Sclerotherapy of abdominal lymphangioma: An effective treatment modality

Abstract

Abdominal lymphangioma is rare in children. The etiopathogenesis and the treatment modalities to be used are not clearly defined. We report a case of a 4-year-old male patient presenting with abdominal pain. On examination, there was a mass in the left flank. CT showed an intraperitoneal multiloculated cystic lesion. The patient was given intralesional bleomycin therapy and followed up, there was no recurrence.

Abdominal malformations should be considered for cases with gradually enlarging abdominal lumps. Treatment with intralesional bleomycin yields excellent outcomes and should be preferred in cases where complete surgical resection is not feasible.

Introduction

Lymphangiomas are a rare congenital lymphatic malformation which can occur anywhere from cervico facial region (75%) to abdomen (5%). They originate due to sequestration of lymphatic tissue which then fail to communicate with rest of the lymphatic system.¹

Our case merits a discussion due to the rarity of the site and the age group at which it was presented, as often abdominal lymphangiomas present in adolescence to early adulthood.

Case report

A previously healthy four-year-old male patient was brought by his mother to the pediatric emergency with a two-and-a-half-year history of abdominal distension. The distention was non-progressive for 2.5 years. No other significant history was noted. The child patient was being managed at a rural center. The ultrasound abdomen performed a year, and a half back was suggestive of gross ascites. He had also undergone ascitic fluid tapping from the abdomen and a Fine Needle Aspiration Cytology (FNAC) from a level II lymph node of the neck. The examination of the ascitic fluid and the lymph node biopsy was inconclusive. The patient was started on anti-tubercular therapy, empirically. Mother noticed no improvement in abdominal distention and hence stopped the medications in five months. Subsequently, the patient underwent screening for abdominal tuberculosis again, which yielded negative results.

The patient was referred from another district hospital for further management. On examination, he was vitally stable. The general physical examination was normal. The abdomen was uniformly distended (Fig. 1). Plain abdominal radiograph suggested displacement of bowel loops by a soft tissue mass, which was insufficient to establish the diagnosis.

Fig. 1.

Uniformly distended abdomen.

Ultrasonography (USG) abdomen revealed a large, multiloculated echoic cystic lesion of size 9.9∗7∗8.8 cm noted in the left hypochondrium. No internal vascularities were noted. Further, Contrast-Enhanced Computed Tomography (CECT) of the abdomen (Fig. 2) revealed a large enhancing multiloculated cystic lesion measuring 23.2∗16.8∗12.4 cm (craniocaudal∗transverse∗anteroposterior) particularly in the left anterior peritoneal cavity, extending from T8 vertebral level superiorly to S4 vertebral level inferiorly. The lesion was displacing the bowel loops posteriorly, superiorly and to the right side. Anteriorly, it was abutting the anterior abdominal wall. Posteriorly, it was causing posterior displacement to the pancreas, descending colon and mesenteric vessel, insinuating in the lesser sac and encasing the splenic vessels. Inferiorly, the lesion indented onto the base of the bladder and abutted the sigmoid colon and rectum. No bowel obstruction was noted (Fig. 2).

Fig. 2.

Contrast-enhanced computed tomography (CECT) films showing the extent of the abdominal lump.

Under sedation, an intralesional Bleomycin Sclerotherapy procedure was performed. The cystic lesion was specifically targeted using an 18-gauge needle. The turbid serous content within the cyst was aspirated. A 10 Fr pigtail catheter was inserted. A solution of bleomycin with a concentration of 4.2 IU (0.3 IU/Kg) was injected into the largest cyst present on the left side using USG guidance. Afterwards, the pigtail was left in-situ and the patient was discharged with instructions to follow up after a month for another bleomycin injection. The pigtail catheter was secured in place. The histopathological examination of the fluid revealed lymphoid cells and pale eosinophilic fluid, indicating the presence of lymph. On follow-up, a repeat USG was done which recorded a decrease of the lesion to 7∗4.5∗4.0 cm. A total of three cycles of bleomycin instillation were performed over the course of three months, and the patient was followed up for a duration of two years. No complications were observed during the follow-up period (Fig. 3). Patient/guardian informed consent was obtained for inclusion in the study.

Fig. 3.

Post sclerotherapy picture at 2 years follow-up. The abdomen is scaphoid.

Discussion

Abdominal lymphangioma can be an incidental finding or can also present with an acute abdomen.^{2, 3, 4} Patient can have chronic non-specific abdominal signs like progressive abdominal distention and abdominal pain.^5,6 Our patient had abdominal distention which was same for 2.5 years. There was no history of abdominal pain, or any other bowel complaints. The abdominal distention was the concern of the mother, for which the patient was brought to us.

Other imaging modalities like USG and CECT are specific to diagnose such cases. A multicystic mass often with internal septations (as in our case) or a well-defined cystic mass is seen on USG.^{7, 8, 9}

In our case, the lesion measured 23.2∗16.8∗12.4 cm and displayed a multiloculated appearance, consistent with a cystic lymphangioma.

In a retrospective study done by Osnat et al, they suggested the indications for surgery (warning signs) in such cases, which were progressive gradual enlargement, multiplication of septa and increased echogenicity of the cystic fluid may.¹⁰

Complete surgical excision has been defined as the treatment of choice.^5,3,11 Complete excision of the entire lesion would have been challenging in cases, potentially leading to recurrence.¹²

Considering the pediatric age group, invasive surgical interventions are usually kept as secondary treatment modality in such cases. The management of this patient was being done during the pandemic, so we took into consideration a few other points also like traveling constraints for patient, restrictions on major elective procedures as the country being on high alert because of the spread of covid, to name a few.

The current primary treatment modality for macrocystic and combined lymphatic malformations (LM) is percutaneous sclerotherapy¹³ and the same was followed in our case. In this case, there were no “warning signs” clinically, and sclerotherapy was chosen.

A retrospective observational study conducted in India has demonstrated that bleomycin is a safe, effective, and cost-efficient treatment option for managing cystic lymphangiomas. Although other agents like OK-432, sirolimus, and doxycycline can also be used but have more side effects than Bleomycin.^14,15

The patient underwent monthly follow-ups for three months for one year, followed by semi-annual check-ups for two years. There was no recurrence. The abdominal LM was completely resolved.

Conclusion

Abdominal LM are uncommon, but when they do occur, they can result in a significantly distended abdomen, as observed in our case. This article aims to highlight the importance of thorough clinical and radiological evaluation before determining the appropriate treatment approach. It is crucial to consider abdominal LM as a potential differential diagnosis for any progressively enlarging abdominal mass. In some cases, these malformations may invade vascular pedicles, making complete surgical resection unfeasible. Intralesional sclerotherapy is a highly effective treatment option, even for abdominal LM, and yields excellent outcomes.

Patients/ Guardians/ Participants consent

Patient/guardian informed consent was obtained for inclusion in the study.

Ethical clearance

Not applicable.

Source of support

None

Disclosure of competing interest

The authors have none to declare.