the head and fetal development.  Encephaloceles are are rare neural tube defects characterized by sac-like protrusions of the brain and the membranes that cover it through openings in the skull. These defects are caused by failure of the neural tube to close completely during fetal development.(1), meningomyelocele Protrusion of the membranes that cover the spine but some of the spinal cord itself through a defect in the bony encasement of the vertebral column. The bony defect is spina bifida(2), teratoma is most common primary brain tumor seen in newborn children. It is more common in males. This is a form of germ cell tumor. The teratoma accounts for about 20% of germ cell tumors.(3)

Etiology: 

Obstruction of the jugular region lymph vessels, which prevent  venous drainage resulting in dilated disorganized lymph channels. The membrane of the cyst can vary in thickness, can be fibromuscular (relating to both fibrous and muscular tissues), and can contain mural thrombi (relating to clots).  The fluid contained in it is generally proteinaceous (relating to or the nature of protein).

Complications: 

Possible airway obstruction, bleeding, infections, edema of the affected areas.

Diagnosis: 

Confirmed through use of  x-ray, ultra-sound, MRI, CT scan

Treatment: 

Surgical removal of abnormal masses.  However, if there has been infiltration of surrounding tissues treatments may include  sclerosings agents, steroids, chemotherapy or even radiation. Another form is treatment is called sclerotherapy.  Sclerotherapy is an injection of a sclerosing (e.g., alcohol) substance directly through the skin into a lesion and is used primarily for slow-flow vascular anomalies, particularly for venous malformation (cavernous hemangioma) and lymphatic malformation (cystic hygroma).  This procedure is performed by interventional radiologists who have experience with vascular anomalies. Various needles are used under ultrasound guidance to obtain access and then the sclerosant agent is injected into the lesion very carefully.  

Prognosis: 

Excellent, although approximately 10% of cystic hygromas may reoccur.

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Cystic hygroma

Definition 

A cystic hygroma is a thin-walled, sac-like structure filled with lymph. It occurs most commonly in the head and neck area and often appears as a soft bulge under the skin.

Causes, incidence, and risk factors 

This condition is a congenital (present before birth) abnormality caused by embryonic lymphatic tissues. Cystic hygromas are tumors derived from these tissues.

Symptoms   

A common symptom is a neck mass noted at birth, or discovered later in an infant after an upper respiratory tract infection.

Signs and tests 

Treatment

Treatment involves complete removal of the abnormal tissue whenever possible. However, cystic hygromas can often invade other neck structures, making this impossible. In these cases, other treatments have been attempted with only limited success. These include injection of sclerosing agents, steroids, chemotherapy agents, and radiation therapy.

Expectations (prognosis)

The outlook is good if surgery can totally remove the abnormal tissue. In cases where complete removal is not possible because of involvement of neighboring nerves or blood vessels, recurrence is common.

Complications 

Bleeding, infection, recurrence of the cystic hygroma, and damage to structures in the neck caused by surgery.

Calling your health care provider

Call your physician regarding any neck mass found in a child or adult.

Update Date: 11/19/2002

Medline Plus Medical Encylopedia

http://www.nlm.nih.gov/medlineplus/ency/article/000148.htm

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Mesenteric cystic lymphangioma associated with intestinal malrotation in a newborn. 

May 2011


Antoniou D, Soutis M, Loukas I, Christopoulos-Geroulanos G.

Source

Department of Pediatric Surgery, Aghia Sophia Children's Hospital, Thivon & Papadiamantopoulou Street, 11527 Athens, Greece. dimitri.antoniou@hotmail.com

Abstract

Mesenteric cystic lymphangiomas are uncommon benign cystic tumors rarely associated with other congenital malformations. We herein describe a case of a 9-day-old female presented with a two-day history of bilious vomiting, feeding intolerance, weakness and lethargy. Preoperative studies were diagnostic of intestinal malrotation. At laparotomy a cysticmesenteric mass was identified. Histopathological examination confirmed the diagnosis of mesenteric cystic lymphangioma. The exact aetiology of mesenteric lymphangioma is unknown and this case supports the theory that lymphangioma is a primary congenital malformation.

PubMed

http://www.ncbi.nlm.nih.gov/pubmed/22145299

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Giant cystic lymphangioma of the small bowel mesentery: case report. 

