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Abdominal expansion using a polytetrafluoroethylene prosthesis in the treatment of Pepper syndrome
J Y Mabrut1, R Dubois, G Pelizzo
1Service de Chirurgie Pédiatrique, Hôpital Edouard Herriot, Lyon, France.
Insights
Stage IV-S neuroblastoma in infants can cause life-threatening compression. Surgical intervention with polytetrafluoroethylene (PTFE) prosthesis successfully relieved compression, enabling tumor regression and remission in two patients.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Infant Health
Background:
- Stage IV-S neuroblastoma is a rare, aggressive childhood cancer.
- Massive hepatomegaly can lead to critical thoracoabdominal compression in infants.
- Standard treatments like chemotherapy and radiotherapy may be insufficient.
Observation:
- Three infants with stage IV-S neuroblastoma presented with severe thoracoabdominal compression due to hepatomegaly.
- Despite chemo- and radiotherapy, urgent surgical intervention was required.
- A polytetrafluoroethylene (PTFE) prosthesis was used for abdominal expansion.
Findings:
- Surgical intervention successfully relieved the life-threatening thoracoabdominal compression.
- Tumor regression allowed for the removal of the PTFE prosthesis in all three patients.
- Two out of three infants achieved complete remission, with long-term follow-up of 32 and 38 months.
- One infant experienced tumor progression and died after 16 months.
Implications:
- Surgical management with PTFE prosthesis is a viable option for critical thoracoabdominal compression in infant neuroblastoma.
- This approach can facilitate tumor regression and improve survival outcomes.
- Further research into optimizing multimodal treatment strategies for stage IV-S neuroblastoma is warranted.
Abstract:
The authors report three cases of stage IV-S neuroblastoma in infants aged 4, 6, and 8 weeks, who despite chemo- and radiotherapy required surgical intervention to urgently relieve major thoracoabdominal compression secondary to massive hepatomegaly. The results were successful, with abdominal expansion being achieved by the introduction of a polytetrafluoroethylene prosthesis, which was removed during the 2nd, 3rd and 7th postoperative month, respectively, after tumor regression. Two children were in complete remission 32 and 38 months later, the 3rd died after 16 months of tumor progression.