Clinical Features and the Use of Three-Dimensional Imaging for Unilateral Lambdoid Synostosis.

INTRODUCTION: True isolated lambdoid craniosynostosis is rare. It requires corrective surgery to prevent intracranial pressure and aesthetic stigma by significant dyscrania. We summarize our case series for lambdoid craniosynostosis outlining the pathophysiology, clinical findings and surgical approaches and outcomes. METHODOLOGY: A retrospective analysis of our data from 2010 to 2020 summarized our cases of true lambdoid synostosis. We have used the medical notes and the radiological findings from computed tomography scans to summarize a case series of isolated lambdoid synostosis. RESULTS: Our case series demonstrated 7 patients with true isolated lambdoid craniosynostosis. In most cases surgical intervention in the form of posterior cranial vault remodeling utilizing a bandeau, based on occipital advancement techniques, has demonstrated the most consistently favorable aesthetic outcome. CONCLUSIONS: Surgical referral to a craniofacial center should be sought early in difficult to diagnose cases. Although rare, surgical intervention is indicated to correct potentially increased intracranial pressure and to ameliorate cranial dyscrania.

FACE-Q Craniofacial Module: Part 1 validation of CLEFT-Q scales for use in children and young adults with facial conditions.

BACKGROUND: The CLEFT-Q includes 12 independently functioning scales that measure appearance (face, nose, nostrils, teeth, lips, jaws), health-related quality of life (psychological, social, school, speech distress), and speech function, and an eating/drinking checklist. Previous qualitative research revealed that the CLEFT-Q has content validity in noncleft craniofacial conditions. This study aimed to examine the psychometric performance of the CLEFT-Q in an international sample of patients with a broad range of facial conditions. METHODS: Data were collected between October 2016 and December 2019 from 2132 patients aged 8 to 29 years with noncleft facial conditions. Rasch measurement theory (RMT) analysis was used to examine Differential Item Function (DIF) by comparing the original CLEFT-Q sample and the new FACE-Q craniofacial sample. Reliability and validity of the scales in a combined cleft and craniofacial sample (n=4743) were examined. RESULTS: DIF was found for 23 CLEFT-Q items when the datasets for the two samples were compared. When items with DIF were split by sample, correlations between the original and split person locations showed that DIF had negligible impact on scale scoring (correlations ≥0.995). In the combined sample, RMT analysis led to the retention of original content for ten CLEFT-Q scales, modification of the Teeth scale, and the addition of an Eating/Drinking scale. Data obtained fit with the Rasch model for 11 scales (exception School, p=0.04). Person Separation Index and Cronbach alpha values met the criteria. CONCLUSION: The scales described in this study can be used to measure outcomes in children and young adults with cleft and noncleft craniofacial conditions.

Quantification of Head Shape and Cranioplasty Outcomes: Six-compartment Volume Method Applied to Sagittal Synostosis.

BACKGROUND: Premature fusion of the sagittal (midline) suture between 2 parietal bones is the most common form of craniosynostosis. Surgical correction is mandated to improve head shape and to decrease the risk of raised intracranial pressure. This study evaluated the utility of 3-dimensional (3D) imaging to quantify the volumetric changes of surgical correction. Currently there is no standardized method used to quantify the outcomes of surgery for craniosynostosis, with the cranial index (width: length ratio) being commonly used. METHODS: A method for quantification of head shape using 3D imaging is described in which the cranium is divided up into 6 compartments and the volumes of 6 compartments are quantified and analyzed. The method is size invariant, meaning that it can be used to assess the long-term postoperative outcomes of patients through growth. The method is applied to a cohort of sagittal synostosis patients and a normal cohort, and is used to follow up a smaller group of synostotic patients 1, 2, and 3 years postoperatively. RESULTS: Statistical analysis of the results shows that the 6-compartment volume quantification method is more accurate in separating normal from synostotic patient head shapes than the cranial index. CONCLUSIONS: Spring-mediated cranioplasty does not return head shape back to normal, but results in significant improvements in the first year following surgery compared with the preoperative sagittal synostosis head shape. 3D imaging can be a valuable tool in assessing the volumetric changes due to surgery and growth in craniosynstosis patients.

Auricular reconstruction.

External ear abnormalities are common. These may affect ear shape, size, prominence and degree of development. They may also be associated with hearing loss. The early identification and management of hearing loss is essential. There are several options for reconstruction of the external ear using both autologous and non-autologous techniques. The aim of this article is to outline the different reconstructive options.

