Successful Management of Cherubism Patient With Staged Bone Grafting and Fat Grafting.

Cherubism is a rare hereditary dysplasia of the craniofacial skeleton with unpredictable course and controversial management. The authors report a case managed at the onset with limited mandibular resection and primary autogenous bone grafting, as well as staged secondary fat grafting for contour definition. Over 5 years, the patient demonstrated no recurrence of deformity except for mild hypoplasia, which was improved with fat grafting. The advantages of this early treatment were the ability to address the social stigma and anxiety at a young age versus conservative management strategies with minimal comorbidity.

Nasal monobloc osteotomy for correction of late nasal and orbital asymmetry of unicoronal synostosis: A morphometric and outcomes study.

BACKGROUND: Craniofacial asymmetry associated with unicoronal synostosis (UCS) may persist into the teenage years despite surgery in infancy. This study evaluated outcomes following a nasal monobloc procedure by mobilizing a united nasomaxillary and bilateral medial orbital segment of bone (nasal monobloc) to perform corrective translational and rotational movement for secondary correction of residual nasal-orbital asymmetry associated with UCS. METHODS: A retrospective review of all UCS patients treated with nasal monobloc at our institution was performed. Demographic information was recorded, and pre- and postoperative 2D imaging was used for morphometric outcome analysis. Outcomes and complications were tabulated. RESULTS: The study included 14 patients (5 males, 9 females; mean age 14.6 years; range 9.6 to 22.5 years; mean follow-up 70.6 months range 12 to 132 months). Ancillary procedures (scar revision, forehead/orbital contouring, MEDPOR® augmentation) were performed in all patients at the time of the nasal monobloc. One patient underwent a repeat procedure 6 years later following technique modification. Additionally, another patient experienced late overgrowth of the frontal sinus with forehead asymmetry. The morphometric analysis demonstrated significant (p < 0.05) pre-op to post-op improvements in naso-orbital asymmetry, as demonstrated by horizontal orbital aperture ratio (0.88 vs 0.99), midline to exocanthion ratio (0.91 vs 0.98), orbital index ratio (1.15 vs 1.01), and midline discrepancy (7.1 degrees vs 2.7 degrees). CONCLUSION: Nasal monobloc osteotomy provides a reasonable surgical treatment to improve both the nasal and orbital asymmetries associated with unicoronal synostosis, including frontal nasal deviation, basal nasal deviation, and orbital aperture asymmetry. It is important to note that confounding anatomic variables such as globe dystopia, strabismus, and scleral show may affect the perception of orbital symmetry.

Morphometric analysis and outcomes following posterior cranial vault distraction in syndromic and multisuture craniosynostosis.

BACKGROUND: Turribrachycephaly is a common feature in many syndromic and multisuture craniosynostoses and is traditionally treated with total cranial vault reshaping and fronto-orbital advancement. A staged approach with posterior cranial vault distraction as a primary procedure followed by anterior cranial vault reshaping has the advantage of reducing the vertical dimension of the skull in a controlled and gradual manner while expanding the cranial volume. The purpose of this study was to evaluate outcomes following posterior cranial vault expansion using distraction osteogenesis at a single tertiary pediatric center. METHODS: This retrospective review included all cases of posterior cranial vault distraction at a single institution from 2008 to 2022 performed by one surgeon. Morphometric outcomes such as Turricephaly Index (TI) and posterior cranial volume (PCV) were assessed from pre- and postoperative computerized tomography scans for patients who underwent posterior cranial vault distraction as a primary first-stage operation. Clinical outcomes and complications were collated. RESULTS: A total of 41 patients (25 females, 16 males; mean age 11 months) with syndromic craniosynostosis (n = 32) and nonsyndromic craniosynostosis (n = 9) were included. The main indication for posterior cranial vault distraction in this cohort was turricephaly (63%). The mean distraction distance was 25.9 mm and the mean decrease in TI was 18%. The estimated increase in PCV from distraction in this cohort was 19.7%. In this cohort of patients, 13 patients (32%) experienced complications but there were no mortalities. CONCLUSION: Posterior cranial vault distraction osteogenesis is an effective surgical procedure to increase intracranial volume and correct turricephaly in children with syndromic and nonsyndromic craniosynostosis. This procedure is considered a safe and effective first-stage cranial operation in children with syndromic craniosynostosis with increased intracranial pressure and or turricephaly.

