Premature Fusion of the Sagittal Suture as an Incidental Radiographic Finding in Young Children.

BACKGROUND: Craniosynostosis typically develops prenatally and creates characteristic changes in craniofacial form. Nevertheless, postnatal forms of craniosynostosis have been described. The purpose of this study was to determine the prevalence of incidentally identified, but temporally premature, cranial suture fusion in normocephalic children. METHODS: Computed tomographic scans obtained from children aged 1 to 5 years evaluated in the authors' emergency department between 2005 and 2016 were reviewed for evidence of craniosynostosis. Patients with prior ventriculoperitoneal shunt, brain or cranial abnormality, or known syndromes were excluded. The presence of craniosynostosis and cranial index was assessed by a panel of three craniofacial surgeons and one pediatric neurosurgeon. Demographic information, fusion type, reason for the computed tomographic scan, and medical history were recorded as covariates. Cranial shape and intracranial volume were calculated using a previously validated automated system. RESULTS: Three hundred thirty-one patients met the inclusion criteria. The mean age was 2.4 ± 1.3 years. Eleven patients (3.3 percent) were found to have a complete (n = 9) or partial (n = 2) fusion of the sagittal suture. All patients had a normal cranial index (0.80; range, 0.72 to 0.87) and a grossly normal head shape. Only two fusions (18.2 percent) were documented by the radiologist. Cranial shape analysis performed in five of the 11 patients showed subtle phenotypic changes along the scaphocephaly spectrum in four patients, with a normal shape in the remaining case. CONCLUSIONS: Sagittal fusion is present in 3.3 percent of otherwise phenotypically normal children aged 1 to 5 years. The clinical significance of this result is unclear, but routine screening of affected patients is paramount. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, IV.

Device Malfunction Associated With Mandibular Distraction for Infants With Robin Sequence.

INTRODUCTION: Mandibular distraction osteogenesis (MDO) effectively treats upper airway obstruction (UAO) in young patients with Robin sequence (RS). The most commonly used MDO devices have internal and external components that require manual activation. Although complications associated with MDO in infants with RS have been well documented, hardware/device malfunction has not been precisely described. The present study reports the authors' recent experiences with such problems, in an effort to shed light on these complications and identify potential steps to mitigate future related issues. DESIGN: The authors reviewed a prospectively gathered database to identify all young children under the age of 3 years who underwent MDO using buried internal devices for UAO associated with grade 3 RS from March 2007 to September 2019. We specifically focused on complications attributable to the hardware itself. RESULTS: Nineteen patients with 40 devices met inclusion criteria. The median age at MDO was 2.3 months (interquartile range 1.4-6.3 months). Intraoperative activation of all devices under direct vision resulted in satisfactory distraction. Four devices (10.5%) developed postoperative complications directly related to the device, including break down of component parts (N = 3) and failure to maintain distraction distance (N = 1). Two patients required surgical replacement of one device each, whereas the remaining complications occurred during the consolidation phase and did not require intervention. CONCLUSIONS: This report documents a series of device/hardware malfunction in infants and young children undergoing MDO for severe UAO associated with RS. Despite rigorous testing and development, these devices may fail, resulting in patient morbidity.

Craniosynostosis Develops in Half of Infants Treated for Hydrocephalus with a Ventriculoperitoneal Shunt.

BACKGROUND: Craniosynostosis following placement of a ventriculoperitoneal shunt for hydrocephalus has been sporadically described. The purpose of this investigation was to determine the general risk of developing craniosynostosis in this patient population. METHODS: The authors retrospectively reviewed records and radiographs of infants who underwent ventriculoperitoneal shunt placement for hydrocephalus from 2006 to 2012. Recorded variables included date of shunt placement, demographics, comorbidities, cause of hydrocephalus, shunt type, and number of shunt revisions. Axial computed tomographic images obtained before and immediately after shunt placement and 2 to 4 years after shunt placement were evaluated by a panel of clinicians for evidence of craniosynostosis. Patients with preshunt craniosynostosis, craniosynostosis syndromes, or poor-quality computed tomographic images were excluded. Data were analyzed using STATA Version 15.1 statistical software. RESULTS: One hundred twenty-five patients (69 male and 56 female patients) were included. Average age at shunt placement was 2.3 ± 2.58 months. Sixty-one patients (48.8 percent) developed craniosynostosis at a median of 26 months after shunt placement. Of these, 28 patients fused one suture; the majority involved the sagittal suture (n = 25). Thirty-three patients fused multiple sutures; the most common were the coronal (n = 32) and the sagittal (n = 30) sutures. Multivariable logistic regression identified older age at shunt placement and more shunt revisions as independent predictors of craniosynostosis. Shunt valve type was not significant. CONCLUSIONS: Craniosynostosis developed in nearly half of infants who underwent ventriculoperitoneal shunt placement for hydrocephalus. The sagittal suture was most commonly involved. The effect of suture fusion on subsequent cranial growth, shunt failure, or the development of intracranial pressure is unclear. CLINICAL QUESITON/LEVEL OF EVIDENCE: Risk, III.

