Search

Introduction Robin sequence (RS) is a leading cause of obstructive sleep apnea (OSA) in newborns. Most studies have focused on understanding anatomic factors leading to OSA and changes in apnea–hypopnea index (AHI) on polysomnography (PSG) beyond the neonatal period. This study aims to define age-related OSA features between patients with RS, without RS and healthy controls using PSG-based analyses of respiratory arousal responses and gas-exchange parameters. Design Retrospective comparison of PSG features in a total of 48 children encompassing three groups: (a) infants with RS (n = 24, Results OSA sleep-stage specific indexes (rapid eye movement [REM] vs non-REM[NREM]) as well as position-specific indexes (supine vs nonsupine) were similar in individuals with and without RS. Relative to the non-RS groups, infants with RS have more sustained hypoxemia (time with SpO2 < 90%) and reduced arousal responses to OSA demonstrated by higher OAHI/respiratory arousal indexes. OAHI/respiratory arousal indexes significantly correlated with the severity of hypoxemia in infants with RS. Conclusion Infants with RS and OSA show reduced arousal responses to apneic events, which correlates with higher hypoxemia severity. OAHI/respiratory arousal indexes in RS may identify high-risk individuals with upper airway obstruction and reduced arousal protective responses.

BACKGROUND Secondary fusion of initially patent cranial sutures after primary correction of nonsyndromic craniosynostosis is rarely reported. This study's aim is to report the incidence and analyze whether there are variables that may predispose to such fusion. METHODS A single-institution, retrospective, case-control study was conducted of all nonsyndromic patients who underwent operative treatment for craniosynostosis from April of 2008 to May of 2017. Patients with less than 1 year of follow-up and/or without a 1-year postoperative computed tomographic scan were excluded. Preoperative, intraoperative, and postoperative variables were analyzed using univariate and multivariate analyses. RESULTS Sixty-six patients were included in the study, with a mean 2.57-year postoperative follow-up. Six patients (8.8 percent) were found to have secondary craniosynostosis, all of whom had fusion of sutures that were initially patent and refusion of the primary pathologic suture(s). Fifty percent of secondary fusions presented as pansynostosis. On univariate analysis, suturectomy with barrel staving (p < 0.01) was significantly associated with secondary suture fusion. On multivariate analysis, bilambdoid suture involvement (p = 0.03) and suturectomy with barrel staving (p = 0.01) were significantly associated with secondary suture fusion. CONCLUSIONS Secondary cranial suture fusion may be a relatively common complication after primary craniosynostosis correction. Suturectomy with barrel staving was independently associated with secondary craniosynostosis. Wide surgical separation of the dura from the cranium and osteotomies across patent sutures may predispose to secondary craniosynostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE Risk, III.

Suboptimal pain management after primary palatoplasty (PP) may lead to complications such as hypoxemia, and increased hospital length of stay. Opioids are the first option for postoperative acute pain control after PP; however, adverse effects include excessive sedation, respiratory depression, and death, among others. Thus, optimizing postoperative pain control using opioid-sparing techniques is critically important. This paper aims to analyze efficacy and safety of combined intravenous (IV), dexmedetomidine, and IV acetaminophen during PP.Review of a cohort of patients who underwent PP from April 2009 to July 2018 at a large free-standing children's hospital was performed, comparing patients who received combined IV dexmedetomidine and acetaminophen with those who did not receive either of the 2 medications. Efficacy was measured through opioid and nonopioid analgesic dose and timing, pain scores, duration to oral intake, and length of stay. Safety was measured by 30-day complication rates including readmission for bleeding and need for supplementary oxygen.Total postoperative acetaminophen (P = 0.01) and recovery room fentanyl (P 0.001) requirements were significantly lower in the study group compared with the control group. Length of stay, oral intake duration, pain scores, total postoperative opioid requirements, and complications rates trended favorably in the study group, though differences did not reach statistical significance.Intraoperative IV dexmedetomidine and acetaminophen during PP provides safe and effective perioperative pain control, resulting in statistically significant decreased need for postoperative acetaminophen and fentanyl. Larger studies are necessary to determine if other trends identified in this study may be significant.