Impact of Low-Dose Computed Tomography and Physician Education on Referral for Head Shape.

Imaging before specialist evaluation of abnormal head shape is associated with a delay in evaluation and an increase in radiation exposure. A retrospective cohort study was performed to identify referral patterns before and after the implementation of a low-dose computed tomography (LDCT) protocol and physician education to examine the intervention's impact on time to evaluation and radiation exposure. Six hundred sixty-nine patients with an abnormal head shape diagnosis at a single academic medical center between July 1, 2014 and December 1, 2019 were reviewed. Demographics, referral information, diagnostic testing, diagnoses, and timeline of clinical evaluation were recorded. Before and after the LDCT and physician education intervention, the average ages at initial specialist appointments were 8.82 and 7.75 months, respectively ( P = 0.125). Children referred after our intervention were less likely to have prereferral imaging than children referred prior (odds ratio: 0.59, CI: 0.39-0.91, P = 0.015). Average radiation exposure per patient before referral decreased from 14.66 mGy to 8.17 mGy ( P = 0.021). Prereferral imaging, referral by a non-pediatrician, and non-Caucasian race were associated with older age at the initial specialist appointment. Widespread craniofacial center adoption of an LDCT protocol and improved clinician knowledge may lead to a reduction in late referrals and radiation exposure in pediatric patients with an abnormal head shape diagnosis.

Considerations for Choice of Cranioplasty Material for Pediatric Patients.

INTRODUCTION: Optimal material and timing of cranioplasty in the pediatric population continue to be debated. Autologous and alloplastic materials have various indications for use and risk factors for complications. METHODS: A single-center retrospective cohort study was undertaken of all pediatric patients who underwent cranioplasty with any material from 1991-2021. RESULTS: 149 cranioplasty implants were included. Younger age (6 years old or under), a diagnosis of craniosynostosis as reason for implant, use of autologous bone, and shorter times to cranioplasty were predictive of need for revision surgery. No factors studied had a statistically significant impact on rate of removal of implant at time of revision surgery. CONCLUSION: Autologous and alloplastic cranioplasty materials both have good outcomes with low rates of revision surgery in the pediatric population. Alloplastic implants may be considered in the setting of infection as reason for craniectomy given the lower rate of revision surgery and need for removal. Patients with craniosynostosis as reason for cranioplasty have a higher risk of requiring revision or additional surgeries, regardless of implant used.

Pediatric craniosynostosis computed tomography: an institutional experience in reducing radiation dose while maintaining diagnostic image quality.

BACKGROUND: Children with craniosynostosis may undergo multiple computed tomography (CT) examinations for diagnosis and post-treatment follow-up, resulting in cumulative radiation exposure. OBJECTIVE: To reduce the risks associated with radiation exposure, we evaluated the compliance, radiation dose reduction and clinical image quality of a lower-dose CT protocol for pediatric craniosynostosis implemented at our institution. MATERIALS AND METHODS: The standard of care at our institution was modified to replace pediatric head CT protocols with a lower-dose CT protocol utilizing 100 kV, 5 mAs and iterative reconstruction. Study-ordered, protocol-utilized and radiation-dose indices were collected for studies performed with routine pediatric brain protocols (n=22) and with the lower-dose CT protocol (n=135). Two pediatric neuroradiologists evaluated image quality in a subset (n=50) of the lower-dose CT studies by scoring visualization of cranial structures, confidence of diagnosis and the need for more radiation dose. RESULTS: During the 30-month period, the lower-dose CT protocol had high compliance, with 2/137 studies performed with routine brain protocols. With the lower-dose CT protocol, volume CT dose index (CTDIvol) was 1.1 mGy for all patients (0-9 years old) and effective dose ranged from 0.06 to 0.22 mSv, comparable to a 4-view skull radiography examination. CTDIvol was reduced by 98% and effective dose was reduced up to 67-fold. Confidence in diagnosing craniosynostosis was high and more radiation dose was considered unnecessary in all studies (n=50) by both radiologists. CONCLUSION: Replacing the routine pediatric brain CT protocol with a lower-dose CT craniosynostosis protocol substantially reduced radiation exposure without compromising image quality or diagnostic confidence.