Severity of Airway Obstruction May Not Correlate With Weight Gain or Failure to Thrive in Infants With Robin Sequence: A Pilot Study.

OBJECTIVE: Patients with Robin sequence (RS) can present with varying degrees of upper airway obstruction, difficulty maintaining adequate weight gain, and failure to thrive (FTT). Although inductive reasoning would suggest that these issues should be interrelated, the relationships between these factors have not been formally studied. This investigation explores the correlation between polysomnographic (PSG) findings, weight gain, and FTT in patients with RS. DESIGN: A prospective database for baseline PSG parameters and serial weight measurements in infants with RS who were admitted for airway obstruction was reviewed. The association between PSG variables and calorie intake with FTT was assessed using univariate and multivariable logistic regression. Categorical analysis of the PSG variables against FTT was explored with a Poisson regression, and linear regression was performed to evaluate the correlation between PSG parameters and percentage of weight gain. RESULTS: Univariate and multivariate logistic regression in RS patients with (n = 13) and without (n = 20) FTT showed no significant association between apnea-hypopnea index (adjusted odds ratio [aOR]: 0.99, P-value = 0.403), O2 nadir (aOR: 0.98, P-value = 0.577), time of O2 saturation below 90% (aOR: 1.03, P-value = 0.574), maximum end tidal carbon dioxide (aOR: 1.0, P-value = 0.977), and average calorie intake (OR:1.02; P-value = 0.984). Furthermore, no significant associations were identified between these variables and weight gain. CONCLUSIONS: This pilot study questions the widely held and intuitively logical belief that poor weight gain and/or FTT should correlate with the severity of upper airway obstruction in patients with RS. Large prospective investigations should be initiated to better explore the authors' findings. Our results also underscore the importance of individualized treatment for these challenging patients.

William H. G. Logan and His Bow.

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.

Heminasal Reconstruction Utilizing Presurgical Planning, Template-Based Cartilage Reconstruction and Tissue Expansion.

Heminasal aplasia is a rare congenital nasal anomaly in which there is unilateral deficiency in both the external nasal anatomy and nasal airway. Unilateral failure in development of a nasal placode in embryogenesis is thought to be the underlying cause of this anomaly. The authors describe the reconstruction of heminasal aplasia in a teenager utilizing a templated cartilaginous framework and tissue expansion. The authors feel the satisfactory results of this technique will be of benefit to other surgeons who may encounter this rare anomaly.

Management and Reconstruction of a Massive Neonatal Neck Teratoma.

Cervical teratoma is a rare tumor comprised of multiple tissue types. These masses can result in significant functional and aesthetic complications, and surgical intervention is the mainstay of treatment. The authors report the treatment and 8-year follow-up of a patient born with a massive cervicofacial teratoma. The mass was diagnosed in utero and required perinatal airway management. The patient underwent several procedures to enhance his appearance and function early on in life with an excellent outcome at intermediate follow-up.

Extremity Findings of Methotrexate Embryopathy.

Background: Methotrexate (MTX) is widely used as an immunosuppressant, chemotherapeutic, and abortifacient agent. It is also a potent teratogen, and intentional or unintentional exposure during pregnancy is associated with heterogeneous birth anomalies. Methods: We retrospectively reviewed a cohort of patients who presented to our clinic with limb anomalies in the setting of MTX embryopathy. Results: In our case series, we describe 7 cases of patients who had limb anomalies with heterogeneous functionality, from severely debilitating to completely asymptomatic. Most of the upper extremity anomalies in our group were managed conservatively. Conclusions: Methotrexate embryopathy is a rare but clinically important entity with phenotypic and functional variability. This series underscores the need for proper counseling of patients and raises concern regarding using this medication for the purpose of abortion.

ABCA12 homozygous mutation in harlequin ichthyosis: Survival without systemic retinoids.

Harlequin ichthyosis (HI) is associated with high mortality. Early systemic retinoids are widely used, although their use remains debatable. We reported two neonates with homozygous mutations in ABCA12 consistent with harlequin ichthyosis who survived to discharge home with intensive care and without use of systemic retinoids.

Neonatal Compartment Syndrome as a Result of Thromboembolic Event.

