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Summary:. Dermatofibrosarcoma protuberans (DFSP) is a rare malignant fibroblastic tumor. DFSP has an insidious onset, slow growth, and heterogeneous presentation that can create a delay in diagnosis and increase morbidity. In this case report, we present a child with DFSP that presented as a large, slow-growing mass over the dorsum of the left foot. She underwent successful surgical excision with no functional sequelae.

Background:. Interventions for type B postaxial polydactyly include suture ligation and surgical excision, yet there is a paucity of literature comparing the outcomes of these procedures. This study sought to compare patient-reported long-term outcomes of postaxial digit excision. Methods:. A six-question survey was distributed from January 2021 to March 2022 to patients who underwent treatment for type B postaxial polydactyly at a single pediatric institution from 2010 to 2016. Patients were queried about the incidence of pain sensitivity, keloid healing, and/or persistent presence of bump (“nubbin”) at the treatment site. Results:. A total of 158 responses accounting for 258 digits were attained for a 53% response rate. The majority of digits (67.4%, n = 174) were surgically excised. Median age at procedure was 49 days: 13.0 days for ligation, 63.0 days for surgical excision. Median age at survey was 8 [IQR 5.4–10.2] years. Short-term (

Background:. Despite rules set forth by the National Resident Matching Program and American Association of Medical Colleges (AAMC), prohibited questions during the residency interview process are well documented. This study describes the prevalence of these encounters by surveying residency applicants to integrated plastic and reconstructive surgery (PRS) programs for the 2022 match cycle. Methods:. An anonymous 16-question REDCap survey was distributed to 2022 cycle applicants of a single PRS program. The applicants were queried about demographic information, interview experience, and questions deemed illegal by the AAMC/NRMP guidelines. Results:. One hundred survey responses were attained for a 33.1% response rate. The majority of respondents were aged 26-30 (76%), women (53%), and white (53%); 33% received 15+ interviews for the application cycle. Seventy-eight percent of respondents reported being asked a prohibited question during at least one interview, with the most common "illegal" question categories being number/ranking of interviews (42%), marital status (33%), career balance (25%), and race/ethnicity (22%). Only 25.6% of applicants considered the subject matter inappropriate, whereas 42.3% were unsure. Although no applicant took action to report the potentially illegal scenarios, 30% said that their experiences influenced their rank list. Conclusions:. Our survey study revealed that prohibited interview questions in PRS residency interviews are common. Permissible lines of questioning and discussion between programs and applicants during residency interviews have been defined by AAMC. Institutions should provide guidance and training to all participants. Applicants should be made aware of and empowered to utilize available anonymous reporting tools.

Summary:. Calcinosis cutis is the term used to describe the deposition of calcium compounds within the skin and subcutaneous tissue, which can occur after the administration of intravenous calcium compounds. Its etiology is broad, and the clinical presentation is variable, creating a diagnostic challenge. Although iatrogenic calcinosis cutis is extremely uncommon, awareness and early diagnosis of this entity can reduce the risks of severe complications, including soft tissue damage, restricted joint mobility, and even nerve compression. Clinical suspicion should prompt a thorough review of the medical history and appropriate radiographic studies. Evidence of extensive soft tissue calcification must be present on radiographic imaging to confirm the diagnosis. Iatrogenic calcinosis cutis is managed conservatively, and resolution of symptoms is expected within 2 months of symptom onset. Herein we report the case of an infant with DiGeorge syndrome who developed iatrogenic calcinosis cutis after receiving an intraoperative infusion of calcium gluconate. Our patient presented with right lower extremity swelling, erythema, and warmth over a broad area of the leg centered on the entry point of the venipuncture. This was initially mistaken and managed as cellulitis, but once an accurate diagnosis was made, the symptoms gradually resolved with conservative care and no functional sequelae. We also present the literature on iatrogenic and idiopathic calcinosis cutis in the pediatric population.

Background:. Sickle cell disease (SCD) leads to the formation of an atypical hemoglobin tetramer with reduced capacity to carry oxygen. Although correlation between SCD and craniosynostosis (CS) has been mentioned, these are mostly small series or case reports. This article aimed to study any correlation between these entities in a large pediatric population. Methods:. We retrospectively reviewed head CT scans of SCD patients from 0 to 8 years of age who required a CT for issues unrelated to their head shape between 2012 and 2020. We excluded patients with known history of CS or any CS-related syndrome, hydrocephalus, shunt placement, history of cranial surgery, or any reported cerebral or cranial shape abnormality. Results:. Ninety-four CT scans were analyzed. The mean age at imaging was 4.48 ± 2.30 years. CS prevalence in this cohort was 19.1%. Analysis between independent variables and patients with +CS showed that SCD-associated vasculopathy, first-degree relatives with SCD, and the use of folic acid had a statistically significant association with CS development. Conclusions:. Approximately 20% of pediatric patients with SCD developed CS. This association was higher in those patients who had a family history of SCD, used folic acid, and had SCD-associated vasculopathy. While the clinical impact of these findings needs more extensive study, centers that manage patients with SCD should be aware of the relatively high concordance of these diagnoses, vigilantly monitor head shape and growth parameters, and understand the potential risks associated with unidentified or untreated CS.

