Lymphocutaneous Fistula As a Long-Term Complication of Multiple Central Venous Catheter Placement

Abstract

We report a case of a lymphocutaneous fistula in a 19-month-old boy who had been a premature neonate, born in the 23rd week of gestation. The fistula, an apparent complication of central venous line placement during the patient's first 5 months of life, was composed of a distinct lymphatic vessel bundle in the right supraclavicular region, with its exit point at the posterior aspect of the right shoulder. The drainage ceased imme-diately after resection and repair of a 1-cm obstruction in the superior vena cava.

We report the case of a 19-month-old boy in whom a lymphocutaneous fistula developed secondary to superior vena cava (SVC) syndrome, thoracic duct injury, or both, after central venous catheter (CVC) placement in early infancy. To our knowledge, this is the 1st such report in the English medical literature.

Case Report

A 19-month-old boy was hospitalized for worsening chronic lung disease with increased oxygen requirement and labored breathing. His medical history included premature birth in the 23rd week of gestation and delivery by cesarean section secondary to chorioamnionitis in a 34-year-old mother (gravida 2, para 1). He was a triplet A neonate weighing 480 g at birth. After resuscitation in the delivery room, the infant had a lengthy stay in the neonatal intensive care unit complicated by prolonged intubation (102 days), pulmonary interstitial emphysema, respiratory distress syndrome, right pleural effusion (nonchylous), necrotizing enterocolitis with jejunal perforation, Grade II intraventricular hemorrhage, and 3 episodes of sepsis. These episodes of sepsis coincided with either a right subclavian Broviac tunneled CVC (Bard Access Systems, Salt Lake City, Utah) or a right internal jugular CVC in place. The right subclavian CVC was difficult to remove because of adhesions. The infant was discharged from the hospital at 6 months of age on supplemental oxygen.

He subsequently developed respiratory syncytial virus (RSV) bronchiolitis at 8 months of age, right chylothorax requiring talc pleurodeses at 9 months of age, chronic lung disease, and failure to thrive, necessitating gastrointestinal tube placement at 15 months of age. Upper-extremity and neck edema, 1st noted in the in-fant at 4 months, remained clinically stable.

At 19 months, when the boy was hospitalized for chronic lung disease with increased oxygen requirement and labored breathing, physical examination revealed a disproportionately large head; facial plethora; and visibly dilated veins over both cheeks, the neck, and the upper torso. There were no heart murmurs or gallops, and breath sounds were clear with poor air movement. The capillary blood gas results on 2 L/min oxygen by nasal cannula were pH, 7.48; carbon dioxide partial pressure (PCO₂), 45 mmHg; and oxygen partial pressure (PO₂), 53 mmHg. Com-plete blood count results and electrolytes were normal. Chest radiography revealed chronic lung changes with no infiltrates or effusions.

The boy's respiratory status improved quickly with aggressive pulmonary toilet, frequent albuterol nebulizer treatments, and steroid therapy. On hospital day 3, a projectile milky discharge was noted coming from the posterior aspect of his right shoulder. This chylous discharge contained sodium (133 mmol/L), glucose (207 mg/dL), triglycerides (3,465 mg/dL), albumin (2.5 gm/dL), and white blood cells (1,260/μ L). Bacterial cultures were negative. The gastrointestinal-tube feedings were changed to an electrolyte solution (Pedialyte; Ross Products Division, Abbott Laboratories, Columbus, Ohio) and the discharge rate fell from 20 to 10 cc/h over the next 24 hours. A computerized axial tomographic (CAT) scan of the neck and chest showed only chronic lung changes. A fistulogram using intravenous contrast under fluoroscopy confirmed the presence of a lymphocutaneous fistula (Fig. 1).

Fig. 1 Fistulogram of lymphocutaneous fistula showing the injection catheter (arrow) in the posterior aspect of the right shoulder (exit site). The fistulogram shows the bundle of lymphatic vessels in the right supraclavicular region, as well as the lymph vessels of the right arm.

The cause of the fistula was suspected to be related to the patient's SVC syndrome. Right-sided cardiac catheterization identified the obstruction (Fig. 2), approximately 1 cm long, in the region of the SVC just above the right atrium. The external jugular ve-nous pressure was 37 mmHg, and the right atrial pressure was 4 mmHg.

Fig. 2 A) Anterior-posterior view of simultaneous collateral vessel and roof of the right atrium-SVC angiograms showing the obstructed SVC with collateral vessels connecting the superiorly located venous chamber and the roof of the right atrium-SVC stump. B) Lateral view angiogram showing the distance (1 cm) between the superiorly located venous chamber and the roof of the right atrium-SVC stump.

SVC = superior vena cava

On hospital day 5, a mediastinotomy was performed, which confirmed the catheterization findings and revealed a large venous vessel 1 cm above the right atrium. The obstructed portion was excised and an end-to-end anastomosis using an autologous pericardial patch was performed. After the surgical repair, the patient's venous pressures above and below the anastomosis were equal.

The postoperative course was complicated by pulmonary hypertension and difficulty in weaning the boy from the ventilator. The fistula discharge resolved immediately after surgery and the superficial collateral veins began to regress. Two-dimensional echocardiography with color flow and bubble contrast confirmed a patent SVC with flow into the right atrium and no evidence of obstruction. The patient was extubated on the 5th postoperative day. The next day, however, he developed fever, leukocytosis, and right lower-lobe pneumonia with respiratory distress that required 50% oxygen by face mask and continuous albuterol nebulization. By the 11th postoperative day, the boy had been weaned to 2 L/min oxygen but remained persistently febrile. The next day, he became pulseless and cyanotic, requiring reintubation and aggressive pressor support using epinephrine and dopamine. Multiple-system organ failure ensued and he died on postoperative day 13. Postmortem examination was declined by the family.

