Ann Thorac Surg 2007;84:1027-1029
© 2007 The Society of Thoracic Surgeons
Case Reports
One-Stage Excision of Massive Cervicomediastinal Lymphangioma in the Newborn
Denis A. Cozzi, MDa,*,
Augusto Zani, MDa,
Giuseppe d’Ambrosio, MDa,
Antonietta Piserà, MDa,
Mario Roggini, MDb,
Francesco Cozzi, MDa
a Pediatric Surgery Unit, Policlinico Umberto I, University of Rome "La Sapienza," Rome, Italy
b Pediatric Radiology Unit, Policlinico Umberto I, University of Rome "La Sapienza," Rome, Italy
Accepted for publication April 26, 2007.
* Address correspondence to Dr Cozzi, Pediatric Surgery Unit, University of Rome "La Sapienza," Policlinico Umberto I, Viale Regina Elena, 324, Rome, 00161, Italy (Email: da.cozzi{at}uniroma1.it).
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Abstract
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We report the first neonate successfully treated with one-stage complete excision of massive cervicomediastinal lymphangioma producing alarming respiratory distress. Early postoperative course was complicated by refractory bilateral chylothorax, which effectively responded to insertion of external pleuroperitoneal shunts. At the 6-year follow-up, the child remains symptom free and recurrence free. One-stage resection for cervicomediastinal lymphangioma is curative and well tolerated even shortly after birth.
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Introduction
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Lymphangioma is a congenital lymphatic malformation with a predilection for the head and neck, usually diagnosed during early infancy. This is a benign lesion, but is often challenging, particularly if there is an extension into the mediastinum [1, 2].
We report the first newborn successfully treated with one-stage complete resection of massive cervicomediastinal lymphangioma.
A 38-week gestation, 3.99 kg boy was born by elective cesarean section after an uneventful pregnancy. Soon after birth, he presented with alarming respiratory difficulty that was attributed to a compressible mass occupying the whole base of the neck. Stridor, intercostal, and substernal retractions were noted. The noticeable collar-shaped lesion was highly suggestive of lymphangioma. Ultrasonography clinched this diagnosis, demonstrating a cervical multiseptate cystic mass that also extended into the mediastinum. A chest x-ray film showed massive bilateral intrathoracic involvement of the lesion, which was compressing and anteriorly displacing the trachea (Figs 1A–1B). Endotracheal intubation was required because of increasing respiratory distress. On day 4 of life, complete excision of the mass was accomplished through a combined collar cervicotomy and mid-sternotomy (Figs 2A–2B). This approach yielded optimal operative exposure, although dissection was tedious and the cysts were often entered. The lesion was stripped off all neighboring vital structures, which were carefully spared, except the thymus. Microscopically, the lesion was composed of many endothelial-lined cysts consistent with the diagnosis of lymphangioma. On postoperative day 3, bilateral pleural effusions occurred, leading to respiratory compromise and mechanical ventilation. Needle aspiration ascertained bilateral chylothoraxes. Initial management consisted of chest tube drainage, fresh-frozen plasma, and total parenteral nutrition. A persistent chyle drainage for more than 3 weeks, exceeding 100 mL/d (22 mL/K g/d), prompted the insertion of bilateral externalized pleuroperitoneal shunts (Denver Biomaterials Inc, Golden, CO). In addition, a temporary tracheostomy was needed because of several failed attempts to extubate. Owing to oropharyngeal dysphagia, a feeding gastrostomy was also required. The chyle leakage eventually ceased and the shunts were removed within 10 weeks of their insertion.
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Fig 1. (a) Anteroposterior chest x-ray film shows a large intrathoracic mass (arrows) corresponding to the mediastinal component of the lesion. (b) Lateral view demonstrates anterior displacement and compression of the intrathoracic trachea (arrows).
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Fig 2. (a) Preoperative appearance of the cervical component of the lesion occupying the whole base of the neck. (b) Operating field after completion of dissection. The aortic arc (A), brachiocephalic veins (B), left vagus nerve (V), left internal jugular vein (J), right innominate artery (I), carotid arteries (C), trachea (T), thyroid (Th), right lung (L), and manubrium of sternum (M) are clearly visible.
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At the child’s 6-year follow-up, he is thriving well with no neurologic, respiratory, or gastrointestinal sequelae.
