Superior vena cava syndrome in a child with mediastinal mass: A case report

Abstract

Facial puffiness as a consequence of superior vena cava syndrome (SVCS) can be a presentation from which the physician would have to conjecture a diagnosis of a mediastinal mass including lymphomas. Pediatric SVCS can rapidly progress and pose a greater challenge for airway protection as was in our case.

Short abstract

Pediatric superior vena cava syndrome (SVCS) due to malignancy can be a rapidly progressive life‐threatening condition that leads physicians to decide between choosing either immediate therapeutic management or further diagnostic procedures considering the case at hand.

1. INTRODUCTION

Superior vena cava syndrome (SVCS) results from the compression of the superior vena cava (SVC) which drains the head, neck, upper limbs, and upper part of the chest and torso, including the viscera above the diaphragm. Malignancy, hematological causes (inc. thrombosis), and congenital heart diseases are the majority of etiological factors in the pediatric population. ¹ The former two are of similar etiology to their adult counterparts. ² The increasing use of intravascular devices such as catheters and pacemakers has increased the risk of thrombosis as a cause. ² Lymphoma (most commonly non‐Hodgkin lymphoma (NHL)) remains the commonest of the oncological etiologies. ¹ , ³ , ⁴ , ⁵ The median age of presentation was 4.75 years with bimodal distribution; the first peak at infancy and later in adolescence. ¹ Here, we present a case of an 8‐year‐old girl who presented with superior vena cava syndrome secondary to a mediastinal mass.

2. CASE PRESENTATION

A six‐year‐old child presented to our outpatient department with complaints of facial puffiness and a feeling of lethargy. She additionally reported shortness of breath and cough for the last 10 days. On examination, there was marked facial edema, and prominent veins were detected on her upper chest (Figures 1 and 2). Breath sounds were decreased in the right supra mammary and mammary areas, but the heart sounds were normal. There was no distention of her abdomen nor the presence of edema in her lower limbs. Her oxygen saturation was also recorded to be normal. On further inquiry, it was found that she was managed as a case of nephrotic syndrome for a month before she was referred to our center. However, the report of urine analysis in our center was normal and without proteinuria. Suspecting a diagnosis of superior vena cava syndrome, a chest X‐ray was ordered which showed a widened mediastinum (Figure 3). A diagnosis of a mediastinal pathology compressing the superior vena cava was made, and a prompt referral to a tertiary care center was done. Steroids were stopped, which she had been prescribed as a part of management for suspected nephrotic syndrome.

FIGURE 1.

Photograph showing prominent facial puffiness and edema

FIGURE 2.

Photograph showing prominent chest veins

FIGURE 3.

Initial X‐ray showing widened mediastinum (solid white arrow)

By the time, she reached the tertiary care center, her shortness of breath had increased (respiratory rate: 34) and her blood pressure had decreased. After initial fluid resuscitation and stabilization, she was admitted for further investigations and management. Her laboratory results showed a hemoglobin level of 11.6 gm% and an LDH level of 1580 U/L. A subsequent computed tomography scan of her chest, abdomen, and pelvis revealed the presence of a large mediastinal mass with infiltration into the right pleura with encasement of mediastinal vessels with multiple renal space‐occupying lesions and peripancreatic nodules suggestive of a diagnosis of lymphoma. Additionally, moderate right‐sided pleural effusion and partial collapse of the right middle lobe and the right upper lobe along with a mild pericardial effusion were also seen. Given the urgency of the situation with the risk of imminent respiratory collapse, chemotherapy with cyclophosphamide, vincristine, and prednisolone was started without a definitive diagnosis via a biopsy. An X‐ray after 8 days of the initial chemotherapy regimen showed a striking shrinkage in her mediastinal mass (Figure 4). This shrinkage did not allow a biopsy to be taken from the mass itself. However, a bone marrow aspiration and biopsy were done which were reported as negative for malignancy. She showed marked improvement in her symptoms and was finally discharged after 2 weeks of hospitalization. She was asked to come for a monthly follow‐up to complete her 4 cycles of chemotherapy.

