Perioperative Comparison Between Open Cranial Vault Remodeling and Distraction Osteogenesis for Unilateral Lambdoid Craniosynostosis.

There are multiple treatment options for unilateral lambdoid craniosynostosis (ULS) including open posterior cranial vault remodeling (OCVR) and distraction osteogenesis (DO). There is a paucity of data comparing these techniques in the treatment of ULS. This study compared the perioperative characteristics of these interventions for patients with ULS. An IRB-approved chart review was performed from January 1999 to November 2018 at a single institution. Inclusion criteria included the diagnosis of ULS, treatment with either OCVR or DO using a posterior rotational flap technique, and a minimum 1-year follow-up. Seventeen patients met the inclusion criteria (12 OCVR and 5 DO). Patients in each cohort were found to have a similar distribution in sex, age at the time of surgery, synostosis laterality, weight, and length of follow-up. There was no significant difference in mean estimated blood loss/kg, surgical time, or transfusion requirements between cohorts. Distraction osteogenesis patients had a longer mean hospital length of stay (3.4 +/- 0.6 d versus 2.0 +/- 0.6 d, P = 0.0004). All patients were admitted to the surgical ward postoperatively. In the OCVR cohort, complications included 1 dural tear, 1 surgical site infection, and 2 reoperations. In the DO cohort, 1 patient had a distraction site infection, treated with antibiotics. There was no significant difference in estimated blood loss, volume of blood transfusion, or surgical time between OCVR and DO. Patients who underwent OCVR had a higher incidence of postoperative complications and the need for reoperation. This data provides insight into the perioperative differences between OCVR and DO in patients with ULS.

Delayed Presentation of Sagittal Suture Craniosynostosis.

BACKGROUND: Sagittal craniosynostosis typically presents as dolichocephaly or less frequently as clinocephaly, a "saddle-shaped" phenotype. This project aimed to characterize clinically relevant differences between sagittal synostosis phenotypes and examine the etiology of the delay in presentation. METHODS: An institutional review board-approved retrospective review was performed from January 1999 to November 2018 at a single institution. Analyses examined correlations between subphenotype, time of presentation, minor suture fusion, developmental delay, and operative technique. RESULTS: One hundred sixty patients diagnosed with single-suture sagittal craniosynostosis were identified. A total of 30.6% had a saddle phenotype (n = 49) and 69.4% had dolichocephaly (n = 111). Patients with the saddle phenotype were more likely to present with a developmental delay and to have at least 1 minor suture fused than patients with dolichocephaly were. Patients with the saddle phenotype presented for surgery at an older age and were more likely to undergo open cranial vault repair, with increased blood loss, higher transfusion volume, and longer time. CONCLUSIONS: This study highlights clinical differences in sagittal craniosynostosis phenotypes and shows that developmental delay is an initial presentation of the saddle phenotype. The saddle phenotype also correlated with fusion of the minor squamous and sphenoid sutures. The link between developmental delay and minor suture fusion was notable and should be explored with a larger sample size. Patients with saddle synostosis present for surgery at an older age than patients with dolichocephaly and therefore are more likely to receive open cranial vault repair, with a taxing intraoperative experience characterized by increased blood loss, increased transfusions, and longer operation time.

Recurrent Carpal Tunnel Syndrome Associated with Mycobacterium szulgai Infection: A Case Report.

CASE: We present the case of an otherwise healthy 77-year-old male retired firefighter and recreational pheasant hunter who presented with recurrent symptoms of carpal tunnel syndrome and tenosynovitis because of Mycobacterium szulgai. He was initially treated unsuccessfully for a presumed seronegative rheumatologic flare, followed by surgical diagnosis and treatment including revision carpal tunnel release with tenosynovectomy, and a secondary debridement and wound closure. His symptoms resolved after several months of multidrug antibiotic therapy with only mild residual median nerve deficit. CONCLUSION: Nontuberculous Mycobacterium infections of the upper extremity are extremely rare and challenging to diagnose/treat. This report highlights diagnostic and surgical challenges in this rarely reported infection.

