O Klein1, E Simon2, L Coffinet3, A Joud4, C Ghetemme4, J-C Marchal4. 1. Service de neurochirurgie pédiatrique, hôpital d'Enfants, CHU de Nancy, université de Lorraine, 4, rue du Morvan, 54500 Vandœuvre-Lès-Nancy, France. Electronic address: oliklein@yahoo.com. 2. Service de chirurgie maxillo-faciale et plastique, CHU de Nancy, université de Lorraine, 54500 Nancy, France. 3. Service d'ORL, CHU de Nancy, université de Lorraine, 54500 Nancy, France. 4. Service de neurochirurgie pédiatrique, hôpital d'Enfants, CHU de Nancy, université de Lorraine, 4, rue du Morvan, 54500 Vandœuvre-Lès-Nancy, France.
Abstract
INTRODUCTION: Nasal dermal sinus in children (NDSC) is a rare malformation (1/20,000 to 1/40,000). Apart from local infection, they present as median nasal lump or pit on the dorsum and their diagnosis and treatment are often delayed. Consequences of untreated NDSC are: local infection, meningitis, and empyema, due to their frequent intracranial extension. PATIENTS AND METHODS: Six cases of NDSC were retrospectively reviewed (5 boys and one girl), all treated between 2006 and 2012 in our institution (Nancy University Hospital). All patients underwent a brain and facial CT-scan and MR imaging to check any bony lesions, skull base extension by foramen cæcum, course of the sinus and the possible associated brain malformations. Evolution, treatment and follow-up (FU) were conducted by the same multidisciplinary team (neurosurgeon, ENT surgeon, and plastic surgeon). Children were operated on by a conjoined approach (cranial and facial) for removal of the sinus and its intracranial extension. RESULTS: Mean age at diagnosis was 12 months (birth-36 months). Initial presentation consisted of three local infections, one dorsum nasal lump, one CSF leakage, and one asymptomatic child. Five children presented with a skull base extension. There were no associated brain malformations. We observed only one surgical complication (bleeding from the anterior part of the superior sagittal sinus during dissection) leading to blood transfusion. Pathology results confirmed three dermoid cysts, one epidermoid cyst, one cyst with granulation tissue, and negative in one case. Average FU was 30.8 months (4-84 months). Two recurrences (same child) occurred, leading to two re-operations. There were no recurrences or complications at the end of FU. CONCLUSION: NDSC are rare malformations, mostly diagnosed before the age of three years, due to an infectious complication. The aim of the treatment is complete removal to avoid recurrence, and a multidisciplinary strategy is required.
INTRODUCTION: Nasal dermal sinus in children (NDSC) is a rare malformation (1/20,000 to 1/40,000). Apart from local infection, they present as median nasal lump or pit on the dorsum and their diagnosis and treatment are often delayed. Consequences of untreated NDSC are: local infection, meningitis, and empyema, due to their frequent intracranial extension. PATIENTS AND METHODS: Six cases of NDSC were retrospectively reviewed (5 boys and one girl), all treated between 2006 and 2012 in our institution (Nancy University Hospital). All patients underwent a brain and facial CT-scan and MR imaging to check any bony lesions, skull base extension by foramen cæcum, course of the sinus and the possible associated brain malformations. Evolution, treatment and follow-up (FU) were conducted by the same multidisciplinary team (neurosurgeon, ENT surgeon, and plastic surgeon). Children were operated on by a conjoined approach (cranial and facial) for removal of the sinus and its intracranial extension. RESULTS: Mean age at diagnosis was 12 months (birth-36 months). Initial presentation consisted of three local infections, one dorsum nasal lump, one CSF leakage, and one asymptomatic child. Five children presented with a skull base extension. There were no associated brain malformations. We observed only one surgical complication (bleeding from the anterior part of the superior sagittal sinus during dissection) leading to blood transfusion. Pathology results confirmed three dermoid cysts, one epidermoid cyst, one cyst with granulation tissue, and negative in one case. Average FU was 30.8 months (4-84 months). Two recurrences (same child) occurred, leading to two re-operations. There were no recurrences or complications at the end of FU. CONCLUSION: NDSC are rare malformations, mostly diagnosed before the age of three years, due to an infectious complication. The aim of the treatment is complete removal to avoid recurrence, and a multidisciplinary strategy is required.