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Year : 2005  |  Volume : 15  |  Issue : 4  |  Page : 517-519
A case of axillary cystic hygroma


SSG Hospital, Medical College, Baroda, Vadodara, Gujarat - 390001, India

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   Abstract 

Lymphangiomas are benign congenital abnormalities of the lymphatic system. They are thought to arise from sequestrations of embryonic lymph sacs; these sacs are found in the neck and retroperitoneum. 75% of lymphangiomas occur in the neck. The abnormal ducts vary in size from microscopic to several centimeters and there is a variable component of fibrous adventitia. The large cystic lesions are also known as cystic hygromas. These lesions are commonly discovered as painless masses in otherwise healthy children under the age of two2.

Keywords: Cystic lymphangioma

How to cite this article:
Mansingani S, Desai N, Pancholi A, Parajapati A, Vohra P A, Raniga S. A case of axillary cystic hygroma. Indian J Radiol Imaging 2005;15:517-9

How to cite this URL:
Mansingani S, Desai N, Pancholi A, Parajapati A, Vohra P A, Raniga S. A case of axillary cystic hygroma. Indian J Radiol Imaging [serial online] 2005 [cited 2020 Oct 30];15:517-9. Available from: https://www.ijri.org/text.asp?2005/15/4/517/28786

   Introduction Top


Hygromas, from the Greek meaning, "moist or watery tumor", are the manifestation of the lymphatic system that occur for the most part in the cervico-thoracic region of the infant and children1. Our patient represents an unusual manifestation of an axillary cystic hygroma as is true with most of the cases; treatment was satisfactorily accomplished with surgical excision.


   Case report Top


A 5 year old girl was referred for the evaluation of aright axillary mass, noticed by her parents since birth gradually increasing in size. The examination revealed an afebrile child with normal vital signs. She was alert and non-toxic. A tense cystic of 10x10 cm sized mass was observed and palpated in the right axillary region. There was no erythema, warmth or tenderness. The mass caused limitation of the arm flexion. The neurological examination was normal.

Laboratory evaluation revealed a normal CBC, electrolytes value, coagulation profile and ESR of 25 mm/hr.

Radiograph of the chest showed soft tissue opacity in the right axial and no underlying rib erosion or destruction noted. Ultrasound examination revealed a 100x92x48 mm sized multilocular and multiseptated mass in the subcutaneous tissue of the right axilla. The lesion is avascular and displacing the axillary vessels superiorly and laterally. No intrathoracic extension seen. MRI done to delineate the mass and its anatomic relationships further, showed the mass of 10 cm cranio-caudally X 5 cm anteroposteriorly X 9 cm transversely. The MRI revealed well defined multicystic septated lesion. Fluid-fluid level is noted within the lesion. One of the cysts shows hyperintense signals on both T1W and T2W images suggestive of hemorrhage within cyst or proteineous fluid. No evidence of thoracic wall invasion seen. The axillary vessels are displaced.

The patient subsequently had surgical excision of the mass. The operative finding and gross pathology revealed 140 gm, 10x10x5 cm sized multicystic and multiloculated mass microscopic diagnosis verified the tumor to be a cystic hygroma (lymphangioma). The patient had an uneventful postoperative course and was discharged 3 days after surgery.


   Discussion Top


Cystic hygromas (lymphangiomas) may be classified as simple, cavernous, or cystic. Simple lymphangiomas are composed of capillary sized, thin-walled lymphatic channels with considerable connective tissue stroma. Cavernous lymphangiomas are composed of actively growing, dilated lymphatic channels in a lymphatic stroma. Cystic lymphangiomas are single or multiple cystic masses having little, if any, communication with normal lymphatic channels. All three types are benign, slow-growing tumors that result from a congenital malformation of the lymphatic system1.

Cystic hygromas are the tumors that commonly appear early in the life and result from blockage of primordial lymph channels or arrest in the normal development, a process that is complete by 2 months of gestational age[2]. The incidence of the cystic hygroma has been reported to be from 1.5 to 2.8/10003.

Approximately 75%-80% of all cystic hygromas involve the neck and the lower portion of the face. In children, the most common location is the posterior cervical space, followed by the oral cavity. In adults, cystic hygromas are more commonly seen in the sublingual, submandibular, and parotid spaces. Other reported locations include the axilla (20% of cases), the mediastinum (5%), the abdominal cavity (colon, spleen, and liver), the retroperitoneum (kidneys), the scrotum, and even the skeleton[8].