Orbital Volumetric Analysis in Patients With Unicoronal Craniosynostosis: A Comparison Between Distraction Osteogenesis and Fronto-Orbital Advancement.

BACKGROUND: Unicoronal craniosynostosis is associated with orbital restriction and asymmetry. Surgical treatment aims to both correct the aesthetic deformity and prevent the development of ocular dysfunction. We used orbital quadrant and hemispheric volumetric analysis to assess orbital restriction and compare the effectiveness of distraction osteogenesis with anterior rotational cranial flap (DO) and bilateral fronto-orbital advancement and cranial vault remodeling (FOAR) with respect to the correction of orbital restriction in patients with unicoronal craniosynostosis. METHODS: A retrospective review of all patients with a diagnosis of unicoronal craniosynostosis and treated with either DO or FOAR from 2000 to 2019 was performed. Preoperative and postoperative total orbital volumes, as well as quadrant and hemispheric volume ratios, were calculated from 3-dimensional head computed tomography scans. Selected preoperative and postoperative orbital measurements, including the maxillary length of the orbit (MLO; zygomaticofrontal suture to the top of zygomatic arch) and the sphenoid length of the orbit (SLO; the top of sphenoid suture to the top of zygomatic arch), were also obtained. RESULTS: Data were available for 28 patients with unicoronal craniosynostosis. Mean preoperative total orbital volume was significantly smaller on the synostotic side compared with the nonsynostotic side (10.94 vs 12.20 cm3, P = 0.04). Preoperative MLO and SLO were significantly longer on the synostotic side compared with the nonsynostotic side (MLO: 20.26 vs 17.75 mm, P < 0.001; SLO: 26.91 vs 24.93 mm, P = 0.01). Distraction osteogenesis and FOAR produced significantly different changes in orbital quadrant and/or hemispheric volume ratios on the nonsynostotic side but not on the synostotic side. CONCLUSIONS: Before correction, patients with unicoronal craniosynostosis have significantly smaller total orbital volumes on the synostotic side compared with the nonsynostotic side and significantly greater MLO and SLO on the synostotic side compared with the nonsynostotic side. There is no significant difference between DO and FOAR with regard to correcting the observed orbital restriction in these patients.

The Relationship Between Epidermal Mitotic Density, Atypical Mitotic Figure Density, Breslow Depth, Ulceration, and Dermal Mitotic Rate in Cutaneous Melanoma: A Retrospective Cohort Study.

Prognostic factors for melanoma include Breslow depth (BD), ulceration, and dermal mitotic rate (DMR). No studies have queried the effect of epidermal mitotic density (EMD) or atypical mitotic figure density (AMD) in an outcome-based assessment. Our objective was to determine if there is a relationship between EMD, AMD, BD, DMR, and ulceration and patient outcomes. This was a retrospective cohort study of 185 cases of thick and thin melanomas. Univariate and multivariate cause-specific regression analysis was performed. There was a positive correlation between EMD and BD (P = .0001). The difference between AMD in thick and thin melanomas was statistically significant. For every unit increase in EMD, patients had a 2.8-fold increase in the risk of distant metastasis; however, statistical significance was lost in the multivariate analysis. In adjusted analyses, ulceration, DMR, and BD were associated with outcomes. There were no statistically significant correlations between AMD and outcomes. This study is limited by its small sample size, diminution of the epidermis in some thick melanomas preventing EMD estimates, and reproducibility of mitotic figure counting. EMD and AMD do not seem to have any independent value in multivariate analyses for melanoma. Ulceration, BD, and DMR were significantly associated with outcomes and further solidify these known predictors of prognosis.

Analysis of Actual Versus Predicated Intracranial Volume Changes for Distraction Osteogenesis Using Virtual Surgical Planning in Patients With Craniosynostosis.

