Pharyngeal Arches, Chapter 2: Ear and Neck Abnormalities.

The pharyngeal arches are the foundation of face and neck development. Impaired development of these embryologic structures can result in craniofacial abnormalities. Surgeons who manage head and neck pathology will invariably encounter conditions associated with aberrant pharyngeal arch anatomy, and a thorough understanding of the normal development of these structures is paramount to accurate diagnosis and treatment. This manuscript is the second of a four-part series written for plastic surgeons, focusing on the abnormal development of pharyngeal arches leading to pathologic ear and neck anomalies seen in clinical practice.

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.

Reconstruction of a Hemirhinectomy Defect Using a Three-Dimensional Printed Custom Soft Tissue Cutting Guide.

ABSTRACT: The 3-stage paramedian forehead flap is the gold standard for subtotal and complete nasal defects, but significant surgeon artistry and experience are required to achieve good, consistent results. The authors describe the use of virtual surgical planning and three-dimensional printing to create a patient-specific soft tissue cutting guide for the design of a forehead flap in the reconstruction of a hemirhinectomy defect. Application of this technology to these challenging reconstructive scenarios promises to improve accessibility and consistency of results.

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.

Quantification of Nasal Septal Deviation With Computed Tomography Data.

BACKGROUND: Despite extensive literature on the classification and management of nasal septal deviation (NSD) for preoperative planning, standardized objective measures to evaluate the NSD severity remains challenging. In this study, we quantitatively analyzed NSD to determine the most predictive two-dimensional (2D) computed tomography (CT)-landmark for overall three-dimensional (3D) septal morphology derived from nasal airway segmentation. METHODS: A retrospective study was conducted at a large academic center. One hundred four patients who underwent CT scans of the face were selected from a computer imaging database. Demographic variables were screened to ensure an equal number of men and women in different age groups. Digital Imaging and Communications in Medicine files were imported for 3D nasal cavity segmentation using 3D Slicer software. A volumetric analysis was performed to determine 3D NSD ratios. These values were compared to previously reported methods of obtaining objective 2D NSD measures using OsiriX and MATLAB software. Maximum deviation values were calculated using OsiriX, while the root mean square values were retrieved using MATLAB. Deviation area and curve to line ratios were both quantified using OsiriX and MATLAB. RESULTS: The data set consisted of 52 men and 52 women patients aged 20 to 100 years (mean = 58 years, standard deviation = 23 years). There was a strong correlation between 3D NSD ratio and maximum deviation (r = 0.789, P < 0.001) and deviation area (r = 0.775, P < 0.001). Deviation area (r = 0.563, P < 0.001), root mean square (r = 0.594, P < 0.001), and curve to line ratio (r = 0.470, P < 0.001) had a positive correlation of moderate strength. The curve to line ratio was not significant (r = 0.019, P = 0.85). CONCLUSIONS: The 2D CT-based NSD landmarks maximum deviation and deviation area were the most predictive of the severity of NSD from 3D nasal cavity segmentation. We present a robust open-source method that may be useful in predicting the severity of NSD in CT images.

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.

Partial Suture Fusion in Nonsyndromic Single-Suture Craniosynostosis.

INTRODUCTION: Partial synostosis of cranial sutures has been shown to have clinical and diagnostic significance. However, there is limited published information about how suture fusion progresses over time. In this study, we evaluate patients with nonsyndromic single-suture synostosis. We aim to define the incidence of partial versus complete suture fusion and whether a correlation exists between the degree of suture fusion and age. METHODS: Two hundred fifty-four patients with nonsyndromic single-suture synostosis were evaluated. Preoperative computed tomography (CT) scans were rendered in 3-dimensions, all sutures were visualized and assessed for patency or fusion, and length of fusion was measured. Findings were grouped according to suture type (sagittal, coronal, metopic, or lambdoid), the degree of fusion (full, >50%, or <50%), and patient age at time of CT scan (0-90, 91-180, 181-360, or >360 days). Data were analyzed to correlate patient age versus the degree of suture fusion. RESULTS: For all patients, 72% had complete and 28% had partial synostosis. Ratios of full to partial fusion for each suture type were as follows: sagittal 97:36, coronal 35:22, metopic 46:4, and lambdoid 4:10. The sagittal, coronal, and metopic groups demonstrated greater probabilities of complete suture fusion as patient age increases (P = .021, P < .001, P = .001, respectively). This trend was also noted when all sutures were considered together by age-group (P < .001). CONCLUSION: We note a partial suture fusion rate of 28.3%. Our analysis shows a correlation between the extent of suture synostosis and patient age. Finally, we demonstrate that different sutures display different patterns of partial and complete fusion.