FIGURE 4.

Change in the mediastinal shadow (solid white arrows) following chemotherapy as shown in the two X‐rays taken 11 days apart (A: before chemotherapy. B: after chemotherapy).

3. DISCUSSION

SVCS obstructs blood flow leading to increased venous pressure along its tributaries. It can be of devastating consequence if associated with airway edema. ⁶ Children are more susceptible than their adult counterparts due to narrower lumen, greater compressibility of the upper airway, and greater edema at onset. ⁷ Management of airways in such a case presents a challenge, as even endotracheal intubation may not guarantee ventilation. ⁸ , ⁹ , ¹⁰ In addition to this, the use of sedatives and anesthetics during the process may decrease the pharyngeal tone, which further compromises the upper airway. ⁹ One should be aware of such a consequence to avoid a fatal catastrophe. Similarly, distal airway compression can also lead to absorption atelectasis leading to the collapse of a segment of the lungs, as was in our case.

In the case of lymphoma, a tissue diagnosis is required to characterize the tumor and define the optimal treatment. For this, a core needle biopsy is usually done. Multiple attempts may be needed to define specific characteristics based on architectural and immuno‐histochemical evaluation. ¹¹ Cervical mediastinoscopy and anterior mediastinoscopy may increase diagnostic sensitivity but carries the added risks of bleeding and airway compromise. ¹² A pathological diagnosis could not be made in our case due to the urgency of the situation, but whenever possible, it is desirable to obtain an adequate tissue specimen to formulate appropriate and effective therapy. It is also important to note that children with SVCS often tolerate the necessary procedures poorly. ¹³ Because of the risks of anesthesia in a patient with airway compromise and embarrassed venous return, as exemplified in our case, empiric treatment may be necessary before a definitive diagnosis is established, to avoid hemodynamic and ventilator destabilization. ¹⁴ The response to chemotherapy was striking which further supported our working diagnosis. LDH which is commonly elevated in lymphoproliferative disorders has prognostic significance and can also be used to monitor treatment response and recurrence of the disease. ¹⁵ , ¹⁶ If the mass were to reoccur, a consensus was made to take a biopsy at that time in the future.

Both Hodgkins lymphoma and non Hodgkins lymphoma have an excellent 5‐year survival of 89.1% and 73.8%, respectively. ¹⁷ , ¹⁸ Treatment should be approached as a near‐curable disease. Literature suggests that in cases of SVCS caused by lymphoma, chemotherapy can be as effective as radiotherapy. ¹³ In accordance with this, our patient received Cyclophosphamide, Prednisolone, and Vincristine with rapid clinical improvement. Radiotherapy was not used in our case.

4. CONCLUSION

In summary, a diagnosis of SVC requires vigilance on the part of the physician, as symptoms may be as trivial as facial swelling alone. It demands high clinical suspicion and conscientious examination. Malignancy, that is, NHL is the commonest of the causes outside infancy. Emergency resuscitation and securing the airway in itself pose a challenge to reducing unfortunate outcomes of a near‐curable pathology. Chemotherapy can lead to rapid resolution of symptoms. Hence, this further emphasizes the need for early diagnosis and treatment for better long‐term outcome.

AUTHOR CONTRIBUTIONS

Ashes Rijal wrote the original manuscript, reviewed and edited the manuscript. Anish Kumar Shrestha wrote the original manuscript, reviewed and edited the manuscript. Sharmila Chaudhary obtained the information, reviewed and edited the manuscript. Anisha Shrestha reviewed and edited the manuscript.

CONFLICT OF INTEREST

The authors have no conflict of interest to declare.

CONSENT

Written informed consent was obtained from the patient for publication of the case report and accompanying images. A copy of the written consent is available for review by the editor in chief of this journal on request.

Rijal A, Shrestha A, Chaudhary S, Shrestha A. Superior vena cava syndrome in a child with mediastinal mass: A case report. Clin Case Rep. 2022;10:e06647. doi: 10.1002/ccr3.6647

DATA AVAILABILITY STATEMENT

All the required information is available in the manuscript itself.