INTRODUCTION: The primary outcome metric in patients with craniosynostosis are changes in intracranial volumes (ICVs). In patients who undergo distraction osteogenesis (DO) to treat craniosynostosis, changes are also dependent on the length of distraction. Virtual surgical planning (VSP) has been used to predict anticipated changes in ICV during cranial vault reconstruction. The purpose of this study is to analyze the actual versus predicted ICV changes using VSP in patients who undergo DO for craniosynostosis management. METHODS: All patients with craniosynostosis treated with DO at a single institution, Rady Children's Hospital, between December 2013 and May 2019 were identified. Inclusion criteria are as follows: VSP planning with predicted postoperative ICV values and preoperative and postdistraction CT scans to quantify ICV. Postoperative ICV and VSP-estimated ICV were adjusted for age-related ICV growth. The primary outcome measure calculated was age-adjusted percent volume change per millimeter distraction (PVCPD), and results were analyzed using paired Wilcoxon signed rank tests. RESULTS: Twenty-seven patients underwent DO for cranial vault remodeling. Nineteen patients were nonsyndromic, and 8 patients were syndromic. The median postoperative PVCPD was 0.30%/mm, and the median VSP-estimated PVCPD was 0.36% per millimeter (P < 0.001). A subanalysis of nonsyndromic patients showed a median postoperative PVCPD of 0.29%/mm in nonsyndromic patients that differed significantly from the VSP estimate of 0.34%/mm (P = 0.003). There was also a significant difference in syndromic patients' observed PVCPD of 0.41%/mm versus VSP estimate of 0.79%/mm (P = 0.012). CONCLUSIONS: Virtual surgical planning overestimates the change in ICV attributable to DO in both syndromic and nonsyndromic patients.

A Posterior Rotational Flap Technique Using Distraction Osteogenesis for Unilateral Lambdoid Craniosynostosis.

INTRODUCTION: The craniofacial asymmetry seen in unilateral lambdoid craniosynostosis may not be effectively treated by posterior cranial vault remodeling, endoscopic suturectomy, and helmet therapy, or suturectomy and distraction osteogenesis alone due to limitations in soft-tissue envelope expansion and relapse of the deformity. The authors report a series of unilateral lambdoid craniosynostosis patients treated with a posterior rotational cranial-flap technique using internal distraction osteogenesis. METHODS: Posterior cranial vault reconstruction combined with internal distraction was used, aided by preoperative virtual surgical planning. An in situ posterior rotational flap osteotomy was utilized to maximize dural preservation. Primary outcome measures included age-adjusted volume change and age-adjusted percent volume change per mm distraction. Distraction characteristics and perioperative characteristics were also assessed. RESULTS: A total of 5 patients were identified. Mean predistraction intracranial volume was 1087.5 cc (SD  = 202.3 cc) and mean postdistraction included intracranial volume was 1266.1cc (SD  = 131.8cc). Mean age-adjusted percent included intracranial volume change was 14.1% (SD  = 9.6%), and mean percent intracranial volume change per mm distraction was 0.43%/mm distraction (SD  = 0.37%/mm distraction). One patient developed a distractor site infection postoperatively that was treated successfully with oral antibiotics. All patients had a Whitaker score of 1 at one year follow up. CONCLUSIONS: Posterior cranial vault remodeling using osteogenesis and a rotational cranial flap technique with dural preservation can be effectively used to maximize bone flap viability and limit postoperative relapse in patients with unilateral lambdoid craniosynostosis. Long term analysis as well as comparison to open techniques will need to be interrogated.

Rectovaginal Fistula Repair 1 Year Later: Lessons Learned.

