Proboscis lateralis with trans-ethmoidal meningocele and congenital nostril hypoplasia: a case report and literature review

Proboscis lateralis is an uncommon craniofacial malformation characterized by the presence of a rudimentary tubular nasal appendage originating from the medial canthal region. The degree of severity varies according to the extent of associated structural involvement, which may include ocular and nasal abnormalities, cleft lip and/or palate, and concurrent intracranial anomalies. Multidisciplinary approach is the mainstay of definite management. We report the case of a 4-month-old infant who presented with a 3-cm tubular lesion arising from the right medial canthus. The lesion contained a central opening with intermittent cerebrospinal fluid leakage, particularly during episodes of crying or straining. The patient was initially misdiagnosed with an ophthalmic disorder and underwent multiple referrals before definitive evaluation at our unit. Computed tomography revealed herniation of a portion of the right frontal lobe through a 2 × 1 cm bony defect into the preseptal region. The lesion was excised, and the fascial defect was repaired following plastic and reconstructive surgical principles. Postoperative recovery was uneventful, and the patient was discharged in stable condition 48 h later. Corrective management of the right hypoplastic nostril was deferred until late adolescence to address potential functional or cosmetic concerns.