Correction of Microstomia Reconstruction With the Use of Acellular Dermal Matrix for Buccal Reconstruction.

Correction of microstomia is challenging with a high rate of recurrence. We report the successful treatment of microstomia using acellular dermal matrix (ADM) as an adjunct for intraoral lining with >1 year of follow-up.A 9-year-old international patient with severe immunodeficiency presented with severe microstomia because of recurrent oral infections. She had undergone 3 previous failed attempts to re-establish an adequate oral opening and was dependent on enteral nutrition via gastrostomy tube. She underwent release of the oral commissure scar contracture and orbicularis oris and the resultant mucosal defect was lined with ADM. A postoperative splint was used for 8 weeks. One-year follow-up demonstrated maintenance of the oral aperture with complete mucosalization of the ADM; the patient was able to resume oral diet and regular dental hygiene.Mucosal reconstruction with ADM is a viable alternative to local flaps and in this case exhibited minimal soft tissue contraction.

Immunogenic potential of human bone marrow mesenchymal stromal cells is enhanced by hyperthermia.

BACKGROUND AIMS: Bone marrow-derived mesenchymal stromal cells (MSCs) have been reported to suppress T-cell proliferation and used to alleviate the symptoms of graft-versus-host disease (GVHD). MSCs are a mixed cell population and at this time there are no tools to isolate the cells responsible for the T-cell suppression. We wanted to find a way to enhance the immune-modulatory actions of MSCs and tried varying the temperature at which they were cultured. METHODS: We cultured human MSCs derived from healthy volunteers at different temperatures and tested their ability to switch macrophage character from pro-inflammatory to anti-inflammatory (M1 type to M2 type). Using an enzyme-linked immunosorbent assay (ELISA), we showed that when MSCs are cultured at higher temperatures their ability to induce co-cultured macrophages to produce more interleukin-10, (IL-10) (an anti-inflammatory cytokine) and less tumor necrosis factor alpha, (TNFα) (a pro-inflammatory cytokine) is increased. We performed Western blots and immunocytochemistry to screen for changes that might underlie this effect. RESULTS: We found that in hyperthermia the heat shock protein, HSF1, translocated into the nucleus of MSCs. It appears to induce the COX2/PGE2 (Cyclooxygenase2/Prostaglandin E2) pathway described earlier as a major mechanism of MSC-directed immune-suppression. CONCLUSION: Hyperthermia increases the efficacy of MSC-driven immune-suppression. We propose that changing the time of MSC administration to patients to mid-to-late afternoon when the body temperature is naturally highest might be beneficial. Warming the patient could also be considered.

The "Double" Tessier 7 Cleft: An Unusual Presentation of a Transverse Facial Cleft.

Congenital macrostomia, or Tessier number 7 cleft, is a rare craniofacial anomaly. We present a unique patient with bilateral macrostomia that consisted of a "double" transverse cleft on the left side and a single transverse cleft on the right side. A staged reconstructive approach was used to repair the "double" left-sided clefts. This staged technique produced a satisfactory aesthetic and functional outcome.

Factors Predicting Hospital Length of Stay Following Pharyngeal Flap for Velopharyngeal Insufficiency.

