The term hygroma means moist tumor. Cystic hygroma colli are anomalies of the lymphatic system characterized by single or multiple cysts within the soft tissue, usually involving the neck. It contains a clear or cloudy fluid-like lymph.
Cystic hygroma (lymphangioma) is often a benign congenital malformation of the lymphatic system that occurs as a result of sequestration or obstruction of lymphatic vessels. These lesions are usually discovered in infant or children younger than two years of age. Occurrence in adults is uncommon. Lymphangiomas are rare. They account for 4% of all vascular tumors and approximately 25% of all benign vascular tumors in children. It usually affects the head and neck (75%), with a predilection for the left side. Within the neck, the posterior triangle tends to be most frequently affected. Approximately 20% occur in the axilla; less common subsites include the mediastinum, groin, and retroperitoneum.
The objectives of this case report are to present the clinical history and surgical findings, review of literature and the unique problems encountered in the surgical management combine with bleomycin injection of this particular patient.
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 system.1
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 age2. The incidence of the cystic hygroma has been reported to be from 1.5 to 2.8/1000.2
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.3
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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.
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 syndrome3.
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 defect.3
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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 component.1,3.
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 component.1,3.
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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 operation.3
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 operation.3
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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.4,5
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.4,5
Several study had report that Intralesional bleomycin injection is useful for the treatment of cystic hygroma. It should be used in patients with large cystic masses and extensive invasion to reduce the risk of injury to vital organs. Bleomycin at dosages of 0.3-0.6 mg/kg was injected into the cysts and repeated injections were performed at 2-6 weeks.4,5
In this study, we used combination therapy, surgical exicion of the mass and bleomycin injection to reduce recurrence of not completely exiced hygroma.
Combination therapy of hygroma by surgical therapy and bleomycin injection can be effective to reduce recurrence of hygroma that wasn’t completely exiced. This procedur can be proposed for any other case of hygroma.
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1. Mansingani, et.al. 2005. A Case of axillary cystic Hygroma. Gujarat: Hospital Medical College India.
2. Muthukumar, et.al. 2012. Giant cystic hygroma colli in children – A rare case report. Online Journal of Otolaryngology.
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3. Jason L Acevedo, et.al. 2014. An Overview : Cystic Hygroma. Medscape : http://emedicine.medscape.com/article/994055-overview
4. Niramis R, et al. 2010. Treatment of cystic hygroma by intralesional bleomycin injection : experience in 70 pateints. Europe Journal of Pediatric Surgery . May :20 (3) :178-182
5. AK Saha, et al. 2013. Effect of intralesional bleomycin as an alternative therapy for cystic hygroma. Bang Med J Khulna 2013; 46 : 12-15
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