Unilateral Pterional Polycraniosynostosis Treated with Craniectomy and Helmet Therapy.

Craniosynostosis is a condition in which one or more of the cranial sutures have fused prematurely, affecting the growth pattern and contours of the infant skull. The pterion is the junction of temporal, frontal, parietal, and sphenoid bones of the skull. We present a case of unilateral pterional craniosynostosis, which was treated with strip craniectomy and helmet therapy.

Frontoethmoidal mucocele following pediatric craniofacial surgery.

BACKGROUND: Mucoceles occur as a result of accumulation and retention of mucous secretions in a paranasal sinus and are uncommon in the pediatric age group. Persistent or intermittent closure of its ostium through a variety of causes, including previous surgery, is implicated in etiology. The authors report 2 cases of frontoethmoidal mucocele that followed box osteotomies for the treatment of orbital dystopia, with medical literature review and discussion of possible causal factors and events. METHODS: Case histories and radiological imaging are presented on 2 patients presenting with frontoethmoidal mucoceles following craniofacial surgery. Both had transcranial craniofacial techniques where all orbital walls and globe are moved en bloc as a "box." RESULTS: Patient 1, a 12-year-old male patient with Crouzon syndrome, developed mucoceles within 18 months of monobloc distraction surgery and box osteotomies. This was successfully marsupialized with a combined external and endoscopic surgical approach. The second patient, a 15-year-old boy with previously corrected right-sided facial cleft, developed mucocele 9 years following box osteotomies; this was successfully managed by endoscopic drainage. Of 3 other patients having similar box osteotomies in our unit, no other mucoceles were noted as complications. CONCLUSIONS: Mucoceles are a rare complication of craniofacial surgery, and literature review confirms a paucity of reports. Only 1 case has previously been alluded to of mucocele complicating box osteotomy for orbital dystopia. Our 2 cases illustrate and highlight a successful management approach in a multidisciplinary craniofacial unit.

Ocular advancement in monobloc distraction.

BACKGROUND: Monobloc distraction has been demonstrated to be an efficacious, safe, and stable method of providing functional and aesthetic improvement in children with syndromic craniosynostosis. Although the movement of bony structures following monobloc distraction has been quantitatively assessed before, objective analysis of the movement of soft tissues, such as the globe, has not been published previously. The authors present a method that assesses globe movement following monobloc distraction, using computed tomographic scan data. METHODS: The preoperative and postoperative computed tomographic scans of 10 patients with Pfeiffer's and Crouzon's syndromes, undergoing monobloc distraction, were assessed. Scan data were loaded into voxel image-rendering software that produced a three-dimensional reconstruction. Direct measurements between preoperative and postoperative images were taken. RESULTS: An average upper face osseous distraction distance of 12.94 mm, with an average lower face distraction of 14.19 mm, was achieved in all 10 children. The authors measured average globe movements on three planes and calculated an average left eye forward movement of 5.28 mm and average right eye forward movement of 6.33 mm. In all, globe advancement was approximately two fifths of the distance advanced by the surrounding bone. CONCLUSIONS: Forward movement of the globe following monobloc distraction has not previously been described. Although there is no current evidence for optic nerve stretch and compromise in monobloc surgery, the functional implications of these findings should be formally assessed.

Unilateral isolated frontosphenoidal craniosynostosis causing frontal plagiocephaly.

Synostotic frontal plagiocephaly is most commonly caused by frontoparietal (unicoronal) synostosis, but may be caused by other fusions along the coronal hemiring. Frontosphenoidal synostosis is a rare cause of frontal plagiocephaly, with only five previously reported cases. We present the case of an 8-month-old male infant with frontal plagiocephaly caused by unilateral isolated frontosphenoidal synostosis mimicking frontoparietal (unicoronal) synostosis. The clinical and radiological features included flattened ipsilateral forehead, retruded ipsilateral maxilla, a midline chin, contralateral deviation of the nasal root and endocranial base, and anomalies of the bony orbit. Unilateral isolated frontosphenoidal synostosis may be misdiagnosed as unicoronal synostosis. Careful physical and radiological assessment is necessary to recognise this synostotic disorder of the coronal hemiring.

Raised intracranial pressure in Apert syndrome.