2011

Khattala K, Rami M, Elmadi A, Mahmoudi A, Bouabdallah Y.

Source

Department of Pediatric Surgery, University of Hospital, Fez, Morocco.

Keywords: Cystic lymphangioma, Pancreas, Retroperitoneal space


Abstract

Cystic lymphangioma is an uncommon mesenteric tumor which is usually reported in children. We describe a case of a 12-years old girl who was admitted in our hospital with abdominal distension and pain. Physical examination showed an abdomino-pelvic mass. Preoperative studies including abdominal ultrasonography and computed tomography failed to determine the cause of the pain. Laparotomy found a giant cystic tumor of the small bowel mesentery. Histological studies confirm the diagnosis of cystic lymphangioma. Based on this case, a review of the literature is suggested.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3204568/?tool=pubmed

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Research Articles and Abstracts:

Cystic hygroma and mid-trimester maternal serum screening.

http://jms.rsmjournals.com/content/14/3/109.long

Prenatal diagnosis of alobar holoprosencephaly with cystic hygroma.

http://www.sciencedirect.com/science/article/pii/S1028455909602138

Characteristics and outcome of fetal cystic hygroma diagnosed in the first trimester

InformaWorld/PubMed

Rapid development of an axillary mass in an adult: a case of cystic hygroma.

Lippincott, Williams & Wilkins

Cystic hygroma in a tactical aviator: a case report.

PubMed

Cystic hygroma of the breast: a rare lesion.

PubMed

Cystic hygroma of the shoulder region. A case report.

Lippincott, Williams & Wilkins/PubMed

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External Links:

Cystic lymphangioma of the pancreas mimicking pancreatic pseudocyst. Jun 2011

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3205361/?tool=pubmed

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Unilocular cystic lymphangioma of thigh-an extremely rare clinical entity. Oct 2010

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3077145/?tool=pubmed

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Cystic Hygroma Support Group

http://www.cafamily.org.uk/Direct/c95.html

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Cystic Hygroma

eMedicine

Author: Holly Neville, MD, Fellow, Department of Pediatric Surgery, University of Texas Southwestern Medical Center at Dallas

http://www.emedicine.com/PED/topic536.htm

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CysticHygroma.co.UK

http://www.cystichygroma.co.uk/

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Cystic Hygroma

AllRef Health.com

http://healthallrefer.com/health/cystic-hygroma-info.html

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Cystic Hygroma

http://www.genetics.emory.edu/pdf/Emory_Human_Genetics_Cystic_Hygroma.PDF

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National Institute of Neurological Disorders and Stroke(1)

http://www.ninds.nih.gov/health_and_medical/disorders/encephaloceles.htm

Meningomyelocele(2)

http://www.medterms.com/script/main/art.asp?articlekey=4350

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Lymphedema People Related Pages:

Lymphangiomas

http://www.lymphedemapeople.com/thesite/lymphedema_lymphangiomas.htm

Lymphatic Malformations

http://www.lymphedemapeople.com/thesite/lymphedema_lymphatic_malformations.htm

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Diagnostic Images:

Yahoo

http://images.search.yahoo.com/search/images;_ylt=A0geu8pxZQNIuloAeNxXNyoA?ei=UTF-8&p=cystic%20hygroma&fr2=tab-web&fr=yfp-t-501

Google

http://images.google.com/images?hl=en&q=Cystic+Hygroma&um=1&ie=UTF-8&sa=N&tab=wi

Otolaryngology Houston

http://www.ghorayeb.com/LymphangiomaNeck.html

DermIS

http://www.dermis.net/dermisroot/en/23135/diagnose.htm

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Diagnostic Codes and External Resources:

ICD-10

D18.140:
Cystic hygroma
D18.130:
Lymphangioma circumscriptum
D18.130:
Lymphangioma circumscriptum cutis
D18.140:
Lymphangioma, cavernous
D18.110:
Lymphangioma, simple

ICD-9

2008 ICD-9-CM Diagnosis 228.1

Lymphangioma any site

  • 228.1 is a specific code that can be used to specify a diagnosis
  • 228.1 contains 11 index entries
  • View the ICD-9-CM Volume 1 228.* hierarchy

228.1 also known as:

  • Congenital lymphangioma
  • Lymphatic nevus
DiseasesDB 7665
MedlinePlus 000148
eMedicine derm/866 
MeSH D008202

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