Posterior Cranial Vault Distraction Osteogenesis Utilizing a Posterior-Superior Distraction Vector in the Treatment of Mercedes Benz Pattern Craniosynostosis.

ABSTRACT: Bilateral lambdoid and sagittal synostosis, or Mercedes Benz Syndrome, is a rare complex craniosynostosis resulting in frontal bossing, a tapered posterior fossa, and an anteriorly displaced cranial vertex. Its ideal surgical correction must result in posterior expansion, skull elongation, and caudal repositioning of the vertex. We present a craniometric analysis of skull changes with posterior-superior distraction and introduce a novel craniometric measure: vertex position. In this study, a retrospective review was performed to analyze outcomes of posterior cranial vault distraction osteogenesis (PVDO) using a posterior-superior distraction vector from 2016 to 2019. Cranial vertex position was measured as a fraction of the occipitofrontal diameter from rostral to caudal (0-1.0). Four patients underwent PVDO at mean age 10.61 ±â€Š3.16 months utilizing a posterior-superior distraction vector. Linear distraction distance averaged 30.30 ±â€Š0.90 mm with a mean consolidation period of 3.98 ±â€Š0.72 months. Mean corrected change in intra-cranial volume was 236.30 ±â€Š3.71 mL, at an average rate of 7.81 ±â€Š2.00 mL/mm of distraction. Increases in anterior cranial height (7.83 ±â€Š2.51 mm), middle cranial height (8.43 ±â€Š4.21 mm), posterior cranial height (13.15 ±â€Š7.45 mm), and posterior cranial fossa height (21.99 ±â€Š8.55 mm) were observed. Cranial vertex demonstrated a mean posterior movement of 0.18 ±â€Š0.13. PVDO utilizing a posterior-superior distraction vector for management of nonsyndromic bilateral lambdoid and sagittal synostosis effectively increases intracranial volume and height and provides an esthetic outcome with posterior movement of the cranial vertex.

Decompressive Cranial Vault Remodeling in Osteosclerotic Robinow Syndrome.

We present a novel application of endocranial burr contouring for cranial vault expansion as a surgical adjunct during decompressive craniectomy in patients with cranial osteosclerosis. A 16-year-old female with osteosclerotic Robinow syndrome complicated by slit ventricle syndrome presented with refractory intracranial hypertension following external ventricular drain placement. Symptoms included severe headaches and altered mental status. Given the severe intracranial volume restriction secondary to massive calvarial thickening (2.5 cm), the patient was taken to the operating room for urgent surgical decompression. After frontal and parietal craniectomy, burr and osteotome contouring were used to remove two-thirds of the endocranial calvarial bone flap thickness resulting in a 9% cranial vault expansion while preserving an overall normal head size. There were no immediate postoperative complications. At over 3 years postoperatively, the patient had reduced headaches, maintained adequate shunt function, and has not required further vault reconstruction.

Pediatric Mandibular Condylar Fractures With Concomitant Cervical Spine Injury: A Treatment Protocol for Prevention of Temporomandibular Joint Ankylosis.

In pediatric patients with mandible fractures and concomitant cervical spine injury (CSI), treatment strategies may occasionally compete: condylar fractures require functional therapy with range of motion exercises of the temporomandibular joint (TMJ), while CSI requires neck immobilization. The authors report the case of a child presenting with bilateral condylar head fractures, a right parasymphyseal fracture, and concomitant CSI treated with cervical collar immobilization, who ultimately developed TMJ ankylosis. Here, the authors review mandibular condylar fracture management and suggest an approach for the pediatric patient presenting with condylar fractures and concomitant CSI requiring immobilization. To preserve range of motion at the TMJ, it is critical to prevent external restriction by prolonged cervical collar use. Instead, these patients benefit from a protocol utilizing conventional halo or noninvasive, pinless halo cervical spine stabilization. Without limitation of the TMJ, condylar fracture rehabilitation and healing may proceed unhindered.

An Intraoperative Salvage After Transection of the Greater Palatine Artery During Cleft Palate Repair: A Case for Buccal Fat and Buccal Myomucosal Flaps.

Recently, several adjunctive procedures have gained traction to aid cleft surgeons in repairing especially challenging palatal clefts. Buccal fat flaps and buccal myomucosal flaps have demonstrated particular utility in reinforcing thin palatal flaps or tissue deficits. Although their use has not been widely accepted, they may be particularly helpful in the setting of significant scarring or vascular compromise. Here the authors describe the case of an intraoperative salvage using bilateral buccal fat flaps and a right buccal myomucosal flap after transection of the right Greater Palatine artery (GPA) during palatoplasty on a 14-month old female with Pierre Robin Sequence and a wide Veau II cleft palate. For this operative salvage, bilateral buccal fat flaps were used to reinforce the hard-soft palate junction and a 4 cm × 2 cm flap of the right-sided buccal mucosa and buccinator muscle was inset along the majority of the right-sided soft and posterior hard palate. At 2 years follow-up, the patient had no significant complications and was doing well with healthy-appearing palatal tissue and age-appropriate speech.

