Large Congenital Facial Teratoma Associated With Neurological Migration Disorder (Polymicrogyria) and Absent Corpus Callosum.

BACKGROUND: Herein, the author report a severe case of large facial mixed germ line tumor associated with neuronal migration disorder, polymicrogyria, and corpus callosum agenesis. This specific association has not been reported as yet. METHOD: A 33-year-old primiparous woman at 38th week, delivered 3820 g male baby, via Cesarian section. Postpartum profuse bleeding was noticed from a large, ulcerative, 7 cm exophytic left facial mass, which was controlled nonoperatively. The treatment included surgical excision and 560 g of large facial tumor. Histology results revealed mixed germ line tumor with grade 3, immature teratoma. RESULTS: Four months postoperatively the teratoma recurred in the buccal, tonsillar, and parapharyngeal areas. Fourteen months following the initial surgical excision the patient died from airway obstruction. CONCLUSION: Due to poor initial prognosis further treatment of the facial malignancy was not feasible. However, initial, early postnatal, excisional surgery provided a prolonged and better quality of life for the patient and family.

Does patient ethnicity affect site of craniosynostosis?

OBJECT: There are no published papers examining the role of ethnicity on suture involvement in nonsyndromic craniosynostosis. The authors sought to examine whether there is a significant difference in the epidemiological pattern of suture(s) affected between different ethnic groups attending a regional craniofacial clinic with a diagnosis of nonsyndromic craniosynostosis. METHODS: A 5-year retrospective case-notes analysis of all cases involving patients attending a regional craniofacial clinic was undertaken. Cases were coded for the patients' declared ethnicity, suture(s) affected by synostosis, and the decision whether to have surgical correction of synostosis. The chi-square test was used to determine whether there were any differences in site of suture affected between ethnic groups. RESULTS: A total of 312 cases were identified. Of these 312 cases, ethnicity data were available for 296 cases (95%). The patient population was dominated by 2 ethnic groups: white patients (222 cases) and Asian patients (56 cases). There were both more cases of complex synostosis and fewer cases of sagittal synostosis than expected in the Asian patient cohort (χ(2) = 9.217, p = 0.027). CONCLUSIONS: There is a statistically significant difference in the prevalence of the various sutures affected within the nonsyndromic craniosynostosis patient cohort when Asian patients are compared with white patients. The data from this study also suggest that nonsyndromic craniosynostosis is more prevalent in the Asian community than in the white community, although there may be inaccuracies in the estimates of the background population data. A larger-scale, multinational analysis is needed to further evaluate the relationship between ethnicity and nonsyndromic craniosynostosis.

Facial paediatric desmoid fibromatosis: a case series, literature review and management algorithm.

Desmoid fibromatosis (also known as infantile or aggressive fibromatosis) is a rare soft tissue tumour that is occasionally seen in children. Although histologically benign, its growth pattern is highly aggressive often showing invasion of surrounding musculature and bone. Frequently found in cosmetically sensitive areas, complete excision can present a challenging problem. However, incomplete surgical excision is associated with high recurrence rates and although the disease responds to chemo and radiotherapy, both carry significant risks in young children. The management of four paediatric desmoid fibromatoses occurring in the midface is discussed. The recent and pertinent literature is comprehensively reviewed and an algorithm for the management of paediatric desmoid fibromatoses is proposed.

Surgical correction of midline nasal dermoid sinus cysts.

Nasal dermoid sinus cysts (NDSCs) are rare congenital anomalies affecting approximately 1 in 30,000 live births. Nasal dermoid sinus cysts are unsightly, prone to infection, and, importantly, may communicate with the central nervous system. Treatment is complete surgical excision. This study retrospectively evaluated management of a large single-center cohort of intracranial NDSCs.Nineteen patients with NDSC were identified from all patients presenting to the Leeds craniofacial service between June 2000 and August 2008. Patient demographics, clinical presentation, preoperative investigations, and surgical procedures undertaken were analyzed.Mean age at presentation and surgery were 6.3 and 7.6 years, respectively. Fifty-three percent were males. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed in 15 and 17 patients, respectively. One patient (5.3%) required local excision only. Eighteen (96.7%) underwent a bicoronal approach, and 13 (68.4%) of these required a craniotomy. The dura was opened in 7 (36.8%) patients. Neither CT nor MRI predicted the presence or absence of intracranial extension in all patients. Positive and negative predictive values for intracranial extension were 85.7% and 50% for CT and were 100% and 50.0% for MRI. Mean follow-up of 4.1 years shows no deep recurrences and 5 (26.3%) were superficial nasal recurrences only.A multidisciplinary approach can achieve good results with infrequent intracranial recurrence. We used a bicoronal approach to facilitate craniotomy when required intraoperatively because imaging is unable to diagnose intracranial extension with sufficient accuracy.

Reconstruction of a severe open distal humerus fracture with complete loss of medial column by using a free fibular osteocutaneous graft.

OBJECTIVE: Open intra-articular fractures of the distal humerus can be associated with considerable bone loss and extensive soft tissue damage. The management of these injuries is quite challenging, and the restoration of elbow anatomy may require multiple bone and soft tissue surgical procedures. The purpose of this case report is to present the option of addressing at the same time a complex skin, muscular, and bone distal humerus defect by using a composite vascularized graft. METHODS: We present a case of a high-energy open fracture of the distal humerus in a polytrauma young patient. Apart from the significant damage of all skin layers and underlying muscle units at the posterolateral side of the elbow, the medial column of the distal humerus (6 cm in length) was completely absent. After patient resuscitation and wound debridement, a free vascularized osteocutaneous fibular graft was used for the reconstruction of the bone defect and the restoration of elbow anatomy. RESULTS: No complications were encountered during the postoperative period, and both bone and soft tissues progressed to sound healing. At 26 months follow-up, the patient had a functional and stable elbow and the Mayo Elbow Performance Score was 85 points, which is equivalent to a good result. CONCLUSIONS: Vascularized osteocutaneous fibular graft could effectively address complex traumatic defects of the elbow joint and enhance the potential for bone healing and early functional recovery.