A 35 weeks’ gestational age boy was found to have a huge neck mass antenatally on ultrasonography (US). Soon after delivery, the patient required mechanical ventilation due to respiratory distress. Clinically, the baby was 2 kg. General examination was unremarkable. Locally, there was a huge cystic swelling mainly on the right side of the neck and extending to the left side (Fig. 1). Laboratory workup was unremarkable. US of the neck at 1 day of age showed bilateral cystic lesions more sizable in the right side. The right and left masses were inseparable from each other. The diagnosis was in favor of a lymphangioma or a cystic hygroma. A computed tomography of the neck with contrast confirmed the above-mentioned findings (Fig. 2a and b). The cystic lesions showed multiple enhancing internal septations and multiple enhancing vascular lesions at the superior aspect. On the third day of life, the lesion in the neck was aspirated and then injected with water-soluble bleomycin solution (0.5 mg/1 ml) under US guidance. The aspirate showed altered blood in areas and serosanguinous fluid from other areas. A total of 2 ml (0.5 mg/1 ml/kg) was injected at different sites into the swelling. This was repeated after 1 week. There was a significant reduction in the swelling after the second intralesional bleomycin injections (IBI) and the patient could be extubated safely. Thereafter, IBI was repeated every 2 weeks under US guidance for up to six injection sessions. The dose was adjusted according to the weight. In the last two sessions the dose of IBI was increased to 1 mg/1 ml/kg. The IBI was tolerated well except for local mild erythema and oropharyngeal disorder requiring nasogastric tube feeding that resolved spontaneously. The swelling markedly decreased in size (Fig. 3a–c). There is no recurrence now for 7 months.
A lymphangioma or a cystic hygroma mainly affects the neck and 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 bronchiolitis 2,3. Surgery is the main tool for therapy except in premature infants and when neurovascular structures are involved 4. The aim of surgery is to remove the swelling without complications. This might not be possible, especially in a huge cervical or cervicomediastinal lymphangioma. There is a mortality rate of 2–10% and morbidities that include damage to neurovascular structures, lymphorrhea, chylothorax, recurrence, oropharyngeal disorders that may persist for several months, and bad cosmetic appearance due to heroic and mutilating surgery 4–7. Ming et al. 8 successfully treated a case of bilateral diaphragmatic lymphangioma with IBI after a failed surgical excision.
IBI is as effective as surgery. It causes regression in 88% of cystic hygromas and complete resolution in 44% 9. It can be used in primary or recurrent cases. However, it is claimed that it cannot be used in a huge cystic hygromas involving the mediastinum, the abdomen, or in life-threatening emergency situations 4,9. After injection, the lesion may enlarge in size and cause more compression. This has been challenged by Ibrahim et al. 10 by treating a huge life-threatening cervicomediastinal cystic hygroma using IBI. In such emergency situations the tracheal intubation should continue during IBI until the lesion regresses in size when extubation can be tried with extreme caution.
Side effects of IBI mentioned in the literature include fever, the risk for marked increase in size, local erythema, temporary oropharyngeal disorder, and pulmonary toxicity. Many authors denied pulmonary toxicity if IBI is given locally in proper doses 4,6,11. The technique of IBI whether soluble solution or fat emulsion is well reported in the literature 4,9,11–13.
Spontaneous resolution is uncommon but has been reported in up to 15% of cases 14. The authors of the present report do not recommend waiting for spontaneous resolution to occur. This may end up with respiratory distress or acute respiratory failure requiring tracheal intubation. A difficult intubation should be anticipated. Neuromuscular blockers should be avoided 15. Alternative equipments should be available. This includes light stylet, a bougie, a video laryngoscope, or a laryngeal mask airway. Rigid bronchoscopy, emergency cannula cricothyrotomy with transtracheal jet ventilation, surgical cricothyroidotomy, or tracheostomy may also be required 16,17.
In conclusion, IBI is a safe and effective alternative to surgery. It can be used as a primary therapy in a huge cervical lymphangioma in a newborn even in acute life-threatening emergency.
Conflicts of interest
There are no conflicts of interest.
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