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Successful Non Surgical Management of a Huge Life Threatening Cervicomediastinal Cystic Hygroma

Ashraf H. M. Ibrahim, Ahmed Kandeel, Mohamed F. Bazeed, Mohammed Shaker and Assia Al Rawaf

Pediatric Surgery, Intensive Care Units Armed Forces Hospital Southern Region,

Radiology Department Faculty of Medicine Mansoura University and

Pediatric Surgery Unit Armed Forces Hospital Riyadh, Saudi Arabia

 

Correspondence

Ashraf H. M. Ibrahim
Armed Forces Hospital, Southern Region
King Faisal Military City
P. O. Box: 5062, Khamis Mushait; Kingdom of Saudi Arabia
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

A cervicomediastinal hygroma may be life-threatening due to compression of the airways. The treatment is challenging especially in emergency situations like acute respiratory failure requiring mechanical ventilation. Surgery has a high rate of morbidity and even mortality. Recurrence and injury to major structures may be unavoidable. The authors report on a six-month-old female who presented with acute respiratory failure requiring mechanical ventilation due to a huge cervicomediastinal cystic hygroma encasing major structures in the chest. This is the first report in the literature about successful management of such life threatening condition using water soluble intralesional bleomycin injections (IBI) without surgery.

Key words: Bleomycin; cervicomediastinal hygroma; acute respiratory failure



CASE REPORT

A 6-month-old female weighing 8 kg presented to the emergency room with respiratory distress. There was a huge ill-defined right sided cystic neck swelling dating since birth. The swelling increased in size over the last few hours. Plain Chest x ray showed that the swelling extends into the right hemi thorax causing shift of the mediastinum to the left. The patient was admitted to the pediatric intensive care unit and mechanically ventilated. A computed tomography of the neck and chest showed a huge multicystic lesion consistent with cystic hygroma involving the right side of the neck and extending into the right chest encasing major structures and causing shift of the mediastinum to the left. The lesion in the neck was aspirated then injected with water soluble bleomycin solution 1mg/ml. The aspirate showed altered blood in areas and serosanguineous fluid from other areas. A total of 8ml (1mg/ml/kg) was injected at different sites into the swelling. There was a significant reduction in the cervical swelling after the second injection 15 days later and the patient could be extubated safely. There was local erythema, fever and transient oropharyngeal disorder with excessive salivation that required nasogastric tube feeding. Two weeks later, the neck component of the swelling was injected for the third time and the patient was discharged home the following day in good condition. The injection was then repeated every two weeks for a total of 10 sessions. The last 6 intrathoracic injections were done under CT guidance and general anesthesia. Ten months later, a repeat CT scan showed 92% reduction in the size of the swelling (Fig 1). The neck lesion has completely disappeared and the patient remained completely asymptomatic.

eArt45-1

Figure 1. A Computed tomography of the neck and chest:

A (axial) & B (coronal reformat) before management revealed: the cystic hygroma is seen involving the superior mediastinum stretching the great vessels and extending into the right side nearly occupying the right upper lung zone and upward into the right side of the neck compressing & displacing the trachea to the left side; calculated volume 124 cm3.

C (axial) & D (coronal reformat) six months after management showed significant reduction in the size. Now it has completely disappeared from the neck with no tracheal compression.

Calculated volume = 10 cm3.

DISCUSSION

A cystic hygroma can occur anywhere in the body. It mainly affects the neck and the axilla in 75% and 20% of cases respectively. The lesion may grow and cause compression, infection and hemorrhage [1]. It may present with clinical signs of acute bronchiolitis requiring mechanical ventilation for several days [2&3].

Surgery is the mainstay therapy except in premature infants and when major neurovascular structures are involved [4]. The goal of surgery is to completely remove the swelling without complications. This is impossible in a cervicomediastinal hygroma encasing major structures. Surgery has a mortality rate of 2-10% and complications that include damage to neurovascular structures, lymphorrhea, chylothorax, recurrence, oropharyngeal disorder that persists for several months. This is in addition to the bad cosmetic appearance due to the heroic and mutilating surgery [4, 5, 6, and 7]. Recently, Ming et al successfully treated a case of bilateral diaphragmatic lymphangioma with intra lesional bleomycin injection (IBI) after a failed surgical excision [8].

IBI is as effective as surgery since it causes regression in 88% of hygromas and complete resolution in 44% [9]. It is indicated in both primary and recurrent cases unless the hygroma is huge involving the mediastineum, the abdomen or in life threatening emergency situations [4, 9]. In the present case the authors used water-soluble IBI in a huge cervicomediastinal lesion encasing major structures and in a life threatening emergency. During the initial injections, the patient was kept intubated in the intensive care unit for fear of the risk of sudden increase in size.

The reported IBI side effects include fever, the risk of marked swelling especially for mediastinal lesions, local erythema and pulmonary toxicity. The latter has been denied by many authors [4, 6, 10]. In the present case, there was a transient oropharyngeal disorder which improved within few days. IBI was first reported by Jura et al in 1977 [11]. The technique of IBI whether a water soluble solution or a fat emulsion is well reported in the literature [4, 9, 10, 12].

In conclusion, IBI is a safe and effective alternative to surgery. It can be used as a primary therapy in an extensive cervicomediastinal hygroma even in acute life threatening emergency.

 

 

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