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.

Fronto-orbital advancement and reconstruction using reverse frontal bone graft without the use of orbital bar: a technical note.

INTRODUCTION: We describe our technique of using reverse frontal bone graft for FOAR for patients with metopic or coronal synostosis and present our complications using the Leeds classification system for complications in craniosynostosis surgery. METHODS: Since April 2015, seventeen patients have been operated using this technique. We perform a frontal bone graft that is then reversed, and supraorbital margins are drilled out. The orbital bar is then removed and drilled down to make bone dust and on-lay bone grafts which are then used to fill gaps on exposed dura and fill in around the temporal region. RESULTS: All 17 patients who underwent this technique have good cosmetic results. We report 5 (29%) complications and 8 (47%) blood transfusions (7 exposures, 1 cell salvage).

Paediatric spinal cord infarction-a review of the literature and two case reports.

Ischemic spinal cord infarction is rare in the paediatric population, and when it does occur, it is usually associated with traumatic injury. Other potential causes include congenital cardiovascular malformations, cerebellar herniation, thromboembolic disease and infection. Magnetic resonance imaging (MRI) findings can be subtle in the early evaluation of such patients. The outcome is variable and depends on the level and extent of the spinal cord infarct and subsequent rehabilitation. Here, we present two cases of ischemic spinal cord infarction in children.

Aspergillus Osteomyelitis of the Skull.

Osteomyelitis of the craniofacial skeleton is rare, with fungal pathogens least commonly implicated. The authors present 2 patients of osteomyelitis of the skull caused by Aspergillus spp. and discuss the diagnosis, clinicopathological course, and management strategies.Late recurrence seen in this type of infection warrants long-term follow-up and a high index of suspicion for the clinical signs associated with recurrence.Such patients would benefit from their surgical debridement being planned and managed via a specialist craniofacial unit, so as to utilize the most aesthetically sensitive approach and the experience of specialists from several surgical disciplines.

Inner table corticectomy of the fronto-orbital bar in correction of metopic and coronal craniosynostoses.

Fronto-orbital advancement is an established method for correction of metopic and coronal craniosynostoses. Many techniques involve creation of a single fronto-orbital bar that is then shaped with osteotomies with or without bone grafting. We present a technique that minimizes osteotomy of the frontal bar and gives superior lateral brow aesthetics.Standard fronto-orbital bar bone cuts are made without a midline osteotomy. Selective inner table corticectomy of the fronto-orbital bar allows the bone to become malleable without greensticking. The need for osteotomy of the fronto-orbital bar is obviated. An additional bandeau is created from the temporoparietal calvaria. The malleable fronto-orbital bar is then fixed to this bandeau. The frontal bar and bandeau complex is then advanced in a conventional manner. The remaining frontal calvaria is then rotated creating a more vertical forehead.This technique has been used in Leeds for more than 10 years with good cosmetic results. It has become our standard method for management of the fronto-orbital bar in correction of nonsyndromic metopic and coronal craniosynostoses.

Frontal sinus mucocele in association with fibrous dysplasia: review and report of two cases.

We present two paediatric cases of fibrous dysplasia (FD) who presented to the craniofacial neurosurgical clinic with ophthalmological symptoms associated with sinus mucoceles. The first patient presented with a history of orbital cellulitis and an increasing bony swelling around the orbit associated with proptosis. Radiological imaging revealed monostotic FD associated with an obstructive mucocele in the frontal sinus with extension into the orbit. The second patient presented with recurrent conjunctivitis, painful proptosis, rhinitis and a bony peri-orbital swelling. Both patients had histological diagnoses of frontal mucoceles invading the orbit in association with FD. They both underwent frontal craniotomies and excision of the mucocele/fibrous dysplastic complex. In summary, mucocele development is an unusual complication of FD, likely to occur secondary to occlusion of the sinus drainage system. Orbital involvement may lead to visual disturbance caused by pressure effects. A multi-disciplinary approach including maxillofacial surgeons, plastic surgeons and neurosurgeons is advocated.

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.

A proposal for a new classification of complications in craniosynostosis surgery.