OBJECTIVE: Routine hospital admission following pharyngeal flap (PF) to correct velopharyngeal insufficiency (VPI) is the standard at most hospitals. Nevertheless, there is increasing resistance from third-party providers to approve stays longer than a "short stay" (23-hour) observation period. The purpose of the current study was to evaluate length of stay (LOS) and document potential influencing factors following PF. DESIGN: Retrospective chart review. Demographic and perioperative data were collected, and statistical analyses were performed to determine associations with hospital length of stay (LOS). Readiness for discharge was determined by oral intake, analgesic requirement, and respiratory status. SETTING: Tertiary care children's hospital Participants: All patients undergoing PF for VPI between 1990 and 2014. OUTCOME MEASURES: (1) LOS, (2) % satisfying all discharge criteria within a 23-hour observational time frame. RESULTS: Seventy-five patients were studied, with an average age of 6.8 years. Mean LOS was 65.4 hours. Only 11 patients (14.9%) met all discharge criteria by 23 hours. Multivariate predictors of shorter LOS were increasing patient age, male gender, lack of syndromic association, administration of an intraoperative antiemetic and steroids, and shorter anesthetic duration. Time to first oral intake correlated positively with LOS. Administration of intraoperative antiemetics increased the odds of meeting all discharge criteria within 23 hours by a factor of 12. CONCLUSIONS: Identification of factors associated with LOS after PF may allow providers to predict and potentially mitigate hospital stays. Nevertheless, most patients undergoing PF are not clinically ready for discharge within a short-stay (23-hour) observation period.

Conjoined Twin Separation: Integration of Three-Dimensional Modeling for Optimization of Surgical Planning.

Conjoined twinning is a rare anomaly, with an incidence of approximately 1 in 100,000 live births. There is a high perinatal mortality rate, but twins who survive pose reconstructive challenges that require meticulous preoperative planning. The authors describe the senior surgeon's career experience with conjoined twin separation, and the evolution of medical modeling and 3-dimensional imaging as a critical component in presurgical planning.The authors performed a retrospective review of all consecutive patients of conjoined twin separation at a single institution from January 2004 to December 2013. Data were collected related to patient demographics, comorbidities, operative technique, perioperative complications, survival, long-term outcomes, and the type of medical modeling system used for preoperative planning.Five sets of conjoined twins underwent separation during the 10-year study period. There were 3 sets of thoraco-omphalopagus twins, 1 set of pyopagus twins, and 1 set of ischiopagus tetrapus twins. The mean age at separation was 70 days, with a mean of 3.5 surgical procedures performed per patient during the first year of life. One set of twins experienced postseparation complications that warranted immediate return to the operating room. The overall survival rate after separation was 70%. The imaging methods used were computed tomography scan with 3-dimensional reconstruction, plaster molds, medical modeling with composite printing, and virtual surgical planning.The use of imaging and medical modeling in presurgical planning has proven to be an important element in optimizing the outcomes for patients with this rare anomaly.

Columellar Reconstruction After Nasal Continuous Positive Airway Pressure Associated Necrosis.

Though a life-saving modality in neonatal intensive care units, nasal continuous positive airway pressure (nCPAP) carries a small risk of irreversible ischemia and necrosis of the columella due to the configuration of the pressure delivery system. Iatrogenic injuries to the columella after nCPAP use result in a spectrum of disfigurement and functional airway obstruction. The authors performed a retrospective review of patients evaluated for nCPAP-related columellar deformities by the Division of Plastic and Reconstructive Surgery at the authors' institution over a 10-year period to assess reconstructive outcomes. Of 7 patients evaluated, 3 underwent reconstruction using a combination of cartilaginous framework reshaping and local tissue flaps. After a mean follow-up period of 78 months, patients had satisfactory aesthetic and functional results. Based on the authors' observations, columellar necrosis secondary to nCPAP can be divided into 3 categories: Type A demonstrates mild notching of the columella; Type B has an absent columella without notable nasal tip depression; Type C has an absent columella with nasal tip depression, with or without external nasal valve obstruction. Reconstructive needs should be individually tailored based on the degree of nasal tip depression, cartilaginous support, and soft tissue availability.

