Cervical cystic hygroma/lymphangioma: an acquired idiopathic late presentation

Lymphangiomas or cystic hygromas are relatively uncommon congenital malformations of the lymphatic system which usually present in the first years of childhood. Presentation in adult life is rare. We present a case of cervical adult cystic hygroma and discuss radiological and histopathological features as well as the management of these neck masses.     

Non surgical management of cystic lymphangioma

Aim/Purpuse: To evaluate our experience of 19 patients of lymphangioma who were treated by intralesional Bleomycin.Materials and Methods: Nineteen patients of lymphangioma aged between 16 days to 11 years were managed in the department. The male-female ratio was of 2:1. Commonest sites were in the neck (58%) followed by axilla (21%). The patients were treated by intralesional bleomycin injection. Bleomycin was given at a dose not exceeding 0.5 unit/kg/dose at interval of 2 weeks. Reduction in size of the mass was noted in between 2 weeks to 16 weeks and number of injections required for each patient varied from 1 to 6. Follow up ranged from 1– 7 Year.Result: In injection group, significant reduction of mass was noted in 84% (n=l6) and 57% (n=11) of them showed complete disappearance. No serious complications were noted in any patient.Conclusion: Our experience showed that Bleomycin in aqueous solution is a good sclcrosing agent in the management of lymphangioma.     

Sclerotherapy for lymphangioma in children

Although surgical excision has been considered to be the treatment of choice by most of the surgeons, sclerotherapy of lymphangioma has gained popularity during recent years. A prospective clinical trial was conducted to evaluate the efficacy of bleomycin and OK-432 sclerotherapies for treating lymphangioma in children. Fifteen patients were enrolled in the study conducted between 1998 and 2002. All patients were hospitalized. Bleomycin or OK-432 was injected into the lesion. Patients were observed in the hospital for 1 day. The response was considered as excellent in cases with total disappearance of the lesion. If the lesion was regressed more than 50% of the original size, the response was considered as good. Little or no change in the size of lesion was considered to be a poor response. Ten girls and five boys with ages ranging from 4 days to 12 years were treated. Five patients had been operated previously for lymphangioma. Most of the lesions were located only in the cervical region (n=13). Other cases revealed extensions into the mediastinum (n=1) or axilla (n=1). Bleomycin (n=8, 2.87+/-2.03 inj.), OK-432 (n=5, 2+/-1 inj.) or both in order (n=2, 6 and 16 injs.) were injected. No allergic reaction, scar formation or pulmonary complication was encountered. Fever (11%), local reactions (4%) and vomiting (2%) were encountered following a total of 55 injections. After a follow-up period of 6-36 months, the responses were excellent in 53.4%, good in 26.7% and poor in 6.6%. Macrocysts disappeared in the remaining 13.3% of patients who had mixed cervicofascial lymphangioma. Lower success rates were encountered among patients who had undergone prior surgery. Sclerotherapy with bleomycin and OK-432 is effective in the treatment of lymphangioma in children. Adverse effects are minor and rarely encountered. However, the surgeon should be alert for the possible serious complications. Sclerotherapy can be also used as an adjunctive therapy in the treatment plan of widespread or mixed forms of lymphangiomas. In the light of our results, sclerotherapy should be preferred as the primary mode of treatment in childhood lymphangiomas.     

A gigant cystic lymphangioma of the neck

The cystic lymphangiomas are congenital pathology of lymphatic system which concern 90% of children population, adults suffer very seldom. The authors present the case of giant cystic lymphangioma of the neck in the 37-year-old man with symptoms of tumor of the neck. The USG, CT and MRI confirm the presence of polycystic tumour with feature of bleeding into one of the cavities. Surgical treatment was performed. The patient is controlled without any relaps. The authors remind this rare pathology that should be considered in case of neck tumours.     