Discussion

Chylous fistulas (pericardial, pleural, and cutaneous) are most often seen as complications of cervical or thoracic surgery, ¹ trauma, ² CVC placement, ³ femoral artery reconstruction, ⁴ renal transplantation, ^5,6 and malignancy. When surgically related, these fistulas usually occur during the early postoperative period. Congenital chylopericardial fistulas have been reported and have presented in children as late as 12 years of age. ^7,8 Lymphangiectasias (pulmonary and gastrointestinal) have been reported in patients with Noonan's syndrome. ⁹ Adults with portal hypertension and hepatic cirrhosis often develop increased lymphatic flow secondary to splanchnic venous congestion and are at risk for chylous fistula formation. ^10,11 Our case is unique in that, to our knowledge, it is the 1st reported case describing the late development of a lymphocutaneous fistula in a young infant after the development of SVC syndrome, which in turn was caused by multiple CVC placement.

The proposed cause of lymphocutaneous fistula formation in this patient is thoracic duct injury together with SVC obstruction as a result of multiple CVC placement during the first 5 months of life. The presence of multiple CVCs, particularly during episodes of infection or sepsis, likely contributed to the development of fibrous adhesions. Over time, the head and neck vessels underwent progressive external compression from the adhesions, fibrosed, and ultimately became obstructed. With the development of SVC obstruction, pressure within both the venous vessels and the lymphatic channels increased, leading to collateral vessel formation (Figs. 1 and 2). The mean pressure in the external jugular vein (venous chamber) was 33 mmHg greater than in the right atrium.

Variations in lymphatic and thoracic duct anatomy have been well described. ¹ Such variations include: multiple branching of the thoracic duct (40%); right-sided termination of the thoracic duct (5%); and thoracic duct termination at the subclavian-jugular junction or the external jugular, innominate, or internal jugular vein. Further, the thoracic duct may extend as high as 5 cm above the clavicle. ¹² Any of these variations, added to the placement of multiple CVCs, could have contributed to or increased the risk of thoracic duct injury in our patient; however, this could not be confirmed in the absence of a postmortem examination.

The diagnosis of chylous fistula is both a clinical and a biochemical process; moreover, mapping the tract is often necessary in order to determine appropriate management. Frequently, though, the fistula involves the pleural or pericardial spaces, necessitating alternative means of mapping the tract. Lymphangiography is considered the gold standard, ² but a CAT scan after ethiodized oil and fat ingestion and lymphoscintigraphy have also been used. ^2,6 Intraoperatively, identification of the thoracic duct injury can be facilitated by Trendelenburg positioning and a Valsalva maneuver. ¹

Chylous fistulas usually close spontaneously with the application of conservative treatment, such as a diet with medium-chain triglycerides or low fat content, parenteral nutrition, repeated aspiration, elevation of the head, or local pressure dressings. Generally, surgical ligation of the chylous fistula tract is recommended only if spontaneous closure fails to occur. In cirrhosis-induced chylous fistulas, a portacaval shunt often proves to be the ultimate correction. ^10,11 Regardless of the method used, resolution of chylous fistulas is imperative to prevent dehydration, metabolic derangement, coagulopathy, peripheral lymphocytopenia, and hypoalbuminemia. Our patient had elevated venous pressures in the neck; therefore, merely ligating the fistula without removing the SVC obstruction would have relieved the drainage only temporarily, until a new fistula developed. Furthermore, since the patient's chylous fistula likely developed as a result of the SVC obstruction, we surmised that removing the obstruction would correct both the fistula and the SVC syndrome. After the surgical repair, there was no pressure gradient between the head and neck vessels and the right atrium.

In nonacute SVC obstruction, which is most often the result of external compression by a malignant tumor, venous collaterals develop and mitigate the SVC syndrome. Moreover, because the patient's prognosis in the case of tumor-induced SVC obstruction is generally poor, palliative therapies, such as radiation, are often preferred. As a result, prospective studies of surgical correction of SVC obstruction have not been performed. Case reports have described venous bypasses in non-neoplastic SVC obstruction. ^13–15 These bypasses involve the insertion of a spiral graft between the innominate vein and the right atrium using a saphenous vein wrapped around a tubular stent. The procedure has been used in children who have developed SVC syndrome following the Mustard procedure for transposition of the great vessels. Use of the spiral vein graft was 1st reported in 1976. ¹⁶ A case series that was started about the same time, using the spiral vein graft in adults with benign SVC obstruction, ¹⁷ showed prolonged patency up to 15 years in 7 of 9 patients. A similar series ¹⁸ using spiral vein grafting or an expanded polytetrafluoroethylene (ePTFE) conduit included an 8-year-old patient with antithrombin III deficiency, in whom an ePTFE graft remained patent at the 2-year follow-up. A retrospective study ¹⁹ of 1,016 patients who underwent heart or heart-lung transplantation showed only 3 who developed SVC stenosis and obstruction; those patients underwent successful percutaneous treatment. In another series of 27 adult patients, the authors ²⁰ concluded that venous reconstruction was superior to single percutaneous transluminal angioplasty. However, repeated angioplasty narrowed the gap between the 2 methods.

Ward and colleagues ²¹ described the use of intravascular stents in 12 pediatric patients who had systemic venous and systemic venous baffle obstructions. To our knowledge, however, no pediatric series of SVC surgical reconstruction has been reported to date. In our patient, resection of the obstruction and an SVC end-to-end anastomosis was performed because of the distorted venous anatomy secondary to multiple CVC placement as discussed. Our case reminds medical practitioners of the complications of multiple CVC placement and, despite the death of our patient, demonstrates that successful surgical treatment of SVC obstruction can be achieved in very young patients.