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Comment
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Cervicomediastinal lymphangioma is an uncommon condition that may represent a serious cause of neonatal airway emergency, such as the present case. Physical and ultrasonographic examination accurately diagnosed the cervical component of the lesion. A chest x-ray film revealed a large intrathoracic space-occupying mass that was easily recognized as the mediastinal extension of the lesion. This simple investigation should always be requested when a cervical lymphangioma is detected, and it remains by far the best diagnostic tool to rule out the presence of a mediastinal component. More sophisticated imaging studies are often unnecessary [1].
Excision of cervicomediastinal lymphangioma is a tedious surgery that needs meticulous attention to detail during dissection because no cleavage plane exists between the tumor and the surrounding nerves and blood vessels. Because the lesion is benign, it is probably best to allow small tumor remnants to remain attached to important vital structures rather than risk their injury. However, no small cyst should be left unopened. Gross and Hurwitt [2] stated that "almost insurmountable technical difficulties may be encountered during the operative dissection." They first advocated a two-stage resection, the cervical component being primarily excised, and the mediastinal portion being removed by subsequent thoracotomy, if sclerosing treatment is unsuccessful.
In our case, the severe respiratory distress, due to compression of the intrathoracic trachea exerted by the large mediastinal component of the lesion, demanded a one-stage resection. This procedure was first outlined by Kirschner [3], who described this technique in an 8-week-old infant operated on through a mid-sternotomy. The advantages of one-stage resection are excellent exposure, avoidance of a second operation, no adjunctive treatment, and no interim complications [1].
Only two cases of cervicomediastinal lymphangioma occurring during the neonatal period have been previously described. Both patients were symptomatic at birth and shared the same unfavorable outcome. The first case was a full-term baby girl who presented with severe respiratory distress within a few hours of life due to tracheal compression [4]. She underwent staged excision of only the cervical portion of the lesion, but died a few months later after additional surgery. The second case was a female newborn with postnatal respiratory insufficiency who underwent an emergency surgical excision through a mid-sternotomy [5]. Postoperatively her pulmonary function did not improve despite mechanical ventilation, and the baby succumbed a few hours after surgery. Postmortem examination demonstrated small, hypoplastic lungs and atelectasis bilaterally. These findings were considered the result of a substantial fetal compression on the developing lungs exerted by the space-occupying lesion.
The postoperative course of our patient was complicated by the development of chylothorax whose occurrence has been also described in a previous report [6]. However, in the present case, chylothorax resulted refractory to conventional conservative measures. Therefore we opted for pleuroperitoneal shunting, which is a reasonable and effective alternative to major surgical procedures in both adults and children, especially when the persistent leak is diffuse, such as in our patient [7, 8].
Intensive respiratory care during the postoperative period is of paramount importance. In our patient, a temporary tracheostomy was needed leading to a steady recovery thereafter. In a previous report, a tracheostomy was indicated due to retained secretions in the tracheobronchial tree [2]. Immaturity of the lungs, as a consequence of the presence of the lesion acting as a space-occupying lesion, may be another explanation of the postoperative respiratory problems encountered in some of these patients [5].
One-stage complete resection for cervicomediastinal lymphangioma is curative, and it is well tolerated even shortly after birth. We believe that the present case represents the youngest patient treated to date.
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References
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- Grosfeld JL, Weber TR, Vane DW. One-stage resection for massive cervicomediastinal hygroma Surgery 1982;92:693-699.[Medline]
- Gross RE, Hurwitt E. Cervicomediastinal and mediastinal cystic hygromas Surg Gynecol Obstet 1948;87599–10.
- Kirschner PA. Cervicomediastinal cystic hygroma: one-stage excision in an eight-week-old infant Surgery 1966;60:1104-1107.[Medline]
- Stratton VC, Grant RN. Cervicomediastinal cystic hygroma associated with chylopericardium Arch Surg 1958;77:887-891.[Abstract/Free Full Text]
- Csicsko JF, Grosfeld JL. Cervicomediastinal hygroma with pulmonary hypoplasia in the newly born Am J Dis Child 1974;128:557-558.[Abstract/Free Full Text]
- Camishion RC, Templeton JY. Cervicomediastinal cystic hygroma Pediatrics 1962;29:831-834.
- Milsom JW, Kron IL, Rheuban KS, Rodgers BM. Chylothorax: an assessment of current surgical management J Thorac Cardiovasc Surg 1985;89:221-227.[Abstract]
- Wolff AB, Silen ML, Kokoska ER, Rodgers BM. Treatment of refractory chylothorax with externalized pleuroperitoneal shunts in children Ann Thorac Surg 1999;68:1053-1057.[Abstract/Free Full Text]
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Abstract
Introduction