BACKGROUND: Raised intracranial pressure is a well-known complication of Apert syndrome. The current policy in the authors' unit is to monitor these patients and only perform surgery when raised intracranial pressure has been diagnosed. The authors present their experience with this protocol, as it allows a more accurate picture of the natural history of raised intracranial pressure in Apert syndrome. METHODS: The records of 24 patients, aged between 7 and 14 years, with Apert syndrome who had been managed expectantly (i.e., with no routine "automatic" early surgery) were reviewed. Data were collected on the incidence, timing, and management of raised intracranial pressure. RESULTS: Twenty of 24 patients (83 percent) developed raised intracranial pressure. The average age of the first episode was 18 months (range, 1 month to 4 years 5 months). Raised intracranial pressure was managed with surgery in 18 patients, including two patients who underwent shunt procedures for hydrocephalus. Two patients had their raised intracranial pressure treated successfully by correcting coexisting upper airway obstruction alone. Seven of the 20 patients (35 percent) developed a second episode of raised intracranial pressure, on average 3 years 4 months later (range, 1 year 11 months to 5 years 9 months). CONCLUSIONS: In Apert syndrome, there is a high incidence of raised intracranial pressure, which can first occur at any age up to 5 years and may recur despite initial successful treatment. Causes of raised intracranial pressure include craniocerebral disproportion, venous hypertension, upper airway obstruction, and hydrocephalus. Careful clinical, ophthalmologic, respiratory, and radiologic monitoring will allow raised intracranial pressure to be diagnosed accurately when it occurs and then treated most appropriately.

Implications of a vertex bulge following modified strip craniectomy for sagittal synostosis.

BACKGROUND: Modified strip craniectomy is a common treatment for early isolated sagittal synostosis. The authors assessed the significance of the development of a progressive vertex bulge following strip craniectomy as a predictor of raised intracranial pressure or multiple suture synostosis. METHODS: All cases of sagittal synostosis treated by modified strip craniectomy (removal of the sagittal suture with lateral barrel staving) at the authors' institution were reviewed. Eighty-nine patients with isolated sagittal synostosis were treated by modified strip craniectomy, usually before 6 months of age, between 1995 and 2005. Seven patients were noted to have developed a progressive vertex bulge. The vertex bulge was noted an average of 8 months postoperatively (range, 2 to 25 months). The clinical records of these seven patients were evaluated with regard to their clinical course, radiologic investigations, genetics testing, intracranial pressure monitoring, and the need for further surgery. RESULTS: Computed tomographic scanning demonstrated new synostosis involving other calvarial sutures in five patients. Five patients underwent intracranial pressure monitoring, and this was elevated in four patients. One patient required a ventriculoperitoneal shunt for hydrocephalus. All patients underwent genetic screening, and two were found to have fibroblast growth factor receptor (FGFR) mutations (one FGFR2 and one FGFR3 mutation). All patients required reoperation (calvarial remodeling) for either raised intracranial pressure, deteriorating head shape, or both. CONCLUSIONS: A progressive vertex bulge after modified strip craniectomy is a sign of possible raised intracranial pressure, the development of progressive multiple suture synostosis, or both. It is an indication for genetic testing for FGFR mutations.

Volumetric short scar rhytidectomy--indications, technique and outcomes.

BACKGROUND: Procedures combining a short scar with superficial musculoaponeurotic system (SMAS) manipulation are increasingly popular for patients with early signs of mid- and lower-facial laxity seeking rhytidectomy. We present the senior author's experience with a short scar volumetric malar imbrication rhytidectomy, which avoids post-auricular incisions and sub-SMAS dissection. PATIENTS AND METHODS: Between January 2004 and April 2007, 54 patients underwent a short scar volumetric rhytidectomy (9.6% of all facelifts). These procedures were primary in 38 and secondary in 16 patients, at a mean age of 49 years (range 35-77 years). Average operating time was 90 min. Resultant vertical and horizontal skin movement at the helical root was recorded. Concurrent procedures included blepharoplasty, canthoplasty, endoscopic forehead rejuvenation and fat grafting. Minimum follow up was 3 months. Pre- and 3 month postoperative photographs of 25 randomly selected patients were rated by three independent surgeons. A seven-point scale was used to grade the improvement in the malar eminence, melolabial fold, jowls and cervicomental angle. The overall aesthetic result was assessed using the MDACS grading system. Statistical analysis was performed using Student's t-tests and general estimation equations where appropriate. RESULTS: There were no significant complications. Three patients developed minor cheek swellings which all settled with antibiotics. Mean postoperative aesthetic outcomes were rated as 'Good' using the MDACS scale (mean score 0.64), with no 'Poor' results. Vertical skin lifting was significantly greater than the horizontal skin lifting (P<0.001). Mild postoperative improvements were noted in the malar eminence soft tissue volume, nasolabial fold diminishment, jowl diminishment and cervicomental angle. CONCLUSION: In the appropriately selected face, short scar volumetric malar imbrication rhytidectomy is a straightforward, safe and effective procedure for improving the early signs of ageing.

