One-stage operation for cervicomediastinal cystic hygroma in infancy

One-stage operation for cervicomediastinal cystic hygroma in infancy Noel L. Mills, M.D.,· and Jay L. Grosjeld, M.D.," New York, N. Y.

CystiC hygroma is a developmental malformation of the lymphatic system that is often detected at birth or in early infancy. The lesion is uncommon in the mediastinum,' but extension into this region often produces alarming respiratory symptoms. The pathogenesis and management of cervicomediastinal cystic hygroma have been controversial. This report presents the results in four patients treated by one-stage resection of combined cervicomediastinal lesions. Pathogenesis

Although most mediastinal cystic hygromas are extensions of a cervical tumor, they may be entirely intrathoracic. It has been suggested that the mediastinal component descends from the neck rather than arising as a primary lesion of the mediastinum." The hygroma is a multilobulated, thin-walled, lymph-containing sac that has a flat endothelial lining. Huntington" theorized that the hygroma sac arises from the mesenchymal tissue and, with ramifications From the Divisions of Pediatric Surgery and ThoracicCardiovascular Surgery. New York University Medical Center, New York, N. Y. Received for publication July 14, 1972. Address for reprints: Alton Ochsner Medical Foundation, 1514 Jefferson Highway, New Orleans, La. 70121. ·Present address: Ochsner Clinic and Ochsner Foundation Hospital, New Orleans, La. 70121. •• Present address: Division of Pediatric Surgery, C. W. Riley Hospital for Children, Indiana University Medical Center, Indianapolis, Ind. 46202.

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and coalescence, acquires communications with veins. Sabin! postulated that a small bud develops from the jugular sac and continues to grow into primitive lymphatics. The infiltrative nature of the proliferating endothelial-lined sacs is the factor responsible for distortion of anatomy and difficulty in eradication. Diagnosis

Mediastinal extension of cystic hygroma should be suspected in all cases in which there is a soft cystic mass in the lower neck. The neck portion of such a mass may be transilluminated. Fluctuation in the size of cervical mediastinal hygromas may be seen during straining and expiratory efforts. The hygroma may displace and compress the trachea, causing symptoms of respiratory obstruction. On fluoroscopy, the mediastinal component may demonstrate a wavy motion with respiration and heartbeat due to the fluid-filled, flaccid consistency of the mass. In certain cases cardiac catheterization may be necessary to determine if the mass is separated from the cardiac chambers. Mediastinoscopy, diagnostic pneumothorax, injection of air or contrast media into the cyst, bronchoscopy, esophagoscopy, and esophagography have not been helpful in delineating the nature of the lesion." Episodes of respiratory distress are among the primary indications for prompt treat-

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Table I. Summary of 4 cases of cervicomediastinal cystic hygroma Patient (sex and age)

1 4 mo.)

I

Clinical findings

X-ray findings

Operative extension

Massive hygroma Right thoracic and with deformity; mediastinal mass; hypoventilation tracheal deviation necessitated excision of chest wall lesion

Mandible, parotid gland, axilla, great vessels, trachea, esophagus, right hilus, prevertebral interdigitation from C. to T 2, thymus 2 Frequent attacks Right mediastinal Subclavian vein, (M, 8 mo.) of respiratory mass; tracheal de- esophagus, right lobe of thymus, viation; pneudistress and inmonia ascending aorta, fection trachea 3 Sudden appearance Mediastinal mass; Thymus, trachea, (M, 16 mo.) of 6 em. cervical severe tracheal pericardium, premass following compression vertebral fascia, respiratory infecaortic arch, thotions racic duct, subclavian vein 4 Recurrent respira- Left mediastinal Left hilum, cervi(F, 23 mo.) cal spine, carotid tory distress; re- mass; tracheal artery, thymus, currence of cystic deviation; pneutrachea, subcervical mass 10 monia clavian vein days after excision (M,

Complications

Result

Transient Horner's No recurrence syndrome, secre- (2 yr.) tions, difficult extubation, copious serous drainage Copious serous drainage

No recurrence (4 yr.)