Skin can be mechanically stimulated to grow through a clinical procedure called tissue expansion (TE). Using a porcine TE model, we determined that expansion promptly activates transcription of SFRP2 in skin and we revealed that in the epidermis, this protein is secreted by Langerhans cells (LCs). Similar to well-known mechanosensitive genes, the increase in SFRP2 expression was proportional to the magnitude of tension, showing a spike at the apex of the expanded skin. This implies that SFRP2 might be a newly discovered effector of mechanotransduction pathways. In addition, we found that acute stretching induces accumulation of b-catenin in the nuclei of basal keratinocytes (KCs) and LCs, indicating Wnt signalling activation, followed by cell proliferation. Moreover, TE-activated LCs proliferate and migrate into the suprabasal layer of skin, suggesting that LCs rebuild their steady network within the growing epidermis. We demonstrated that in vitro hrSFRP2 treatment on KCs inhibits Wnt/b-catenin signalling and stimulates KC differentiation. In parallel, we observed an accumulation of KRT10 in vivo in the expanded skin, pointing to TE-induced, SFRP2-augmented KC maturation. Overall, our results reveal that a network of LCs delivers SFRP2 across the epidermis to fine-tune Wnt/b-catenin signalling to restore epidermal homeostasis disrupted by TE.

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.

Objective: Oronasal fistula (ONF) is a known complication after primary palatoplasty (PP). Studies investigating the effect of perioperative antibiotics on fistula rates after PP are limited by inadequate sample size or reliance on self-reporting through national databases. In this study, the authors evaluated the association between single-dose perioperative antibiotics and postoperative fistula rates after PP at a single institution. Design: A retrospective study. Participants: Children younger than 2 years who underwent PP from April 2009 to September 2019 were included. Interventions: Patients were divided into 2 categories: Group 1 received a single intraoperative dose of IV antibiotic, while group 2 did not. Main Outcome Measure(s): Outcome measures included ONF formation, length of stay (LOS), and 30-day readmission rates. Multivariable firth logistic regression, quantile regression, and χ2 tests were performed. Results: Of the 424 patients, 215 and 209 patients were in groups 1 and 2, respectively. The overall ONF rate was 1.9% among all patients. Patients in group 1 experienced an ONF rate of 3.3%, while patients in group 2 had an ONF rate of 0.5%. After correcting for confounding variables, the difference in ONF rates was not statistically different ( P = .68). Median LOS was 35.7 hours and 35.5 hours ( P = .17), while the rate of readmission within 30 days was 4.7% and 2.4% for group 1 and 2, respectively ( P = .96). Conclusions: Administration of a single-dose perioperative antibiotic did not decrease fistula formation after PP, nor did it affect the patient’s LOS or 30-day readmission rate.

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:. 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.

While the lip deformity in unilateral and bilateral cleft lip and cleft palate patients may be the primary focus of parents, most surgeons agree that it is the stigmata of the cleft nasal deformity that is most conspicuous. The cleft lip nasal deformity has been a complex source of innovation for pediatric plastic surgeons for decades. Techniques have evolved as knowledge and experience have grown. Thoughtful surgeons continue to be critical of their outcomes and modify their technique throughout their careers. Here we discuss the fundamentals of the cleft lip nasal deformity and its primary and delayed repair.

BACKGROUND The partner hospital model identifies hospitals in the developing world to educate and enable local surgeons to deliver effective cleft care. This study aimed to determine the outcomes of this model on safety, education, and quality of surgical care. METHODS Twelve partner hospitals, sponsored by Smile Train for 5 or more years and distributed over 4 continents, were selected. Activities at each institution were evaluated using cleft surgical data, and surveys were completed by hospital leadership. RESULTS A mean of 82% of cleft patients at partner hospitals underwent sponsored surgeries. After partnership, all 12 hospitals implemented preoperative checklists for cleft surgery, and 5 implemented checklists for other surgeries. All hospitals had personnel who received safety training as a result of partnership. There was no change in 30-day reoperations or readmissions. Follow-up rate increased by 18% (P = 0.03). Facilities recruited 1.8 additional cleft surgeons (P < 0.01) and increased the number of cleft surgical trainees by a mean of 13.3 annually (P = 0.012); 2.5 ± 1.7 additional ancillary services were added, resulting in 75% of partner hospitals having a basic multidisciplinary cleft team (surgery, speech, and dental) compared with 25% prior to partnership (P < 0.01). Total cleft surgeries, alveolar bone grafts, and percentage of secondary surgeries increased significantly as length of partnership progressed (P < 0.01). CONCLUSIONS Smile Train's partner hospital model increases both the volume and quality of cleft care delivered at these institutions. Safety initiatives for cleft care demonstrate effects extending to global surgical care delivered at partner hospitals.