Neonatal compartment syndrome is a rare condition characterized by progressive limb ischemia and tissue necrosis manifesting at birth or in the immediate postpartum period. Early recognition of clinical features and immediate surgical intervention offer the best prognosis, but unfamiliarity with this uncommon entity often results in delayed diagnosis and catastrophic consequences, including limb amputation. We present a case in a preterm neonate who developed a proximal arterial thrombus after sustaining limb ischemia in utero. This case demonstrated that even delayed treatment with appropriate therapy can result in salvage of the limb. Clinicians should be aware of the characteristic skin findings and institute appropriate measures to determine the presence or absence of compartment syndrome.

A Rare Pediatric Case of Lacrimal Gland Pleomorphic Adenoma.

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.

Tracheocutaneous Fistula Closure with Turnover Flap and Polydioxanone Plate.

An alternative surgical treatment is proposed for closure of tracheocutaneous fistulas. The authors present a new technique for reconstruction of persistent tracheocutaneous fistula resultant from temporary tracheostomy. The single-stage closure under local anesthesia involves a fistulous tract turnover flap with a perforated 0.15 mm polydioxanone plate between the flap and the subcutaneous closure. This article presents 3 cases of persistent tracheocutaneous fistula treated by this method. At follow-up examination after follow-up, no recurrent fistula formation had occurred, and no respiratory deformity was present.

Aggressive Digital Papillary Adenocarcinoma of the Hand Presenting as a Felon.

Aggressive digital papillary adenocarcinoma is a rare eccrine sweat gland malignancy that is frequently misdiagnosed at initial presentation. Histologically, this tumor is similar in appearance to many adenocarcinomas and as such may be diagnosed as a metastatic lesion. We present the case of a patient with digital papillary adenocarcinoma, which was initially diagnosed as a felon. No consensus has been published regarding the treatment of this disease. A review of the diagnosis, pathology, treatment, and adjunctive treatments of aggressive digital papillary adenocarcinoma are also included.

Thermal Burn Injury from a Wedding Ring: An Unusual Case.

Thermal ring injuries are rarely reported in the literature. For this reason, treatment is varied without a standard approach. We describe a case of a thermal wedding ring injury sustained during a welding accident. It is critical to understand the 3 zones of burn injuries when managing these infrequent cases. Furthermore, the dynamic progression that ensues a thermal burn will directly affect outcome. A case is presented along with a graduated approach to the management of such injuries.

Malleolar Ulceration Induced by Hydroxyurea Therapy for Chronic Eosinophila.

A 59-year-old African American female presented to a plastic surgery office in consultation for a very painful non-healing wound of her right lateral malleolus. An incisional biopsy was performed and ultimately a diagnosis of hydroxyurea-induced ulcer was concluded. Descriptions of this entity are rare in the surgical and wound care professional literature. This diagnosis does appear in the hematology/oncology literature; however, these practitioners may not be the first to examine a patient with the development of such an ulcer. This article presents the pathophysiology and clinical presentation of hydroxyurea-induced ulceration as well as the characteristics of hydroxyurea-induced ulceration in addition to the case study.

Clinical Question: In women who have undergone breast cancer surgery, including lymph node removal, do blood pressure measurements taken in the ipsilateral arm increase the risk of lymphedema?

Throughout the healthcare industry fears of taking blood pressure in arm of patients who have undergone breast cancer surgery have been propagated for decades and continue to be recommended by multiple medical societies and healthcare organizations. However, these precautions are not well based on evidence-based medicine and may have a more historical and traditional basis. The purpose of this study was to review current evidence-based research as well as current guidelines regarding ipsilateral arm blood pressure measurements in women who have undergone breast surgery for cancer including lymph node removal.

Clinical Inquiry-In women who have undergone breast cancer surgery, including lymph node removal, do blood pressure measurements taken in the ipsilateral arm increase the risk of lymphedema?

Throughout the healthcare industry fears of taking blood pressure in the arms of patients who have undergone breast cancer surgery have been propagated for decades and continue to be recommended by multiple medical societies and healthcare organizations. However, these precautions are not well based on evidence-based medicine and may have a more historical and traditional basis. The purpose of this study was to review current evidence-based research as well as current guidelines regarding ipsilateral blood pressure measurements in women who have undergone breast surgery for cancer including lymph node removal.

Clinical Inquiry-In women who have undergone breast cancer surgery, including lymph node removal, do blood pressure measurements taken in the ipsilateral arm increase the risk of lymphedema?

Throughout the healthcare industry fears of taking blood pressure in the arm of patients who have undergone breast cancer surgery have been propagated for decades and continue to be recommended by multiple societies and healthcare organizations. However, these precautions are not well based on evidence-based medicine and may have a more historical and traditional basis. The purpose of this study was to review current evidence-based research as well as current guidelines regarding ipsilateral arm blood pressure measurements in women who have undergone breast surgery for cancer including lymph node removal.