Matrix metalloproteinases and their inhibitors in bone remodelling following distraction osteogenesis of the sheep mandible.

AIM: Matrix metalloproteinases (MMPs), together with their tissue inhibitors (TIMPs), are responsible for the controlled degradation of collagen and other matrix substrates in bone and other tissues. This study evaluated the expression of MMPs and TIMPs in bony remodelling in a bilateral sheep mandible model up to 12 months following lengthening by distraction osteogenesis. METHODOLOGY: Sheep mandibles were harvested 3, 6, 9 or 12 months following lengthening by bilateral mandibular distraction (1 mm/day for 20 days). Undistracted sheep mandibles were used as controls. The tissues underwent routine histology and immunohistochemical staining with monoclonal antibodies specific to MMPs 1-3 and TIMP-1, 2. Matrix and cell staining was assessed using a semi-quantitative analysis. RESULTS: Matrix metalloproteinases and their tissue inhibitors (TIMPs) expression levels were marked at 3 months and decreased thereafter becoming similar to undistracted controls by 12 months. The histologic development of mature lamellar cortical bone was similar to undistracted controls by 9 months following distraction. CONCLUSIONS: A temporal expression of MMPs and TIMPs was found in distraction osteogenesis. MMPs and TIMPS may, in part, reflect the state of bony remodelling following mandibular lengthening by distraction osteogenesis. Matrix metalloproteinases and TIMP expression were comparable to undistracted controls by 12 months, suggesting that equilibrium had been achieved and that bony relapse is unlikely.

Inguinal canal "lipoma".

Groin dissection was performed in adult male post-mortem subjects to establish the prevalence of inguinal canal "lipoma." Thirty-six body halves (age range 24-92 years) were studied. Of these, 27 (75%) contained a discrete mass of fat within the inguinal canal. This mass was always continuous with the preperitoneal fat through the deep inguinal ring. Nineteen of these 27 masses (70%), displayed a characteristic pedunculated form with a bulbous distal end. Eighteen of the 36 dissections (50%), revealed a mass more than 4 cm in length. Six dissections showed extension of the mass beyond the superficial inguinal ring and three of these six (8% of the 36 groins studied) showed distortion of the proximal spermatic cord with a mass at the superficial inguinal ring. The masses submitted for histology comprised mature adipose tissue and all but two of these were reported as having an adherent capsule. No significant correlation was found between mass length and either subject age or body mass index (BMI) but a statistically significant correlation between the length of the fat mass on the left and right sides was shown. This study demonstrates that the inguinal canal "lipoma" is a common feature in an adult male population and may be of sufficient size to cause clinical misdiagnosis. The high prevalence, characteristic location and appearance of the "lipoma" suggest a developmental etiology.

A compliant tip reduces the peak pressure of laparoscopic graspers.

BACKGROUND: Previous studies have demonstrated that high pressures are generated at the tips of laparoscopic graspers, which can cause tissue injury. This study examines the effect of a compliant edge on tip pressure. METHODS: One of a pair of identical laparoscopic graspers was modified by refashioning the tip out of silicone. A thin film pressure transducer (I-scan 6900 sensor) was deployed between the jaws of the grasper and a simulated tissue (leather strap). Load and handle pressures were kept constant. Peak tip pressure readings were taken as the load orientation was increased from the perpendicular to 135 degrees (n = 10). The data was analysed using anova and a post-hoc Duncan's multiple range test. RESULTS: Peak tip pressures generated by the compliant tipped grasper were significantly less than those developed by the unmodified grasper, particularly at high-load orientations. CONCLUSION: High pressures generated at the tip of laparoscopic graspers can be reduced by altering the mechanical properties (compliance) of the tip.