INTRODUCTION: Rectovaginal (RV) fistulas are notoriously difficult to treat. Various methods for repair exist, and refinements in techniques can lead to "successful" outcomes. Review of the literature demonstrates that outcomes studies are scarce and mostly limited to comments on closure rate. We have experienced "success" in our own series with 100% closure rate, regardless of fistula etiology and comorbidities (radiation, inflammation, etc). However, long-term outcomes, including various complications and quality of life changes, have previously been underreported. METHODS: Critical analysis of various outcomes after fistula repair in 14 patients was performed. Patients were surveyed and interviewed with regard to problems before and after fistula repair to obtain objective data focusing on their experience and outcomes. Conclusions are based on physician assessment and patient surveys 1 year after fistula repair and at least 6 months after ostomy reversal and are discussed within the context of data from the literature. RESULTS: Overall satisfaction rate after repairs was high. All patients would undergo attempt at repair again regardless of complications or functional changes (not present before repair). After repair, sexual dyspareunia affected 5 patients (36%); however, most abstained from sexual activity when their RV fistula became apparent. No patient admitted to dyspareunia before the development of their RV fistula. Anal sphincter and defecation function, as well as stool continence, were judged by surgeons and patients uniformly as adequate. However, 3 patients (21%) complained of intermittent problems with urination. A new/different type of pain affected 2 of 4 patients with Crohn disease. One of these patients subsequently developed a new postsphincteric RV fistula. Another patient noted new intermittent vaginal discharge after ostomy reversal, and magnetic resonance imaging suggested a residual fistula, which was not seen on follow-up sigmoidoscopy and "Blue Dye Test." CONCLUSIONS: We previously reported on algorithms for repair and refinements in techniques for "successful" repair of RV fistulas with zero recurrence rate. Long-term follow-up indicates, however, that although the overall satisfaction rate after surgery is high, true "success," defined as permanent fistula closure, is not necessarily problem free. Long-term morbidity and the management of other unique sequelae and problems are underreported.

A Comparison of Intracranial Volumes and Metopic Index in Patients With Isolated Metopic Ridge, Metopic Craniosynostosis, and Normal Healthy Children.

BACKGROUND: Previous research has shown that patients with metopic craniosynostosis have significantly reduced intracranial volumes (ICVs) compared to normal healthy children. Furthermore, the metopic index (ratio of midfrontozygomatic diameter to maximal cranial width) has been described as an anthropometric cranial index for patients with metopic craniosynostosis. We aimed to determine whether patients with isolated metopic ridge have significantly different ICVs or metopic indices than normal children and patients with metopic craniosynostosis. METHODS: A retrospective chart review of all patients with a diagnosis of a metopic ridge or metopic craniosynostosis was performed from 2000 to 2015 at Rady Children's Hospital. Patients were grouped based on computed tomographic scans consistent with metopic craniosynostosis versus metopic ridge. RESULTS: Data were available for 15 metopic ridge patients, 74 metopic craniosynostosis patients, and 213 normal patients. Mean metopic ridge ICV was greater than mean metopic craniosynostosis ICV at 4 to 6 months and 7 to 12 months. Controlling for age and sex, the difference in ICV associated with metopic ridging was 197.484 cm3 and 137.770 cm3 at 4 to 6 and 7 to 12 months, respectively. Similarly, mean metopic index was significantly greater in metopic ridge patients compared to mean metopic craniosynostosis at 4 to 6 months and at 7 to 12 months. CONCLUSIONS: Our study provides volumetric and anthropometric data to support the hypothesis that isolated metopic ridge is an intermediate phenotype between metopic craniosynostosis and normal cranial anatomy. We hope that characterizing the spectrum of disease involving premature closure of the metopic suture with regard to ICV and metopic index will aid physicians in their management of patients with isolated metopic ridge.

Increased Risk of Cutaneous Diseases in Multiple Myeloma Patients.

Introduction Multiple myeloma (MM) is a monoclonal gammopathy characterized by malignant proliferation of plasma cells in the bone marrow, leading to the overproduction of monoclonal immunoglobulins. Knowledge of cutaneous findings associated with multiple myeloma is limited. This study aims to characterize cutaneous manifestations in patients with MM or monoclonal gammopathy of undetermined significance (MGUS). Methods This is a retrospective study of patients seen at a single institution between January 2000 and January 2019 with a diagnosis of "multiple myeloma," "monoclonal gammopathy of undetermined significance," or "smoldering myeloma," and an on-site dermatology clinic visit. Results Twenty patients met the inclusion criteria. Most patients were male and Caucasian. Comorbid cutaneous malignancies were noted in 65% of patients (n = 13). Basal cell carcinoma (BCC) was characterized in 55% of patients (n = 11), followed by squamous cell carcinoma in 50% of patients (n = 10), and melanoma in 10% of patients (n = 2). Conclusions Patients with monoclonal gammopathy may be predisposed to developing cutaneous malignancies and skin infections. Given the low prevalence of monoclonal gammopathy, larger multi-center studies with a control cohort may be necessary to delineate the significance of these comorbid skin conditions.

