CASE PRESENTATION: A 4 years old girl presented with history of recurrent haemoptysis. Bronchoscopic evaluation excluded a foreign body aspiration but revealed right bronchial mucosal hyperaemia and varices. Diagnosis of right unilateral PVA was suspected on transthoracic echocardiography which demonstrated hypoplastic right pulmonary artery and non-visualization of right pulmonary veins. Final diagnosis was confirmed on cardiac CT angiography. A conservative treatment approach was opted with consideration for pneumonectomy in future when she is older.
CONCLUSION: Rarer causes should be considered when investigating for recurrent haemoptysis in children. Bronchoscopy and cardiac imaging are useful tools to establish the diagnosis of unilateral PVA in our case.
RECENT FINDINGS: Transpetrygoid is the most utilized approach with modifications suggested to limit bone removal, exposure and preservation of the neurovascular structures as dictated by the extent of the lateral recess. As more experience is gained, extended transphenoidal techniques were also successfully used for access. Lateral transorbital is a new approach to the lateral recess investigated in cadavers. IIH treatment is still controversial in the setting of SSLRE, but it appears rationale to evaluate, monitor and treat if necessary.
SUMMARY: SSLRE management should be tailored to the specific anatomical variances and cause. Modifications of techniques have been described giving different options to access the lateral recess. Successful repair for spontaneous SSLRE may require treatment of IIH if present, but the long-term outcome is still unclear.
METHOD: This was a retrospective clinical record review study carried out at a tertiary centre from June 2013 until May 2017. A total of 55 locally recurrent NPC patients (rT1-rT4) underwent EETN performed by single skull base surgeon with curative intention with postoperative adjuvant chemotherapy but without postoperative radiotherapy.
RESULTS: There were 44 (80.0%) males and 11 (20.0%) females, with mean age of 52.5 years. The mean operating time was 180 min (range 150-280 min). 85% (47/55) of patients achieved en bloc tumour resection. 93% (51/55) of patients obtained negative microscopic margin based on postoperative histopathological evaluation. Intraoperatively, one (1.8%) patient had internal carotid artery injury which was successfully stented and had recovered fully without neurological deficit. There were no major postoperative complications reported. During a mean follow-up period of 18-month (range 12-48 months) postsurgery, five patients (9.1%) had residual or recurrence at the primary site. All five patients underwent re-surgery. One patient at rT3 passed away 6 months after re-surgery due to distant metastasis complicated with septicaemia. The 1-year local disease-free rate was 93% and the 1-year overall survival rate was 98%.
CONCLUSIONS: EETN is emerging treatment options for locally recurrent NPC, with relatively low morbidity and encouraging short-term outcome. Long-term outcome is yet to be determined with longer follow-up and bigger cohort study. However, a successful surgical outcome required a very experienced team and highly specialised equipment.
SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12070-023-03625-4.
SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12070-022-03347-z.