Background:. The mendosal suture joins the interparietal and inferior portions of the occipital bone. Persistent patency of this suture can result in bathrocephaly, an abnormal occipital projection. This study aims to determine normal temporal fusion of the mendosal suture and cranial shape of the patients with persistent suture patency. Methods:. A retrospective review of head CT scans in patients aged 0–18 months who presented to the emergency department between 2010 and 2020 was completed. Presence and patency of the mendosal suture were assessed. Cranial shape analysis was conducted in the cases that presented with 100% suture patency and age-matched controls. An exponential regression model was used to forecast the timing of suture fusion. Results:. In total, 378 patients met inclusion criteria. Median age at imaging was 6.8 months (IQR 2.9, 11.6). Initiation of mendosal suture fusion was observed as early as 4 days of age and was completed in all instances except one by age 18 months. Most patients had either a complete or partial suture fusion (66.7% versus 30.7%, respectively), and 2.6% of patients had 100% suture patency. Cranial shape analysis demonstrated increased occipital projection in patients with 100% suture patency compared with their controls. Exponential regression model suggested that the mendosal suture closure begins prenatally and typically progresses to full closure at the age of 6 months. Conclusions:. Prevalence of a patent mendosal suture was 2.6% overall. Mendosal suture fusion initiates in-utero and completes ex-utero within the first 18 months of life. Delayed closure results in greater occipital projection.

Summary:. Radiation therapy following oncologic resection can result in incisional breakdown, delay or cessation of wound healing, and exposure of structures. Impaired wound healing often mandates free tissue transfer for definitive closure and preservation of function. We present the case of a 16-year-old male patient who had a major incisional dehiscence following resection of a synovial sarcoma of the lower leg after postoperative irradiation. The progressive wound breakdown failed to respond to local wound care or negative pressure therapy and progressed to expose tendons of the distal leg. The patient was successfully treated with SkinTE, an autologous homologous skin graft and casting. Healing was slow but the wound filled with granulation tissue (which covered the tendon), epithelialized, and led to an excellent cosmetic and functional result. This highlights the potential of stem cell therapy and other forms of regenerative methods in healing of complicated wounds.

Summary:. Tibial nerve entrapment is uncommon in the pediatric population, and presents diagnostic and treatment challenges. We present the unusual case of a 3-year-old male child with progressive lower leg atrophy of an unknown etiology. Preoperative electrodiagnostic testing and magnetic resonance imaging suggested proximal tibial neuropathy. Surgical exploration showed compression of the tibial nerve at the inferior fascial edge of the long head of the biceps femoris and at the soleal sling. Release and external neurolysis led to improvement of distal leg motor function.

Summary:. Epithelioid sarcoma is a rare, high-grade malignant soft tissue tumor that is often misdiagnosed. Classified as a mesenchymal malignancy, it exhibits both mesenchymal and epithelial markers. Occurrence in children under age 10 is extremely rare. This report describes the clinical course and management of a 5-year-old girl who presented with epithelioid sarcoma in the distal extremity. The lesion was initially misdiagnosed and treated for over a year as a common wart.

Summary:. Post-traumatic ectopic nail is an uncommon entity that is occasionally observed after trauma to the fingertip and nail, resulting in aesthetic and functional morbidity. We report a case of post-traumatic ectopic nail in a 3-year-old girl following trauma to her index finger and subsequent surgical intervention to remove an inclusion cyst. The unusual clinical sequence is presented to highlight the etiology and treatment of this rare lesion.

Summary:. Premature fusion of a cranial suture is known to result in characteristic changes in the head shape, even when the synostosis involves only part of the suture. We report an unusual case of a patient seen at the age of 2 months for an abnormal head shape that was present at birth. The phenotype was consistent with an isolated fusion of the frontosphenoidal suture, but the suture was open on a high-resolution computed tomography scan finding. There was no improvement in cranial form after 6 months of helmet therapy, and a follow-up computed tomography scan image taken at age 10 months showed the development of bilateral isolated frontosphenoidal synostosis. This case highlights that an abnormal head shape may, in some patients, predate radiographic evidence of craniosynostosis.

