ABSTRACT
AIMS AND OBJECTIVES: This study aimed to analyze the spectrum and surgical outcome of cases of craniosynostosis operated at a tertiary referral institute in India. DESIGN: This was a cross-sectional study. MATERIALS AND METHODS: We retrospectively examined 60 cases of craniosynostosis operated at our institute from 2008 to 2014 (with a minimum follow-up of 2 years). Data was collected including name, age, gender, involved sutures, other medical conditions, whether syndromic craniosynostosis or not, whether symptoms and signs of intracranial hypertension were present or not, associated findings on magnetic resonance imaging of brain and cervico-medullary junction, type of surgery performed, age at which surgery was performed, perioperative complications (if any), and findings on follow-up. To be able to analyze the surgical results, we used the seven category classification system used by Sloan et al. RESULTS: Craniosynostosis affected more men than women. The incidence of syndromic craniosynostosis was 11.67%. Mean age at first surgery was 3.85 years. Chiari malformation was present in 80% of the Crouzon's syndrome cases, 62.5% of the oxycephaly cases, and 4.44% of the non-syndromic, non-oxycephaly cases. Intracranial hypertension was present in 80% of the Crouzon's syndrome cases, 75% of the oxycephaly cases, and 6.67% of the non-syndromic, non-oxycephaly cases. Perioperative complications were present in 42.86% of the syndromic craniosynostosis cases, 50% of the oxycephaly cases, and 15.56% of the non-syndromic, non-oxycephaly cases. Compromised overall correction was present in 4 of 7 cases of syndromic craniosynostosis, 3 of 8 cases of oxycephaly, and 2 of 45 cases of non-syndromic, non-oxycephaly group. CONCLUSION: The study highlights the importance of educating the masses so that cases of craniosynostosis present early. The incidence of Chiari malformation, intracranial hypertension, and perioperative complications was significantly higher in the syndromic craniosynostosis and oxycephaly groups than in single-suture craniosynostosis. The best surgical outcome and the least perioperative complications were seen in the trigonocephaly group. Compromised overall correction and reoperations were more common in the syndromic and complex craniosynostosis groups than in single-suture craniosynostosis.
ABSTRACT
BACKGROUND: Accessory breast, also known as supernumerary breasts, polymastia, or mammae erraticae, is a clinical condition of having an additional breast. Accessory breasts are usually seen along the embryonic milk line, with the majority located in the axilla. Polythelia is the presence of an additional nipple. We report a rare case of dorsal accessory ectopic breast with three nipples (two well formed and one rudimentary) occurring along with lipomeningomyelocele and diastematomyelia. CASE DESCRIPTION: We report the case of an 18-year-old female who presented with chief complaints of swelling over the upper back since birth and spastic weakness of bilateral lower limbs with inability to walk since 2 years. Three-dimensional computed tomography scan of the dorsal spine was suggestive of a wide bony defect in the posterior spinal elements from D3 to D9 vertebrae. Diastematomyelia was also seen. Magnetic resonance imaging of the dorsal spine was suggestive of a complex spinal dysraphism with lipomeningomyelocele and diastematomyelia. During surgery, the patient's accessory breast was removed, lipomatous tissue and bony septum were excised, and dural repair was done. Histopathological examination was consistent with accessory ectopic breast with lipomeningomyelocele. CONCLUSION: Dorsal accessory breast, although a rare entity, whenever present should alert the clinician regarding the possibility of an underlying occult spinal dysraphism (OSD). Therefore, dorsal accessory breast can also be considered as a marker of OSD.
ABSTRACT
BACKGROUND: Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Mixed olfactory neuroblastoma which contains areas of divergent differentiation is even rare. Till date, only 4 cases of mixed olfactory neuroblastomas have been reported. CASE DESCRIPTION: We report the case of a 46-year-old male who presented with the chief complaints of nasal bleeding and nasal obstruction since 4 months. Radiological imaging was suggestive of a large heterogeneous mass in the left superior nasal cavity with extensions into bilateral maxillary, ethmoidal, and sphenoidal sinuses, as well as into the anterior cranial fossa. Bifrontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities and paranasal sinuses from below was done. Postoperative radiological imaging was suggestive of gross complete excision of the mass. Histopathological diagnosis was "mixed olfactory neuroblastoma-carcinoma (squamous and glandular differentiation) Hyams grade IV." On immunohistochemistry, the tumor cells were positive for neuron specific enolase (NSE), synaptophysin, chromogranin, and CD56 and peripherally for S100. Because of personal reasons, the patient did not take adjuvant radiotherapy. He presented again after 2 months with a full blown recurrence of esthesioneuroblastoma with similar extensions as before. The patient is now planned for salvage surgery followed by adjuvant chemoradiation. CONCLUSION: We report the 5th case in the world of mixed olfactory neuroblastoma-carcinoma with squamous and glandular differentiation. From an analysis of the findings in the 5 reported cases of mixed olfactory neuroblastomas, one might infer that a separate subcategory of ONB, i.e., mixed ONB, should be considered because mixed ONBs have an aggressive behavior, high rates of recurrence, and these tumors should be treated aggressively by multimodality treatment.
