Mark Liddington

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.

Free Vascularized Fibular Bone Graft

Autologous bone grafting for large segmental bone defects >6–8 cm, ±soft tissue cover, such as seen in posttraumatic (Fig. 21.1a–d) or postinfectious bone loss and tumor resection (Fig. 21.2a).

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 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.

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

Abstract 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.

Reconstruction of the irradiated perineum following extended abdomino-perineal excision for cancer: An algorithmic approach

Our unit has implemented an algorithm for irradiated perineal reconstruction incorporating current evidence and a new technique in line with the advent of laparoscopic tumour excision. Our approach attempts to maintain the benefits patients derive from minimally invasive oncological surgery. Four consecutive patients had uterine retroversion to obturate pelvic deadspace and reconstruct the posterior vaginal wall. Age range was 41-84 years and mean follow-up of 21 months with mean in-patient stay of 7 days. All patients had neoadjuvant radiotherapy or chemoradiation for low rectal/anorectal adenocarcinoma. All patients had laparoscopic Extended APER and contiguous posterior vaginal wall excision and reconstruction with uterine retroversion and z-plasty skin closure. One patient required ultrasound aspiration of a pre-sacral seroma at two months. No patients returned to theatre for major complications. We highlight one minor and no major complications associated with an algorithmic approach incorporating our method of uterine retroversion and z-plasty parallel to traditional flap reconstruction methods.

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.

AbstractOsteomyelitis 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.