The Safety and Efficacy of Dorsal Root Entry Zone Lesioning for Pain Management in Patients With Brachial Plexus Avulsion: A Systematic Review and Meta-Analysis.

BACKGROUND AND OBJECTIVES: Persistent neuropathic pain after brachial plexus avulsion (BPA) is common and generally nonresponsive to medical management. Dorsal root entry zone (DREZ) lesioning is the last resort for pain management in patients with BPA. This study aims to investigate and compare the outcomes and complications of DREZ procedures. METHODS: A systematic literature search was conducted to identify all related studies. Comparisons were based on the number of patients with preoperative pain vs postoperative pain, with the effect size calculated using the risk ratio. Mean visual analog scale (VAS) scores were extracted and analyzed between interventions. A meta-regression analysis was performed to identify risk factors for final outcomes. The rates of complications were also assessed and analyzed between interventions. RESULTS: A total of 30 studies with 917 patients (90.0% male and 10.0% female, mean age: 42.9 ± 16.6) were included in this systematic review. Of the 917 patients who underwent surgery, 655 (71.4%) patients had significant pain reduction at the last follow-up ( P < .05). The weighted mean preoperative VAS score was 8.3 ± 1.3, compared with postoperative VAS scores (1.9 ± 2.2); a significant improvement was observed ( P < .05). The subgroup analysis showed that microsurgical DREZotomy (MDT) is associated with better outcomes in terms of VAS score improvements compared with radiofrequency (RF)-assisted DREZ lesioning ( P < .05). Meta-analysis showed that the relative risk of motor deficits was significantly lower in the MDT group, compared with the RF-assisted group ( P < .05). Meta-regression showed that older age is correlated with an elevated risk of postoperative motor deficits compared with the incidence of sensory loss. CONCLUSION: DREZ lesioning is effective for intractable pain alleviation after BPA. Compared with RF-assisted DREZ lesioning, MDT is associated with better VAS score improvements and a lower rate of postoperative motor weakness.

Closed Incision Negative Pressure Therapy in Oncoplastic Surgery Prevents Delays to Adjuvant Therapy.

Breast reductions, including oncoplastic breast surgery (OBS), have high postoperative wound healing complication (WHC) rates, ranging from 17% to 63%, thus posing a potential delay in the onset of adjuvant therapy. Incision management with closed incision negative pressure therapy (ciNPT) effectively reduces postoperative complications in other indications. This retrospective analysis compares postoperative outcomes and delays in adjuvant therapy in patients who received ciNPT on the cancer breast versus standard of care (SOC) after oncoplastic breast reduction and mastopexy post lumpectomy. Methods: Patient demographics, ciNPT use, postoperative complication rates, and time to adjuvant therapy were analyzed from the records of 150 patients (ciNPT = 29, SOC = 121). Propensity score matching was used to match patients based on age, body mass index, diabetes, tobacco use, and prior breast surgery. Results: In the matched cohort, the overall complication rate of ciNPT-treated cancerous breasts was 10.3% (3/29) compared with 31% (9/29) in SOC-treated cancerous breasts (P = 0.096). Compared with the SOC-treated cancerous breasts, the ciNPT breasts had lower skin necrosis rates [1/29 (3.4%) versus 6/29 (20.7%); P = 0.091] and dehiscence rates [0/29 (0%) versus 8/29 (27.6%); P = 0.004]. In the unmatched cohort, the total number of ciNPT patients who had a delay in adjuvant therapy was lower compared to the SOC group (0% versus 22.5%, respectively; P = 0.007). Conclusion: Use of ciNPT following oncoplastic breast reduction effectively lowered postoperative wound healing complication rates and, most importantly, decreased delays to adjuvant therapy.

A Novel Frameshift Mutation in KAT6A Is Associated with Pancraniosynostosis.

De novo heterozygous mutations in the KAT6A gene give rise to a distinct intellectual disability syndrome, with features including speech delay, cardiac anomalies, craniofacial dysmorphisms, and craniosynostosis. Here, we reported a 16-year-old girl with a novel pathogenic variant of the KAT6A gene. She is the first case to possess pancraniosynostosis, a rare suture fusion pattern, affecting all her major cranial sutures. The diagnosis of KAT6A syndrome is established via recognition of its inherent phenotypic features and the utilization of whole exome sequencing. Thorough craniofacial evaluation is imperative, craniosynostosis may require operative intervention, the delay of which may be detrimental.

A Late Presentation of Hand Pain with Skin Changes.

A middle-aged homeless man presented with ongoing left-hand pain and worsening cutaneous phalangeal color changes. On examination, digital ischemia was observed with associated dry gangrene. Doppler examination showed impaired distal arterial flow. Angiography showed irregular digital perfusion. The patient later provided vital information to secure the diagnosis without further clinical workup. This quiz discusses the presentation, differential diagnosis, and treatment of a rare clinical scenario.