Autologous breast reconstruction timing continues to be controversial. The purpose of this study was to examine delayed autologous breast reconstruction at a center favouring immediate reconstruction to better understand factors driving the decision to delay reconstruction.We performed a retrospective cohort study of all free autologous breast reconstruction patients between 2005 and 2009, focussing on ethnicity, cancer stage, unilateral or bilateral reconstructions, initial management, distance from the institution, and average income. Delayed reconstructions were compared to immediate reconstructions. All delayed reconstructions were surveyed to examine treatment and reconstruction decisions and satisfaction.Of 709 patients, 169 (24%) underwent delayed treatment. Delayed reconstruction patients had higher cancer stages (p0.001), higher rates of pre-reconstruction radiation therapy (64% vs. 20%, p0.0001) and higher rates of unilateral reconstruction (64% vs. 48%, p0.001). Seventy delayed patients responded to the survey (41%), with 75% having had their initial mastectomy at an outside health system. Only 51% discussed immediate reconstruction prior to electing delayed treatment and 41% had no discussion regarding advantages or disadvantages to reconstructive options. Approximately 30% noted no choice in their reconstructive timing. Forty five percent would elect immediate reconstruction if given the option.This study demonstrates that women may not be receiving all available information prior to undergoing mastectomy for initial breast cancer treatment. As a significant portion of women electing delayed reconstruction would elect immediate autologous reconstruction if given the option again, there is room for improvement in pre-operative patient education and in the education of our oncology colleagues.

The goals of successful palate repair include optimizing speech and feeding, mitigating adverse maxillary growth effect, and avoiding fistulae. The necessity of vomerine and/or nasal-side mucosa repair has not been tested. The purpose of this study was to compare the outcome of palate repairs with and without nasal mucoperiosteal closure. The authors used the null hypothesis.This was a retrospective analysis of consecutive cleft palate repairs performed between 2001 and 2004. Group 1 underwent two-layer repair (oral and nasal/vomerine mucoperiosteal flaps), and group 2 underwent one-layer closure (oral mucoperiosteal flaps) only. Both groups underwent double-opposing Z-plasty posteriorly. Demographic and perioperative outcome variables were recorded and compared statistically.Group 1 consisted of 51 children (23 boys and 28 girls), and 80 percent were nonsyndromic. Group 2 included 29 patients (15 boys and 14 girls), and 72 percent were without an associated diagnosis. Age at repair was similar (20.80 and 15.17 months, respectively). Operative time was less for one-layer repair (84 versus 135 minutes) (p = 0.0001). Complications, length of stay, and follow-up length were similar between the two cohorts. Velopharyngeal dysfunction was rare in both groups. A single fistula occurred in each group. Anthropometric data revealed larger maxillary arc and tragus-subnasale lengths in group 2. Growth velocities were similar in both groups.The goals of cleft palate repair can be efficiently achieved using a one-sided oral mucoperiosteal repair only. Omitting the nasal-side and vomer repair does not increase fistula formation or prove detrimental to velopharyngeal function, and may facilitate maxillary growth.