颈部囊性淋巴管瘤患者术后疗效观察

目的:探讨囊性淋巴管瘤的治疗方法及疗效。方法:10例颈部囊性淋巴管瘤患者于1998-07-2008-03接受改良手术切除,即敞开囊腔后,在直视下行囊外切除。1例外院手术后腮腺深叶复发患儿及1例1岁患儿接受平阳霉素局部注射治疗。结果:10例患者肿瘤完全切除,未出现神经损伤等明显并发症,随访0.5～9.0年无复发;2例局部注射治疗者,肿物基本消失。结论:颈部囊性淋巴管瘤应以手术治疗为主,开囊直视下手术有利于肿瘤完全切除而又不会损伤周围组织。     

Cervical cystic hygroma]

We report the case of a 27-year-old patient suffering from a cystic hygroma of unknown etiology. She presented with an asymptomatic fluctuant mass in the right posterior triangle of the neck. NMR-scans revealed a cystic tumor in the above-mentioned area without enhancement after administration of gadolinium. The cystic hygroma was surgically excised. Three and six months after surgery there was no sign of recurrence. By means of the presented case report and review of literature we discuss diagnosis and treatment of cystic hygromas.     

Congenital cervical choristoma of the neck mimicking cystic lymphangioma

IntroductionChoristoma is a mass presenting normal histology, but in an abnormal location. Cystic choristoma is rarely reported in the head and neck region. Neonatal cystic masses in the neck suggest usually correspond to a diagnosis of cystic lymphangioma.Case reportWe report a case of a congenital cystic choristoma of the neck clinically and radiologically mimicking cystic lymphangioma.DiscussionCongenital cystic choristoma is an extremely rare lesion, essentially described in neonates, composed of various types of tissues. The diagnosis of congenital cystic choristoma may be suggested on imaging and must be confirmed by histopathological examination. Treatment consists of complete surgical resection.     

Treatment of lymphangioma with OK432 in children

From 1998 to 2003 6 children with lymphangioma of cervico-facial region were treated with OK-432 in Polish Mother Health Institute. Three of them had nonresectable leasion and two required tracheostomy during first months of life. Each patient received from 1 to 10 sessions of obliteration. In total 23 sessions were performed. The youngest patient treated with OK-432 was 2 weeks and the oldest 2 years old. The response to treatment was very good in 4, good in 1 and fairly good in 1 case. In three patients criodestruction was applied as an additional treatment. In two children residual lesions were subsequently removed surgically. There were no serious complications. After obliteration sessions only 2-3 day temperature increase and local inflammation occurred. We consider obliteration with OK-432 as a treatment of choice in lymphangiomas in children.     

颈部囊状淋巴管瘤合并感染的诊治体会

目的总结颈部囊状淋巴管瘤合并感染的诊断和治疗经验.方法分析2例患者的临床病理资料和治疗效果.结果反复穿刺抽液1例,病灶清除1例,均治愈,随访5年无复发.结论穿刺抽液、外科手术是治疗本病的有效方法.     

Cervical thymic remnants in children

OBJECTIVE: Define the clinical presentation, diagnostic value of preoperative imaging, surgical management, and outcomes of treatment of congenital cervical thymic remnants in children. DESIGN: Retrospective cohort. SETTING: Single tertiary care institution. PATIENTS: 20 children who underwent excision of cervical thymic remnant, 1975-2006. MAIN OUTCOMES MEASURED: Utility of preoperative imaging to diagnose cervical thymic anomalies; success of surgical treatment of cervical thymic remnants. RESULTS: A total of 20 children were identified, with an average age of 6.98+/-5.63 years. All ectopic thymus tissue was found in the embryonic distribution area associated with the third branchial pouch. Fourteen patients underwent excision of a cystic ectopic thymus. Four of these patients exhibited lesions isolated to the cervical region, and 10 patients displayed lesions involving cervicomediastinal areas. Six patients underwent excision of solid ectopic cervical thymus, and each of these was an unanticipated mass encountered during surgical dissection for other procedures. 83% of patients with solid ectopic cervical thymus presented at age 3 or younger. Physical exam and preoperative imaging correctly diagnosed thymic remnants in 15% patients. Resection of thymic remnants was successful in all patients, and there were no recurrences. CONCLUSIONS: Though rare, thymic remnants should be considered in the differential diagnosis of masses presenting in locations associated with derivatives of the third branchial pouch. Though preoperative imaging is helpful in identifying the extent of these lesions, congenital thymic remnants prove difficult to diagnosis radiologically. Surgical excision is the diagnostic and therapeutic treatment of choice in the management of cervical thymic remnants.     