Spring-assisted minimally invasive repair of sagittal craniosynostosis.

Craniosynostosis surgery is intended to repair cranial deformity, reduce the risk of increased intracranial pressure from cephalocranial disproportion, and reduce the risk of developmental delays. In recent years, minimally invasive surgical techniques have been developed to achieve these goals with less tissue disruption, lower rates of transfusion, and shorter recovery time. The operation focuses on unlocking the fused bones, while reshaping relies on an adjunct, most commonly a postoperative cranial molding helmet. As an alternative to the care-intensive helmeting process, reshaping with implanted cranial expander springs has emerged. In this video, the authors demonstrate their technique for spring-assisted minimally invasive repair of sagittal craniosynostosis. The video can be found here: https://vimeo.com/513923721.

Aging of the Adult Bony Orbit.

BACKGROUND: Accurate quantitative data of the adult bony orbital volume and dimension are needed for treatment optimization. In the present study, the authors aim to evaluate adult orbital volume and corresponding linear dimensions according to age, volume, and individual symmetry. METHODS: Seventy computerized tomography facial scans of adults were randomly chosen from an institutional database and 3-dimensionally reconstructed. Studies were excluded for orbital pathology or incomplete radiographic data. Anatomic landmarks were marked. Interval linear distances and orbital volumes were calculated. Data were analyzed using paired T-tests, independent T-tests, linear regression analysis, and 1-way analysis of variance. RESULTS: A total of 140 orbits from 70 patients were analyzed (female = 35, male = 35), ranging from 20 to 88 years of age. Orbital volume was similar between an individual's left and right side; however, a difference was observed in vertical orbital height, orbital width, inferior orbital rim position, orbital roof length, orbital width, and medial orbital wall length (0.45, 0.64, 0.4, 0.77, 0.97, and 5.1 mm, respectively; P < 0.05). In comparison to females, males averaged larger orbital volume by 3.07 cm (29.58 cm versus 26.51 cm, P = 0.0002), medial wall length by 2.66 mm (P < 0.05), and orbital width by 2.66 mm (P < 0.05). Orbital volume did not correlate with patient age, while lateral wall length was correlative. CONCLUSION: This accurate normative data of the adult bony orbit impacts key aspects of patient diagnosis and treatment and also suggest clinical signs of periorbital aging are not due to bony orbital changes.

Markedly Elevated Intracranial Pressure Treated With Cranial Vault Expansion, Instead of CSF Shunting, in a Child With Hurler-Scheie Syndrome and Multiple Suture Craniosynostosis.

Despite a known association of mucopolysaccharidoses (MPS) and craniosynostosis, treatment of elevated intracranial pressure (ICP) in these patients is primarily cerebrospinal fluid (CSF) shunting. We present a unique case of Hurler-Scheie syndrome with multisuture craniosynostosis and elevated ICP, without ventriculomegaly, where elevated ICP was successfully treated with extensive cranial vault expansion and shunt placement was avoided. Patients with MPS should be evaluated for craniosynostosis, and calvarial vault expansion may be considered as a viable treatment alternative to CSF shunting for elevated ICP in select patients.