Posterior cranial vault distraction osteogenesis has recently been introduced to treat patients with multisuture syndromic craniosynostosis and is believed to provide greater gains in intracranial volume. This study provides volumetric analysis to determine the gains in intracranial volume produced by this modality.This was a two-center retrospective study of preprocedure and postprocedure computed tomography scans of two groups of 15 patients each with syndromic multisuture craniosynostosis treated with either fronto-orbital advancement or posterior cranial vault distraction osteogenesis. Scan data were analyzed volumetrically with Mimics software. Volumetric gains attributable to growth between scans were controlled for.The mean advancements were 12.5 mm for fronto-orbital advancement and 24.8 mm for distraction osteogenesis. The mean difference in volume between the preoperative and postoperative scans was 144 cm(3) for fronto-orbital advancement and 274 cm(3) for (p = 0.009). After controlling for growth, the corrected mean volume difference was 66 cm(3) for fronto-orbital advancement and 142 cm(3) for distraction osteogenesis (p = 0.0017). The corrected mean volume difference per millimeter of advancement was 4.6 cm(3) for fronto-orbital advancement and 5.8 cm(3) for distraction (p = 0.357).In this retrospective study, posterior cranial vault distraction osteogenesis provided statistically greater intracranial volume expansion than fronto-orbital advancement. The volume gains per millimeter advancement were similar between groups, with a trend toward greater gains per millimeter with distraction osteogenesis. Gradual expansion of the overlying soft tissues with posterior cranial vault distraction osteogenesis appears to be the primary mechanism for greater volume gains with this technique.Therapeutic, III.

Background—Asthma has been associated with eosinophil activation, measured in serum, sputum, bronchoalveolar lavage (BAL) fluid, and urine. A whole blood automated method was developed to assess eosinophil and neutrophil activity in terms of peroxidase content and cell morphology using the Bayer haematology analyser. The method was applied to an in vitro stimulation model when fMLP was added to whole blood and the samples were then analysed for changes in granularity and shape. In addition, cells stimulated with interleukin (IL)-8 were examined by electron microscopy. Methods—A cross sectional analysis was performed on venous blood from nonatopic, non-asthmatic normal subjects (n = 37), mild (n = 46) and symptomatic (n = 22) asthmatic patients on inhaled ‚2 agonist only, and more severe asthmatic patients (n = 17) on inhaled and oral corticosteroid therapy. Samples were analysed by the haematology analyser and peroxidase leucograms gated using the WinMDI software program. Results—There were significant diVerences in the amount of light scatter by the neutrophil populations in the symptomatic (p = 0.007) and severe asthmatic (p = 0.0001) groups compared with the control group. However, abnormalities in eosinophil populations were not observed. In vitro activation of whole blood with fMLP caused similar changes in neutrophil light scatter, suggesting that neutrophil activation is present in peripheral blood of symptomatic asthmatic patients. IL-8 caused a change in shape of the neutrophils seen using transmission electron microscopy. Conclusions—Evidence of neutrophil activation can be seen in whole blood from patients with asthma using a novel automated method. This may potentially be applied to other inflammatory diseases. (Thorax 2000;55:471‐477)

Endogenous exhaled nitric oxide (NO) is increased during the late response to inhaled allergen in patients with asthma and may be bronchoprotective in asthma or have a deleterious effect when generated in excess under inflammatory conditions. To investigate this, we evaluated the effect of inhibiting endogenous NO production with nebulized NG-nitro-L-arginine methyl ester (L-NAME), a nonselective NO synthase (NOS) inhibitor, on early and late asthmatic responses to inhaled allergen in patients with mild allergic asthma. After a screening allergen challenge (AC), 22 male patients attended two visits conducted in a double-blind, randomized, placebo-controlled, crossover manner. Twelve patients demonstrating an early asthmatic response only (single responders) inhaled either L-NAME 170 mg or 0.9% saline 20 min before AC, with exhaled NO and FEV1 measured for 3 h. Ten patients demonstrating both early and late asthmatic responses (dual responders) were studied in a similar fashion but inhaled two further doses of L-NAME or placebo 3.5 and 7 h after the initial dose, with exhaled NO and FEV1 measured for 10 h. L-NAME reduced exhaled NO levels by 77 +/- 5% (p < 0.01) and 71 +/- 7% (p < 0.01) in single and dual responders, respectively, but had no significant effect on early or late asthmatic responses. Following AC in single responders, the mean (+/- SEM) maximum fall in FEV1 after L-NAME and saline was 21.2 +/- 2.9% and 23.8 +/- 3.0%, respectively, and in dual responders, 31.2 +/- 4.5% and 31.8 +/- 5. 8% during the early asthmatic responses, and 27.4 +/- 3.9% and 30.6 +/- 4.5% during the late asthmatic responses, respectively. Area under the curve (AUC) did not significantly differ. AUC0-2 h in single responders after L-NAME and saline was 20.2 +/- 3.9 and 24.9 +/- 4.4 Delta% FEV1/h, and in dual responders, 37.6 +/- 8.4 and 36.7 +/- 8.4 Delta% FEV1/h, respectively, and 106.2 +/- 18.9 and 117.1 +/- 22.4 Delta% FEV1/h, respectively, for the AUC4-10 h. This study suggests that in mild allergic asthma, endogenous NO neither protects against nor contributes to the processes underlying airway responses to inhaled allergen.