OBJECTIVE Complications have been used extensively to facilitate evaluation of craniosynostosis practice. However, description of complications tends to be nonstandardized, making comparison difficult. The authors propose a new pragmatic classification of complications that relies on prospective data collection, is geared to capture significant morbidity as well as any "near misses" in a systematic fashion, and can be used as a quality improvement tool. METHODS Data on complications for all patients undergoing surgery for nonsyndromic craniosynostosis between 2010 and 2015 were collected from a prospective craniofacial audit database maintained at the authors' institution. Information on comorbidities, details of surgery, and follow-up was extracted from medical records, anesthetic and operation charts, and electronic databases. Complications were defined as any unexpected event that resulted or could have resulted in a temporary or permanent damage to the child. RESULTS A total of 108 operations for the treatment of nonsyndromic craniosynostosis were performed in 103 patients during the 5-year study period. Complications were divided into 6 types: 0) perioperative occurrences; 1) inpatient complications; 2) outpatient complications not requiring readmission; 3) complications requiring readmission; 4) unexpected long-term deficit; and 5) mortality. These types were further subdivided according to the length of stay and time after discharge. The overall complication rate was found to be 35.9%. CONCLUSIONS The proportion of children with some sort of complication using the proposed definition was much higher than commonly reported, predominantly due to the inclusion of problems often dismissed as minor. The authors believe that these complications should be included in determining complication rates, as they will cause distress to families and may point to potential areas for improving a surgical service.

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.

Preoperative and postoperative developmental attainment in patients with sagittal synostosis: 5-year follow-up.

OBJECT: The object of this study was to clarify whether improved developmental attainment following surgical correction of sagittal synostosis (SS), previously identified at initial postoperative assessment, is maintained at longer-term follow-up at 5 years of age. METHODS: The study involved 32 children with SS who underwent corrective surgery at a mean (± SD) age of 8.5 ± 7.25 months (range 2.8-39.9 months). All the children were assessed preoperatively, at 7 months postoperatively, and at 5 years of age, using the Griffiths Mental Development Scales. A control group consisted of 23 children with SS who had received developmental assessment on 2 or more occasions without surgical intervention (8 of these children had had follow-up at 5 years of age). RESULTS: The data indicated that, prior to surgical correction, children with SS had poorer Gross Locomotor function than other areas of development and that, following surgical intervention, the deficit resolved (even where there was severe developmental delay). The results further showed that improvement in Gross Locomotor function observed at 7 months postoperatively was further improved upon by 5 years of age. The same was true for their overall General Quotient, even in those children exhibiting severe developmental delay. Lesser improvements across time were shown for other skill areas. The children with SS who did not undergo surgery did not show any improvement in development, and in fact a deterioration in fine locomotor control was identified in these patients. CONCLUSIONS: The results of this study suggest that corrective surgery for SS has a positive early impact on development, which is maintained and improved upon by 5 years of age, and that this surgery therefore offers more than simply a cosmetic improvement. Furthermore, the results suggest that not operating on children with SS means not only that this opportunity for developmental gain is missed, but that it may also cause an actual deterioration in developmental attainment.

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.

A rare diagnosis of dedifferentiated liposarcoma of the orbit.

We report the case of a 56-year-old female who presented to the ophthalmic casualty department with proptosis and diplopia. Initially, non-specific orbital inflammation was suspected while investigations were ongoing. A trial of steroids failed to resolve the proptosis to a significant extent. A CT scan showed a well-defined orbital mass, which on orbital biopsy was shown to be a primary orbital liposarcoma of the dedifferentiated variety. This was confirmed by expert histopathological analysis. An exenteration followed by removal of orbital bone was required to achieve total tumour removal. Radiotherapy was delivered to reduce the chance of micro-metastasis. Orbital liposarcoma is an extremely rare tumour. The dedifferentiated variety is even rarer, with only a few reported cases. Lack of information about this potentially aggressive tumour may create therapeutic dilemmas as to the best treatment approach. Similarly, prediction of an accurate prognosis for the patient may be difficult due to the rarity of this condition.