婴幼儿颈部巨大囊状淋巴管瘤的诊治体会(附8例报告)

目的 评价婴幼儿颈部巨大囊状淋巴管瘤（GCCL）治疗中的几个临床问题。方法 回顾性分析1996年4月至2000年6月收治的8例GCCL患儿临床资料，结合随访结果探讨相关问题。结果 全部患儿疗效满意，随访1－5年无复发，患儿发育及颈部外观正常。总结了本组GCCL的临床特征，对GCCL诊断中的穿刺，气管切开术及瘤体处理方式等几个临床问题进行了评价。结论 一般不提倡行气管切开术来处理婴幼儿GCCL患者的急性喉梗阻，穿刺可作为诊治GCCL的一种辅助手段。根据婴幼儿GCCL的结构特点选择不同的瘤体处理方式是提高疗效，减少手术并发症的关键。     

Cervical presentations of thymic anomalies in children

OBJECTIVE: To better define the clinical manifestations, radiologic imaging and the surgical management of cervical thymic lesions in children. STUDY DESIGN: Multi-center retrospective case review. METHODS: The charts of all children with pathologically confirmed thymic lesions at six children's hospitals (1990-2002) were reviewed for demographics, physical findings, X-ray findings, operative outcomes and pathology. RESULTS: There were a total of 15 children, 2 of whom had ectopic cervical thymus and 13 who had thymic cysts. They ranged in age from 1 month to 18 years. Thymic lesions were more common in males. Ectopic cervical thymus was best defined by MRI whereas thymic cyst had a more consistent appearance on CT. All children had successful surgical resection with no recorded complications or recurrences. CONCLUSIONS: Cervical thymic lesions are rare. Ectopic cervical thymus tends to be found primarily in infants whereas thymic cysts occur in a wider age range. Radiologic imaging is important but is not histologically specific. Definitive diagnosis and cure requires complete surgical excision.     

Cervical vestibular evoked myogenic potentials in children

IntroductionCervical vestibular evoked myogenic potential is a test used in neurotological examination. It verifies the integrity of vestibular function through a muscular response evoked by an acoustic stimulation which activates the saccular macula. Normal standards in adults have been established, however, there are few published data on the normal responses in children.ObjectiveTo establish normal standards for vestibular myogenic responses in children without neurotological complaints.MethodsThis study's design is a cohort with cross-sectional analysis. The sample consisted of 30 subjects, 15 females (50%) and 15 males (50%).ResultsThe age of the subjects ranged between 8 and 13 years, with a mean of 10.2 (± 1.7). P1 peak showed an average latency of 17.26 (± 1.78) ms and a mean amplitude of 49.34 (± 23.07) μV, and the N2 peak showed an average latency of 24.78 (± 2.18) ms and mean amplitude of 66.23 (± 36.18) μV. P1–N2 mean amplitude was 115.6 (± 55.7) μV. There were no statistically significant differences when comparing by gender or by laterality.ConclusionWe established normal values of cervical myogenic vestibular responses in children between 8 and 13 years without neurotological complaints.     