Both sexes are affected equally except in the groin where the incidence is 5 times greater in males[4].

Before the end of the first year of life, 50-60% of hygromas appears and before the end of the second year 80-90% occurs. Rarely cystic hygroma appear in adults.

Many cystic hygromas identified in early gestation by USG are associated with fetal hydrops, numerous associated anomalies and significant incidence of fetal demise. Cystic hygromas have almost a 50% association with chromosomal disorders such as aneuploidy. Cystic hygroma is strongly associated with Turner syndrome (predominantly a 45 XO karyotype), trisomy 21 (Down syndrome), trisomy 18 (Edwards syndrome), and Noonan syndrome5.

Grossly, cystic hygromas are multiloculated, multilobular masses composed of many individual cysts. Microscopically, the cyst walls consist of a single layer of flattened epithelium and the spaces may or may not have may blood containing capillaries suggesting that there may be combined vascular and lymphatic defect6.

On USG, the classic finding is a cystic mass with multiple septa appearing as multiple, asymmetric, thin-walled cysts. Doppler study is to evaluate the vascularity of the mass and to document the relation with the adjacent vessels5. On CT, they appear as nonenhancing, thin-walled, multiloculated masses with a near-water attenuation value. The presence of contrast enhancement of the wall or internal septations suggests superimposed infection or a hemangiomatous component1,7. On MRI, lymphatic vascular malformations have a typical solid appearance with low intensity on T1-weighted spin-echo image, which is equal to that of venous vascular malformations and less intense than that of hemangiomas. Contrast-enhanced T1-weighted images show a very low central intensity with a typical rim enhancement of the lymphatic lesion with cystic hygroma. Moderate intensity at T2-weighted spin-echo image. Cystic hygromas are complex masses with septations and fluid-fluid levels. Their signal intensity is greater than that of fat on T2-weighted and STIR images. The surrounding fascial planes are obliterated if the tumor infiltrates the adjacent soft tissues1.

The size of the lymphangioma appears to be directly related to the degree of obstruction of lympho-venous drainage. The clinical progression of cystic hygroma is variable. Most of these lesions will seem to enlarge intermittently or continuously. Spontaneous regression is rare. Excision is accepted therapy and since cystic hygromas are not malignant neoplasms, there is no need to sacrifice normal structures during operation4.

Meticulous surgical excision in one or more stage is the most accepted treatment. Other types of treatment have been proposed as adjuvant such as radiotherapy and injection of sclerosant agents, but they are controversial. It should be excised when the diagnosis is made because of the danger of severe complications, recurrence, fistula formation or infections have been reported.[8]

 
   References Top

1.Som PM, Lidov M, Lawson W. Hemorrhaged cystic hygroma and facial nerve paralysis: CT and MR findings. J Comp Asst Tomog. 1990. 14(4): 668-671.  Back to cited text no. 1    
2.Joseph AE, Donaldson JS, Reynolds M. Neck and thorax venous aneurysm: association with cystic hygroma. Radiology. 1989. 170: 109-112.  Back to cited text no. 2    
3.Marchese C, Saving E, Dragon E et al: cystic hygroma prenatal diagnosis and genetic councelling, Prenatal diagnosis. 1985, 5:221-227.  Back to cited text no. 3    
4.Yuh WT, Buehver LS, Kao SC et al: Magnetic resonance imaging of pediatric head and neck cystic hygromas. Ann otol rhinol laryngol, 1991; 100:732-742  Back to cited text no. 4    
5.Durre S, Holly N: EMedicine- cystic hygromas. May 6, 2002.  Back to cited text no. 5    
6.Heether J, Whalen T, Doolin E: follow up of complex unresectable lymphangioma. Am surg 1994; 60: 840-841  Back to cited text no. 6    
7.Lee and Stanley, CT and MRI correlation, 3rd edition, vol: 2.  Back to cited text no. 7    
8.Zadvinskis DP, Benson MT, Kerr HH, et al. Congenital malformations of the cervicothoracic lymphatic system: embryology and pathogenesis. RadioGraphics 1992; 12:1175-1189.  Back to cited text no. 8  [PUBMED]  

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Correspondence Address:
P A Vohra
B/103, Sapan flats, Near Motherís School, Harinagar road, Gotri, Baroda
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.28786

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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    Abstract
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