A Simple Mandibular Distraction Protocol to Avoid Tracheostomy in Patients With Pierre Robin Sequence.

OBJECTIVE: Recent treatment goals for Pierre Robin sequence (PRS) focus on avoiding tracheostomy through modalities such as mandibular distraction osteogenesis (MDO). We primarily evaluated the efficacy of our straightforward MDO treatment protocol for resolution of PRS-associated airway obstruction while secondarily analyzing patient characteristics associated with success or failure of MDO. DESIGN: A retrospective chart review before and after treatment. SETTING: Tertiary institutional center and private practice setting. PATIENTS: All patients were diagnosed with PRS and treated with MDO, according to the MDO treatment protocol, by a single surgeon with the same operative technique from 1999 to 2013. A sample size of n = 38 met the inclusion criteria. Data assessed included tracheostomy status (pre-MDO, post-MDO, or none), microlaryngoscopy and bronchoscopy (MLB) findings, multipositional airway study results, clinical resolution of airway obstruction following MDO, and patient characteristics. MAIN OUTCOME MEASURE: Resolution of airway obstruction with avoidance of a tracheostomy. RESULTS: Prior to referral for MDO, five patients required urgent tracheostomy. Of patients without a pre-MDO tracheostomy (n = 33), two patients required tracheostomy post-MDO, while 94% avoided tracheostomy with clinical resolution of airway obstruction (n = 31). On secondary analysis, anatomic abnormalities diagnosed by MLB were associated with a higher rate of tracheostomy (P = .037), confirming the utility of preoperative evaluation with MLB; otherwise, no patient characteristics demonstrated significance in patient selection. Follow-up ranged from 6 months to 10 years (mean = 6.45 years, median = 7.61 years). CONCLUSIONS: Our treatment protocol demonstrates MDO is highly effective for resolving severe airway obstruction related to PRS. Based on secondary analysis, our simplified protocol does not require amendment.

An Analysis of Inhalation Injury Diagnostic Methods and Patient Outcomes.

The purpose of this study was to compare patient outcomes according to the method of diagnosing burn inhalation injury. After approval from the American Burn Association, the National Burn Repository Dataset Version 8.0 was queried for patients with a diagnosis of burn inhalation injury. Subgroups were analyzed by diagnostic method as defined by the National Burn Repository. All diagnostic methods listed for each patient were included, comparing mortality, hospital days, intensive care unit (ICU) days, and ventilator days (VDs). Z-tests, t-tests, and linear regression were used with a statistical significance of P value of less than .05. The database query yielded 9775 patients diagnosed with inhalation injury. The greatest increase in mortality was associated with diagnosis by bronchoscopy or carbon monoxide poisoning. A relative increase in hospital days was noted with diagnosis by bronchoscopy (9 days) or history (2 days). A relative increase in ICU days was associated with diagnosis according to bronchoscopy (8 days), clinical findings (2 days), or history (2 days). A relative increase in VDs was associated with diagnosis by bronchoscopy (6 days) or carbon monoxide poisoning (3 days). The combination of diagnosis by bronchoscopy and clinical findings increased the relative difference across all comparison measures. The combination of diagnosis by bronchoscopy and carbon monoxide poisoning exhibited decreased relative differences when compared with bronchoscopy alone. Diagnosis by laryngoscopy showed no mortality or association with poor outcomes. Bronchoscopic evidence of inhalation injury proved most useful, predicting increased mortality, hospital, ICU, and VDs. A combined diagnosis determined by clinical findings and bronchoscopy should be considered for clinical practice.

The impact of neoadjuvant hedgehog inhibitor therapy on the surgical treatment of extensive basal cell carcinoma.