The spheno-occipital synchondrosis is a driver of cranial base and facial growth. Its premature fusion has been associated with midface hypoplasia in animal models. The authors reviewed computed tomographic scans of patients with Apert and Muenke syndrome, craniosynostosis syndromes with midface hypoplasia, to assess premature fusion of the spheno-occipital synchondrosis when compared with normal controls.Ninety head computed tomographic scans of Apert syndrome patients and 31 head scans of Muenke syndrome patients were assessed, in addition to an equal number of control scans. Spheno-occipital synchondrosis fusion on axial images was graded as open, partially closed, or closed. Analysis focused on ages 7 to 14 years, as no control patient fused before age 7 or had failed to fuse after age 14.All 38 Apert syndrome patients aged 7 to 14 had some degree of spheno-occipital synchondrosis closure, compared with 29 of 38 matched controls (p = 0.0023). Seventeen of 20 Muenke syndrome patients showed closure, compared with 14 of 20 matched controls (p = 0.4506). Partial fusion was seen as early as age 2 in Apert syndrome and age 6 in Muenke syndrome patients; the earliest fusion was seen at age 7 in the control group.Compared with matched controls, the spheno-occipital synchondrosis closes significantly earlier in patients with Apert syndrome but not Muenke syndrome. This correlates well to reported incidences of midface hypoplasia in these syndromes. Although causality cannot be concluded from this study, an association exists between midface phenotype and degree of spheno-occipital synchondrosis closure.Risk, II.

Background Hypercoagulability or thrombophilia is a group of inherited or acquired conditions associated with a predisposition to thrombosis. Most hypercoagulable states alter the blood itself or affect the vasculature, directly creating a detrimental environment for microsurgery. The authors present their series of hypercoagulable patients who underwent free flap reconstruction. Methods A retrospective review was conducted of all free flaps performed between January 1, 2005, and October 1, 2010, at the University of Pennsylvania. A total of 2032 flaps were performed. Forty-one patients or 58 free flaps (2.9 percent) were identified as having a diagnosed thrombophilia or previous thromboembolic event. Results Of the 41 patients, 36 were women and five were men. Diagnosis included factor V Leiden mutation, protein C deficiency, hyperhomocysteinemia, antiphospholipid antibody syndrome, prothrombin gene mutation, factor VIII elevation, anticardiolipin antibody syndrome, and essential thrombocytosis. The group of patients with prior thrombotic events (many with concomitant events and diagnoses) included deep vein thrombosis, pulmonary embolus, myocardial infarction before the age of 50, and embolic stroke. Twelve patients (29.3 percent) were actively followed by a hematologist. The rate of thrombosis was 20.7 percent (12 flaps), including those occurring intraoperatively. The salvage rate for a postoperative thrombosed flap in this group was 0 percent. The flap loss rate was 15.5 percent. Conclusions Although hypercoagulability produces an unfavorable condition for microvascular reconstruction, free tissue transfer is feasible. In this series, the authors had an 80 percent success rate. Collaboration with a hematologist may be helpful. In this group, flap thrombosis seems to occur in the delayed period. Even with operative reexploration, salvage rates have not been promising. Clinical question/level of evidence Risk, IV.