None

No recurrence (4 mo.)

Air leak from cervical drain

No recurrence (2 yr.)

Legend: C" Sixth cervical vertebra. T2, Second thoracic vertebra.

ment of cervicomediastinal cystic hygroma. These episodes mayor may not follow infection or hemorrhage into a cystic area, and they may result in death of the infant. 1 Infection of the cyst will commonly follow an upper respiratory infection. Mediastinal infection resulting in death has been reported in a case in which the cervical portion of the lesion was resected and the mediastinal portion was left intact. 6 Venous obstruction may likewise occur, as the lesion is in continuity with the superior vena caval system. Chylothorax and chylopericardium have been reported as complications of mediastinal hygroma. 2, 7 As yet, malignant change in a cystic hygroma has not been reported. In addition to the life-threatening complications, cosmetic deformity is part of the rationale for early treatment of these lesions. Case reports Four patients, ranging from 4 to 23 months of age, were operated upon for large,

multiloculated cervicomediastinal cystic hygromas. Indications for operation were recurrent respiratory infections, respiratory distress, gross deformity, and superior vena caval compression. There were no deaths in the group. Table I summarizes the pertinent data in these 4 cases.

Treatment The recommended treatment for cervicomediastinal cystic hygroma has varied from radical forequarter amputation" to watchful expectancy in the hope that the lesion will subside. Radiotherapy to the mediastinum or insertion of radium seeds was recommended some years ago." 10 This treatment now is known to offer the danger of thyroid cancer or impairment of growth to the vertebral cervical epiphyses leading to scoliosis." Aspiration has also been advocated, but it offers the disadvantage of introducing infection. Injection of sclerosing agents such as glucose, Lipiodol, and tincture of iodine

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tions, with the lower portion of the mass being subsequently removed, sclerosed, or radiated, it is our belief that a single-stage procedure offers a number of advantages: ( 1) Excellent exposure is afforded. (2) A second operation and a second anesthesia are avoided. (3) Sclerosing agents or x-ray treatments with their inherent dangers are avoided. (4) The interim complications of hygroma are avoided. In Case 4 of this report, the cervical portion of the hygroma had recurred in the interim between the first and second procedure. One-stage excision of a cervicomediastinal cystic hygroma has also been reported in an 8-week-old infant by Kirschner." Credit for the median sternotomy approach was given to Robert S. Litwak, M.D., in that report. Surgical technique

Fig. 1. A transverse cervical incision is carried to the midline. At the base of the neck, it is continued inferiorly as a median sternotomy.

have occasionally been successful after resection of the cervical portion of a cervicomediastinal hygroma. However, since direct communications to veins and to the thoracic duct have been demonstrated, injection therapy presents certain risks. The lesions are near the brachial plexus, carotid vessels, and jugular veins; therefore thrombosis or nerve damage may be induced. Cervicomediastinal hygroma has frequently been managed by two-stage resection as described by Camishion and Templeton." Gross and Hurwitt? stated that "intimate involvement of regional tissues may cause almost insurmountable technical difficulties in surgical dissection" and advised removing the cervical component first and leaving the mediastinal portion until a later date if the cyst could not be entirely removed through the thoracic outlet. Although combined cervicomediastinal lesions have frequently been managed by two-stage opera-

The procedure for one-stage removal of a cervicomediastinal cystic hygroma is begun with a transverse cervical incision which is extended to the midline. At the base of the neck, it is continued inferiorly as a median sternotomy (Fig. 1). The cervical portion of the mass is dissected out prior to the sternotomy. The sternocleidomastoid muscle on the affected side is divided 2 em. from its origin. Interlacing tongues of hygroma are traced and dissected circumferentially from cervical vessels, nerves, and trachea. Vital and functionally important structures are not sacrificed. When the mass is mobilized to the thoracic inlet, a sternotomy is performed. The internal mammary vessels are immediately divided and ligated on the ipsilateral side. The dissection is carried along these common pathways of extension of the hygroma: (1) posterior to the subclavian and innominate veins and anterior to the arch vessels; (2) retroesophageal and anterior to the vertebral column; (3) laterally into the thoracic inlet and superior sulcus; and (4) inferiorly in sheets along the pericardium and into the thymus. Since hygromas are benign, strict tumor technique is unnecessary. The mass commonly incorporates the phrenic or vagus nerves, which may be stripped of hygroma