Background:. Patients born with a cleft lip and palate undergo multiple surgical procedures, increasing the infants’ exposure to anesthesia and potential risk of neurocognitive delays. In addition, these numerous procedures lead to greater use of hospital resources. This study analyzes the differences in anesthetic exposure and perioperative characteristics between performing combined versus separate primary palatoplasty (PP) and placement of bilateral myringotomy tubes (BMT). Methods:. We reviewed patients younger than 2 years who underwent PP and BMT at our institution from June 2014 to January 2019. Patients who underwent PP and BMT during the same admission (combined group = 74) and during separate admissions (separate group = 26) were studied. Due to the small sample size in the separate group, additional data for this group were gathered by propensity score matching by gender, race, and American Society of Anesthesiology class. Statistical analyses were carried out to identify significant differences between the 2 groups. Linear regression was performed to compare perioperative variables. Results:. One hundred patients met the inclusion criteria. There were no significant differences in demographic characteristics or American Society of Anesthesiology class, surgery and anesthesia duration, dosage of intraoperative dexmedetomidine, hospital length of stay, and perioperative complications (P > 0.05). Per contra, combined group had significantly lower intraoperative dosages of fentanyl (P = 0.01) and shorter postoperative care unit stay (P = 0.047). Conclusions:. This study documents decreased postoperative care unit time and anesthesia drug exposure without increased length of stay or perioperative complications in patients undergoing combined PP and BMT. These results support combining PP and BMT to reduce exposure to potentially neurotoxic medications and to increase efficient utilization of hospital resources.

Summary:. The Logan Bow is an external device comprising a curved bow-shaped metal bar, with spikes attached to its cross bars, and is widely used as part of a cleft lip management to maintain postoperative apposition and to avoid excessive strain after cheiloplasty for a cleft lip. Since its first description by William Hoffman Gardiner Logan, in the early 20th century, no significant modifications have been made to its original design. Even though this external device continues to be commonly used by cleft care providers, there is a paucity of objective evidence regarding its potential benefits and adverse effects even after almost 100 years since its original description. The goal of the current historical manuscript is to provide the reader an engaging study on the life of the man who invented this device, W. H. G. Logan, his initial description of the bow that bears his name, and how this external device is being used.

Summary:. Anatomic variations in peripheral nerves and the perineural environment are common and can contribute to acute or chronic neuropathy in certain individuals. Awareness of these variants is relevant to understanding both the etiopathogenesis and the increased susceptibility to nerve injury in some patients. We present a 4-year-old boy who sustained a permanent injury to the upper brachial plexus from a relatively minor trauma. Surgical exploration revealed a variation in upper trunk anatomy that likely contributed to this outcome.

Purpose: Sagittal craniosynostosis (SC) is usually diagnosed during early childhood due to the presence of an abnormal head shape termed scaphocephaly. However, patients with radiographic evidence of a prematurely fused sagittal suture but normal head shape (normocephalic) have been reported. A recent study from our group found that 3.3% of normal children under 5 years of age have partially or completely fused sagittal sutures by computed tomography but no corresponding changes in cranial form. The purpose of this paper is to validate our preliminary study with a larger cohort of patients, and to analyze factors that may be associated with incidental finding of early suture fusion. Methods: We reviewed computerized tomography (CT) scans of patients from 0 to 71 months of age who presented to the emergency department (ED) of our tertiary care institution between 2008 and 2020. Patients who were found to have syndromes related with craniosynostosis, phenotypes consistent with craniosynostosis, VP shunt placement, brain or cranial abnormalities, were excluded. A panel of two craniofacial surgeons reviewed the CT scans for presence of craniosynostosis (CS). Demographic information, gestational age, past medical and family history, medications, chief complaint and fusion type were recorded as covariates. Possible medical or demographic differences between patients with and without craniosynostosis were analyzed using unpaired t-test and Chi-square/Fisher’s exact test; Firth logistic regression was performed to determine the association between the independent covariates and the presence of CS in two sub-cohorts; patients ≤ 24 months of age and patients over 24 months of age. Results: We identified 870 patients with a mean age of 30.1 months. 41 patients (4.71%) had fusion the sagittal suture (25 complete, 16 incomplete); all patients had a normal cranial index (width/length >0.7). The prevalence increased from 0-24 months, then was constant from 25-71 months. In the affected patients under two years of age, those with family history of neurodevelopmental disease had 49.32 (95% CI [4.28, 567.2]) times higher odds of developing CS. Sub-cohort encompassing patients above 24 months of age showed no variable as independent predictor for developing CS. Conclusion: Normocephalic SC in young children is common. While the impact of this condition is unknown, the correlation with family history of neurodevelopmental disease is concerning.

Summary:. Pleomorphic adenoma of the lacrimal gland is a rare benign finding in adults, and extremely uncommon in children. These tumors often present with increased propotosis or hypoglobus, and they invariably require operative excision. While many of these lesions can be removed through a subcranial or transorbital exposure, larger and more posterior tumors occasionally necessitate an intracranial approach. This report describes a large lacrimal gland pleomorphic adenoma in a 16 year-old patient that required use of an expansive intracranial exposure to ensure complete excision including the capsule. We contrast this case and its management to other pediatric cases described in the literature.