BACKGROUND: Although hedgehog inhibitor therapy (HHIT) is offered as isolated medical treatment for extensive basal cell carcinoma (BCC), there is little evidence on the use of HHIT before definitive surgical intervention. In order to better define the utilization of HHIT for extensive BCC, we evaluated the impact of neoadjuvant HHIT on the subsequent surgical resection and reconstruction. METHODS: An IRB-approved, retrospective chart review was performed of patients who received HHIT as initial treatment for extensive BCC. Patients who discontinued HHIT and underwent surgical resection were included. Evaluation included BCC tumor response to HHIT, operative data, pathological data, radiation requirements, and evidence of tumor recurrence. RESULTS: Six patients were identified with tumors of the face/scalp (n = 4), trunk (n = 1) and upper extremity (n = 1). Hedgehog inhibitor therapy continued until tumors became unresponsive (n = 3, mean = 71 weeks) or side effects became intolerable (n = 3, mean = 31 weeks). In each case, a less extensive surgery was performed than estimated before HHIT. In 3 cases, significant bone resection was avoided. All resected specimens contained BCC. Four specimens exhibited clear margins. Postoperative radiation was performed in cases with positive margins (n = 2), and 1 patient experienced local recurrence. Length of follow-up was 5.7 to 11.8 months (mean = 8.23 months). CONCLUSIONS: Although HHIT was not curative for extensive BCC, HHIT can decrease the morbidity of surgical treatment and increase the likelihood of curative resection. For patients with extensive BCC, a combined neoadjuvant use of HHIT and surgical treatment should be considered.

The evaluation of physical exam findings in patients assessed for suspected burn inhalation injury.

The purpose of this investigation was to evaluate the utility of singed nasal hair (SN), carbonaceous sputum (CS), and facial burns (FB) as indicators of burn inhalation injury, when compared to the accepted standard of bronchoscopic diagnosis of inhalation injury. An institutional review board approved, retrospective review was conducted. All patients were suspected to have burn inhalation injury and subsequently underwent bronchoscopic evaluation. Data collected included: percent burn TBSA, burn injury mechanism, admission physical exam findings (SN, CS, FB), and bronchoscopy findings. Thirty-five males and twelve females met inclusion criteria (n = 47). Bronchoscopy was normal in 31 patients (66%). Data were analyzed as all patients and in subgroups according to burn TBSA and an enclosed space mechanism of injury. Physical exam findings (SN, CS, FB) were evaluated individually and in combination. Overall, the sensitivities, specificities, positive predictive values, and negative predictive values calculated were poor and inconsistent, and they did not improve within subgroup analysis or when physical findings were combined. Further statistical analysis suggested the physical findings, whether in isolation or in combination, have poor discrimination between patients that have and do not have inhalation injury (AUC < 0.7, P > .05) and poor agreement with the diagnosis made by bronchoscopy (κ < 0.4, P > .05). This remained true in the subgroup analysis as well. Our data demonstrated the findings of SN, CS, and FB are unreliable evidence for inhalation injury, even in the context of an enclosed space mechanism of injury. Thus, these physical findings are not absolute indicators for intubation and should be interpreted as one component of the history and physical.

Estrogen treatment of acetic acid burns to the vagina, cervix, and perineum: a case report and review of the literature.

In colposcopic evaluation of the cervix, acetic acid of 3 to 5% is commonly used for identification of preneoplastic and neoplastic cells. Acetic acid is a known caustic substance and has the potential to cause irritation and chemical burns when there is sufficient concentration or duration of contact. The authors present a unique case of a woman who inadvertently received undiluted acetic acid during a routine colposcopy, resulting in significant chemical burns of the vagina, cervix, and perineum. Her burns were treated with topical estrogen cream of 1 g twice daily applied directly to the wounds. The burn wounds were fully healed within 8 weeks without complication or additional treatment. At 6 months after the injury, the patient was allowed to engage in sexual activity, and vaginal dilation and pelvic floor therapy were initiated. At 12 months postinjury, her only symptomatic scarring at the left vaginal wall continues to improve. Thus, topical estrogen treatment of 1 g applied twice daily should be continued until burn scar maturation is complete and treatment improvement plateaus in cases of burns to the vagina, cervix, and perineum. This case is further clinical evidence of estrogen's positive effect on wound healing and its potential role in burn treatment.

