Treatment of giant cervico-mediastinal lymphatic malformations: a case series

Abstract

BackgroundLymphatic malformations are histologically benign vascular structures that vary in anatomic lesion and size. Extensive head and neck lymphatic malformations may be life-threatening. In the present study, we described three difficult-to-treat infants with giant cervico-mediastinal lymphatic malformations accompanied by severe respiratory distress.Case presentationsCase 1. A Korean girl born at a gestational age of 37 weeks and weighing 2920 g had a large cervical mass compressing the trachea. Despite initial OK-432 sclerotherapy, the mass extended over the contralateral retropharyngeal space and mediastinum. Although the cervical mass was completely excised, our patient was not weaned off the ventilator. The mediastinal lymphatic malformation was excised, and our patient underwent continued intensive respiratory care with nasal continuous positive airway pressure for 6 months. She is now 5 years old and doing well without any sequelae. Case 2. A 5-month-old Korean boy showed respiratory difficulty with feeding intolerance after partial excision of a neck lymphatic malformation. We found that the remnant cervical mass had grown into the retropharyngeal space and mediastinum. After a second operation for the cervico-mediastinal mass, he experienced severe respiratory difficulty requiring ventilator care for 6 months. However, he died from central-line fungal sepsis. Case 3. A 30-day-old Korean girl was referred for remnant lymphatic malformation after partial excision. The cervical mass extended to the mediastinum and occupied half of the thoracic cavity, encasing all of the major vessels. After surgical excision, she underwent ventilator care and pleurodesis three times with doxycycline for recurrent pleural effusion. At the age of 26 months, she was weaned off supplementary oxygen and she showed normal development without any sequelae.ConclusionsDespite difficulties in the treatment process, combinations of delicate surgical treatment, appropriate adjuvant sclerotherapy, and intensive respiratory supportive care could result in a good outcome. However, complications due to long-term intensive care could still be considered.

Bronchogenic cyst of the posterior neck mimicking lymphatic malformation

“…The differential diagnosis for a cystic mass in the head and neck region is broad and includes thyroid cysts, cervical thymic cysts, branchial cleft cysts, dermoid cysts, epidermoid cysts, cystic neuromas, teratomas, and vascular malformations including lymphatic malformations 2,3 . Lymphatic malformations are benign neoplasms that arise due to disorganized development of the lymphatic system which can lead to pain, swelling, cellulitis, and compression of adjacent structures 4,5 . In the presented case, the suspicion for a macrocystic lymphatic malformation was high given the history of being present at birth and occasional changes in size, a physical examination demonstrating compressibility and mobility and an ultrasound demonstrating a dilated cystic structure.…”

Long-term results of sclerotherapy for cervical-thoracic-mediastinal lymphatic and venous malformations: A case report

“…The injury caused during thoracotomy was relatively large, the intraoperative bleeding was relatively heavy, and there were complications such as nerve damage. So-Hyun Nam et al [9] performed surgical resection of LM in 3 infants and young children, but it was difficult to completely remove the lesions, and one of the infants died of postoperative infection. Amy W. Cheng et al [10] performed surgical resection of LM in 3 children with thoracoscopy, which is less invasive, but the intraoperative risks are still relatively high, such as bleeding.…”

Intermittent Administration Regimen of Sirolimus for Refractory Cervicofacial Lymphatic Malformation

“…Most patients achieve good results after these combined traditional treatments, 2,3,5,10 even for giant cervico-mediastinal cases. 4 Sirolimus, as an off-label use, further clinical trials are needed to determine its role as first-line treatment for complex LMs, especially its long-term safety remains unclear. Interestingly, Amodeo et al 13 reported that the early attempt with sirolimus before SD was ineffective.…”

“…Moreover, SCL 18 and RA 19 are also effective methods for residual microcystic lesions. Most patients achieve good results after these combined traditional treatments, 2,3,5,10 even for giant cervico-mediastinal cases 4 . Sirolimus, as an off-label use, further clinical trials are needed to determine its role as first-line treatment for complex LMs, especially its long-term safety remains unclear.…”

“…3 A total of 80% to 90% of LMs occur in the head and neck region. 4 The upper aerodigestive tract can be compressed and narrowed by mass effect from adjacent high tension macro-cystic LMs, laryngeal or para-pharyngeal micro-cystic LMs, or circumferential subglottic LMs. These conditions mandate early aggressive intervention.…”