A Comparison of Intracranial Volume Growth in Normal Children and Patients With Metopic Craniosynostosis.

BACKGROUND: The impact of metopic craniosynostosis on intracranial volume (ICV) and ICV growth is unclear. In addition, the relationship between head circumference (HC) and ICV in these patients is not previously described. METHODS: A retrospective review of 72 patients with metopic craniosynostosis was performed. The ICVs were calculated from manually segmented preoperative computed tomography scans. Magnetic resonance imaging data for 270 healthy children were available. The ICVs were calculated in FreeSurfer.First, a growth curve for metopic patients was generated and a logarithmic best-fit curve was calculated. Second, the impact of metopic craniosynostosis on ICV relative to healthy controls was assessed using multivariate linear regression. Third, the growth curves for metopic patients and healthy children were compared.Pearson's correlation was used to measure the association between HC and ICV. RESULTS: Mean metopic ICV was significantly lower than normal ICV within the first 3 to 6 months (674.9 versus 813.2 cm; P = 0.002), 6 to 9 months (646.6 versus 903.9 cm; P = 0.005), and 9 to 12 months of life (848.0 versus 956.6 cm; P = 0.038). There was no difference in ICV after 12 months of age (P = 0.916).The ICV growth in patients with metopic craniosynostosis is defined by a significantly different growth curve than in normal children (P = 0.005).The ICV and HC were highly correlated across a broad range of ICVs and patient age (r = 0.98, P < 0.001). CONCLUSION: Patients with metopic craniosynostosis have significantly reduced ICVs compared to healthy children, yet greater than normal ICV growth, which allows them to achieve normal volumes by 1 year of age. The HC is a reliable metric for ICV in these patients.

Morphologic Forms and Classification of Dermal Mitotic Figure Density in Primary Cutaneous Melanoma: A Retrospective Study.

New American Joint Committee on Cancer eighth edition staging parameters have removed mitotic rate as a stage T1 category criterion, but it remains embedded in the synopsis of primary cutaneous melanoma (CM). A paucity of data is available, characterizing atypical mitotic forms in CM. In this study, we classify the various morphologic forms of atypical mitoses, characterize mitotic figure density, and examine the correlation between atypical mitotic figures and Breslow depth. We performed a retrospective study of 185 thick (>0.8 mm) and thin (<0.8 mm) CM specimens. Metaphase mitotic figures represented the highest percentage of total mitotic figures in cases of thick melanoma (40%) and were the second most common in thin melanoma (18%). The average Breslow depth for melanoma harboring starburst mitoses was 2.85 mm, compared with the average Breslow depth of all thick melanoma cases, 1.88 mm. The average thickness of melanoma cases containing tripolar mitoses was 2.28 mm. Breslow depth correlated with the number of atypical mitotic figures in both thick and thin melanomas (the Pearson correlation test, r = +0.18, P < 0.01). Metaphase and prophase mitoses are a common finding in both thick and thin melanomas. Although atypical mitoses were indiscriminate, starburst and tripolar (ie, multipolar) mitoses were only inherent to cases of thick melanoma (stage T3). In sum, our study reveals a parallel relationship between the density of atypical mitotic figures and Breslow depth.

Histiocyte-rich Discoid Lupus Erythematosus: A Peculiar Perifollicular Distribution Histologically Mimicking an Acneiform Disorder.

The histologic profile of discoid lupus erythematosus typically involves a vacuolar interface reaction with an associated superficial and deep perivascular infiltrate composed of lymphoplasmacytes. We present a unique case of discoid lupus erythematosus in which cluster of differentiation 68 (CD68) immunochemistry identified widely dispersed histiocytes. Few reports of histiocyte-rich cutaneous lupus erythematosus exist in the literature, and these lymphohistiocytic infiltrates, when present on the H-zone of the face, could be misconstrued as acne/rosacea. Our case demonstrates that cutaneous lupus erythematosus can present with a predominantly histiocytic infiltrate, a pattern dermatopathologists should be aware of to avoid non-recognition or misdiagnosis.