Paranasal mucoceles in children with cystic fibrosis

Sinus mucocele is rare in the paediatric age, and so far no prevalence data have been reported in children with Cystic Fibrosis (CF). Moreover, safety and efficacy of endoscopic management of sinus mucoceles has been widely proven but only in the adult population. The aim of our study was to evaluate the prevalence of this complication and the efficacy of endoscopic sinus surgery in CF patients during the initial years of life. Among the 242 CF patients born in the period between 1990 and 2001 and in regular follow up at our CF Centre, 90 patients with possible symptoms of chronic upper airways disease (CUAD) underwent a comprehensive ENT examination including rhinofibroscopy. In selected cases a CT scan of the paranasal sinuses was also performed. CUAD was diagnosed in 55/90 because of the consistent presence of nasal obstruction, combined with at least two other nasal symptoms such as chronic nasal discharge, snoring, epiphora. Diagnosis of mucoceles (five maxillary bilateral mucoceles, one maxillary unilateral, three maxillary and etmoidal mucoceles) was done by means of CT scan in 9/15 who performed the examination. Median age at the diagnosis was 4+/-0.5 years, ranging from 0.5+/-7 years, showing a prevalence of 16.4% (9/55) among patients with symptoms. Endoscopic sinus surgery was performed in all the cases. The follow-up period ranged from 3 months to 6 years with no recurrence observed. Sinus mucocele in CF population is less unusual than expected and a high degree of suspicion is needed. Endoscopic sinus surgery seems to be a safe and efficient treatment of this complication also in a paediatric population at a high risk as for the CF patients.     

Surgical treatment of cervicofacial cystic hygromas in children

AIM: The aim of this study was to evaluate the results of surgical treatment of cervicofacial cystic hygromas in children. PATIENTS AND METHODS: Medical records of 17 patients who were operated for cervicofacial cystic hygroma between 1985 and 2004 were evaluated in terms of age, gender, symptoms, diagnostic workups, outcomes and complications. RESULTS: There was a slight male predominance -- 10 (59%) boys and 7 (41%) girls. Nine (53%) out of 17 lesions were located on the left side of the neck, 7 (41%) lesions were located on the right side of the neck and 1 lesion (6%) was located in the middle of the neck. Eleven (65%) lesions were located in the infrahyoid region, 6 (35%) lesions in the suprahyoid region. Following surgical excision of the lesion, we encountered 4 postoperative complications: 1 recurrence (6%), 2 facial paralyses (12%) and 1 collection of fluid (6%) at the resection site. The patient who had a recurring lesion needed to be reoperated, other complications were treated conservatively. CONCLUSIONS: Cervicofacial cystic hygromas are easy to diagnose. There is no need for expensive and time-consuming imaging studies. Surgery seems the treatment of choice. However, nonsurgical treatment options may be considered for the lesions located over the parotid region in order to avoid complications of surgery.     

Endoscope-assisted excision of macrocystic lymphangioma in neck via anterior chest approach in infants and children

Objectives

Macrocystic lymphangioma of the neck is traditionally removed via an overlying incision near the mass. The resultant scar can be aesthetically displeasing, and endoscope-assistant surgery is still rarely used in pediatric subjects. So we report the endoscope-assisted excision of the macrocystic lymphangioma via anterior chest, and explore its feasibility, validity and security.

Methods

From June 2005 to December 2008, 6 infants and children with macrocystic lymphangioma accepted endoscope-assisted excision via anterior chest approach.

Results

All procedures were successfully performed using the endoscope-assisted approach. There were no conversions of the operations or postoperative complications. All patients and their parents were satisfied with the cosmetic results.

Conclusions

Endoscope-assisted excision of the macrocystic lymphangioma via anterior chest approach in children can be applied effectively, safely and feasibly, allowing adequate exposure for dissection, and resulting in a good cosmetic result, and it would be considered as a new surgical approach for these patients.     

Post cricoid lymphangioma

A 69-year-old female patient presented with symptoms characteristic of globus pharyngeus and barium swallow examination was normal. Her symptoms persisted and pharyngoscopy was undertaken; a post-cricoid polyp was found and removed. Histological examination revealed this to be a lymphangioma. Histological appearances, pathogenesis and treatment are discussed. The case illustrates that persisting symptoms presenting as globus pharyngeus should be further investigated to exclude rare lesions.     

Nasal polyps and sinusitis in children with cystic fibrosis

Eighteen children suffering from cystic fibrosis and nasal polyps were studied. Two patients presented with nasal polyps at two years of age; the remaining patients had an initial polypectomy at any age up to fifteen years (mean age of seven and a half years). There were no obvious correlations between the cultures of the sinus washouts and the corresponding sputum samples. Organisms were only grown from five of the fifteen wash outs and from nine of the sputum samples. The patients with severe polyposis were not obviously more atopic.