Giant scalp melanoma: a case report and review of the literature.

INTRODUCTION: Among malignant melanoma lesions, those occurring on the scalp and neck have a particularly poor prognosis. In this case report, we present the largest melanoma of the head and neck and one of the largest melanomas of any anatomic site reported in the literature to date. METHODS: The biopsy revealed left scalp melanoma with a Breslow's thickness of at least 14 mm, and final needle aspiration of lymphadenopathy was consistent with malignant melanoma. Preoperative staging was T4aN3Mx. Wide local excision with 3-cm margins was performed, which included excision of the left ear en-bloc, along with a selective left neck dissection. Reconstruction was performed with a Bilayer Wound Matrix (Integra, 311 Enterprise Drive, Plainsboro, New Jersey) and eventual thin split-thickness skin graft. RESULTS: Final pathology of the left scalp en-bloc excision was a 14.5 × 10.4 cm malignant melanoma, Breslow's thickness of 18 mm. Numerous lymph nodes were positive for melanoma as well. Final pathologic staging was determined to be T4b N3 M1, Stage IV. Later the patient underwent split-thickness skin graft placement on the left scalp acellular dermal matrix, which healed with complete graft take. DISCUSSION: This case report demonstrates a unique presentation of a giant melanoma. With few other cases reported for comparison, it appears our patient's prognosis is poor, despite treatment according to current guidelines.

The Bikini Inset: a reliable method for postradiation breast reconstruction with transverse rectus abdominis myocutaneous and free abdominal tissue transfer.

The combination of radiation and mastectomy reduces the 3-dimensional topography of the breast into a relatively inelastic, 2-dimensional plane. This environment presents specific challenges to aesthetic breast reconstruction with autologous tissue transfer, and a relative sparsity of information exists in the surgical literature on how to address these challenges. Accordingly, this article details a formalized and reproducible approach for flap inset in postradiation breast reconstruction. We outline a novel technique for optimizing the recipient bed and present a sequential flow for contouring the autologous abdominal flap, so that it recreates the individual subunits of an aesthetic breast.

Levamisole-induced necrosis of skin, soft tissue, and bone: case report and review of literature.

This represents the largest case of skin necrosis related to levamisole, a common cocaine contaminant, requiring closure with skin grafts, and is the only case resulting in nasal amputation, central upper lip excision, extremity bone necrosis, and above knee amputation. The case report is followed by a review of the literature. Unique considerations for the full-thickness necrosis induced by levamisole vasculitis are highlighted, including antibody level monitoring, need for multiple excisions, timing of skin grafting, and potential for soft tissue and bone necrosis as well. A 54-year-old man presented to an outside facility with fever, generalized weakness, and agranulocytosis, with a history of cocaine use 3 weeks before. After admission, he developed generalized violaceous lesions and an elevated p-antineutrophilic cytoplasmic antibody and was diagnosed with disseminated vasculitis and agranulocytosis secondary to levamisole-contaminated cocaine exposure. On transfer to the authors' facility, 52% TBSA was involved with violaceous, nonblanching lesions, which progressed to full-thickness necrosis. Local wound care continued until necrotic areas fully demarcated and progressive necrosis stabilized, and skin grafting for closure was not performed until antibody levels normalized. Current treatment of levamisole-induced skin rash or necrosis focuses on discontinuation of levamisole. As demonstrated by this case, extensive necrosis secondary to levamisole-induced vasculitis can be successfully treated with multiple excisions until necrosis stabilizes, and then, split-thickness autografts may be applied. In areas with poor vascular supply or areas with poor functional prognosis, amputation may ultimately be required.