Closed-Suction Drains After Subcutaneous Mastectomy for Gynecomastia: Do They Reduce Complications?

BACKGROUND: In cases of refractory gynecomastia, surgical excision of excess glandular breast tissue is often warranted. Closed-suction drain placement is commonplace; however, the effect of drains on preventing complications after male breast surgery has not been fully elucidated in the literature. OBJECTIVE: To investigate the effect of drains on reducing seroma and hematoma after subcutaneous mastectomy for gynecomastia. METHODS: Retrospective chart review of patients undergoing subcutaneous mastectomy for gynecomastia over a 10-year period. Charts were reviewed for demographics, BMI, comorbidities, type of local anesthesia, specimen weight, use of liposuction, and placement of drains. Outcomes were determined by seroma or hematoma in the 30-day postoperative period. RESULTS: A total of 163 breasts were studied (group I = no-drain, n = 46; group II = drain, n = 117). Group I had a higher rate of clinically significant seromas requiring needle aspiration than group II (6.5 vs 0%, p = 0.0214). There was no difference in rates of hematoma (group I vs II, 2.2 vs 6.0%, p = 0.443) and total fluid collections (group I vs II, 19.6 vs 16.2%, p = 0.647). BMI, use of local anesthesia, specimen weight, and use of liposuction were not significantly associated with postoperative complications. CONCLUSIONS: Closed-suction drains may reduce rates of clinically significant seromas requiring needle aspiration. Though drains carry their own pertinent adverse effects (e.g., patient discomfort and anxiety, cost, and additional clinic visits), there remains no universal standard of care and surgeon practices vary widely. Given the potential to reduce seroma rates, we recommend that surgeons consider placing drains and discuss with patients the benefits and risks of drains after surgical treatment of gynecomastia. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to Table of Contents or the online Instructions to Authors www.springer.com/00266 .

PREVELENCE OF PRIMARY ALDOSTERONISM IN AN URBAN HYPERTENSIVE POPULATION.

OBJECTIVE: To determine the prevalence of primary aldosteronism (PA) in hypertensive patients presenting to the primary care clinic at The Mount Sinai Hospital, regardless of the degree of hypertension and to identify clinical criteria that should prompt screening for PA. METHODS: An aldosterone:renin ratio (ARR, cutoff ≥20, with plasma aldosterone concentration [PAC] ≥10 and suppressed renin) was used to prospectively screen 296 hypertensive patients (blood pressure [BP] ≥140/90) over the age of 18 from August 2012 through May 2013. Subjects who screened positive then underwent confirmatory oral salt load testing (OSLT). RESULTS: Of the 296 patients, 14 screened positive for PA, an overall prevalence of 4.7%. Six of the 14 cases underwent confirmatory OSLT, upon which 2 were confirmed positive, for a prevalence of 0.7%. Overall, patients with confirmed PA were more likely to have resistant hypertension (42.9% vs. 18.1% (P = .0334)) and require more antihypertensive agents (2.8 ± 1.2 agents vs. 2.1 ± 1.1 agents, P = .0213). There was a trend toward lower potassium values in the cases. CONCLUSION: The prevalence of PA in our clinic is much lower than in reports from certain "at-risk" populations. PA screening is indicated in patients with resistant hypertension, regardless of serum potassium levels. ABBREVIATIONS: ARR = aldosterone:renin ratio ACTH = adrenocorticotropic hormone AVS = adrenal venous sampling BP = blood pressure MRA = mineralocorticoid receptor antagonist OSLT = oral salt load confirmatory test PA = primary aldosteronism PAC = plasma aldosterone concentration PCP = primary care provider PRA = plasma renin activity.

Clinical Factors Affecting Length of Stay After 100 Consecutive Cases of Primary Cleft Lip Repair.