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for long lengths without damage. Extension to the thymus is managed by excising the gland. Posteriorly, the dissection becomes tedious as extensions are traced into the prevertebral musculature and paravertebral tissue. Damage to the stellate ganglion should be avoided to prevent Horner's syndrome. It is desirable to keep the tumor intact to maintain the correct dissection plane. Leaks in sizable cysts are closed with 5-0 cardiovascular silk to maintain spherical continuity which facilitates the dissection. No small cysts should be left unopened. Mediastinal and pleural catheters are used for drainage as there is considerable loss of serous fluid for 48 to 72 hours postoperatively. Drainage of the neck may lead to pneumothorax and is unnecessary because of the cervicothoracic continuity. Replacement of drainage is carried out on a milliliter for milliliter basis with Plasmanate or AIbumisol to avoid protein deficit. Antibiotics are used to avoid infection of the numerous recesses of serous fluid that are ideal culture media. Aggressive respiratory management, correlated with frequent blood gas analyses, is extremely important in the postoperative management. Summary

Cystic hygroma is a developmental malformation of the lymphatic system often detected at birth or in early infancy. Although its commonest location is the neck, mediastinal extension often produces alarming respiratory symptoms which require immediate attention. This report suggests that a combined cervical midsternotomy incision for one-stage resection of large cervicomediastinal hygromas is the treatment of choice. Four infants with these unusual massive lesions have been successfully managed by this pro-

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cedure. Significant advantages of this approach include (l) ease of exposure, (2) avoidance of a second operation, (3) no necessity for sclerosing agents or x-ray therapy, and (4) no interim complications of the hygroma. REFERENCES Nanson, E. M.: Lymphangioma (Cystic Hygroma) of the Mediastinum, J. Cardiovasc. Surg. 9: 447, 1968. 2 Camishion, R. c., and Templeton, 1. Y.: Cervicomediastinal Cystic Hygroma, Pediatrics 29: 831, 1962. 3 Huntington, G. S.: The Anatomy and Development of the Systemic Lymphatic Vessels in the Domestic Cat, Philadelphia, 1911, Memoranda of the Wister Institute Annals of Biology. 4 Sabin, F. R.: in Heikel, F., and Mall, F. P., editors: Manual of Human Embryology, vol. 2, Philadelphia, 1912, J. B. Lippincott Company, p. 709. 5 Childress, M. E., Baker, C. P., and Samson, P. c.: Lymphangioma of the Mediastinum: Report of a Case With Review of the Literature, J. THoRAc. CARDIOVASC. SURG. 31: 338, 1956. 6 Gross, R. E., and Hurwitt, E. S.: Cervicomediastinal and Mediastinal Cystic Hygromas, Surg. Gynecol. Obstet. 87: 599, 1948. 7 Stratton, V. C., and Grant, R. N.: Cervicomediastinal Cystic Hygroma Associated With Chylopericardium, Arch. Surg. 77: 887, 1958. 8 Ravitch, M. M.: Radical Treatment of Massive Mixed Angiomas (Hemolymph Angiomas) in Infants and Children With Report of Hemipelvectomy in 13-Day-Old Infant, Ann. Surg. 134: 228, 195 J. 9 Goetsch, E.: Hygroma Colli Cysticurn and Hygraom AxilJare. Pathologic and Clinical Study and Report of 12 Cases, Arch. Surg. 36: 394, 1938. 10 Figi, F. A.: Radium in the Treatment of Multilocular Lymph Cysts of the Neck in Children, Am. J. Roentgenol. Radium Ther. Nucl. Med. 21: 473, 1929. II Kirschner, P. A.: Cervicomediastinal Cystic Hygroma: One-Stage Excision in an EightWeek-Old Infant, Surgery 60: 1104, 1966.