OBJECTIVE: To analyze the hospital course of 100 consecutive infants after primary cleft lip repair (PCLR) and identify factors related to length of stay (LOS). DESIGN: Retrospective analysis of 100 consecutive infants who were routinely admitted after PCLR. SETTING: Tertiary care center. PATIENTS: One hundred consecutive infants undergoing PCLR. Demographic and perioperative data were collected and analyzed. MAIN OUTCOME MEASURE: LOS, planned before data collection. RESULTS: Male:female ratio was 65:35. Seventy-two infants had unilateral cleft lip; syndromic association was documented in 15 patients. Mean age and weight at PCLR were 5.6 ± 4.0 months and 6.7 ± 1.3 kg, respectively. Mean duration of surgery was 2.5 ± 0.9 hours, and mean duration of general anesthesia was 3.4 ± 0.9 hours. A total of 3.3 ± 1.5 mL of intraoperative local anesthetic was used per patient. Intravenous fluids were necessary after transfer from the post-anesthesia care unit to the general ward in 98% of patients. Almost half (44%) of all patients received intravenous morphine 23 hours or more after hospital admission. Mean LOS was 35.8 ± 13.9 hours. No association was identified between patient demographic factors and LOS. Multivariate linear regression models identified significant positive correlation between LOS and duration of general anesthesia (P = .002). Greater volume of postoperative oral intake (P = .000) and higher acetaminophen dosage on the floor (P = .000) correlated with decreased LOS. CONCLUSIONS: This study identifies perioperative factors associated with LOS. Our findings question the safety of routine outpatient or short-stay observation after PCLR.

Endocortical plating of the bandeau during fronto-orbital advancement provides safe and effective osseous stabilization.

BACKGROUND: Ectocortical resorbable plate fixation has become a standard method of fixation during fronto-orbital advancement (FOA) in young children. Plate hydrolysis occurs slowly and can cause visible prominences, sterile abscesses, and osseous depressions that can persist after complete resorption. Although endocortical placement avoids contour issues, the safety and effectiveness of this technique are undocumented. METHODS: A review of our prospectively collected craniofacial database was performed. All patients undergoing FOA by a single craniofacial team at a single institution from 1997 to 2011 were examined. Inclusion criteria were as follows: (1) unicoronal, bicoronal, or metopic synostosis; (2) resorbable endocortical fixation of the bandeau; and (3) follow-up for 1 year or longer. Evaluation included patient demographic data, postoperative clinical course, and computed tomography imaging when available. RESULTS: Seventy-three patients met the inclusion criteria. Fusion involved the unicoronal (n = 26), bicoronal (n = 19), and metopic (n = 28) sutures. Mean age at operation was 8.3 months (range, 2.7-35.5 mo), and follow-up was 4.5 years (range, 1.0-9.9 y). No endocortical or ectocortical sterile abscesses were documented in our series. Postoperative complications included hematoma (n = 2), infection (n = 2), wound breakdown (n = 3), cerebral contusion (n = 2), and cerebrospinal fluid leak (n = 1); none of these issues were related to endocortical absorbable fixation. Fifty-eight patients (80%) were categorized as Whitaker classification I/II; and 15 patients (20%), Whitaker classification III/IV. Postoperative computed tomography (mean follow-up, 4.6 y) was obtained in 34 patients (47%). All plates were completely resorbed, and there were no bone or soft tissue irregularities in the region where the plates were placed. CONCLUSIONS: Endocortical resorbable fixation is a safe and effective method of osseous stabilization during FOA for craniosynostosis in young children.

Incidental findings on preoperative computed tomography for nonsyndromic single suture craniosynostosis.

Although the diagnosis of nonsyndromic single suture craniosynostosis (NSSC) can usually be made by clinical examination, computed tomography (CT) is still commonly used in preoperative evaluation. This practice has been questioned in light of recent studies that document a small, but measurable, increased risk of malignancy from CT-associated radiation. The purpose of this study was to examine whether preoperative CT for patients with NSSC provided clinically important information beyond confirmation of craniosynostosis. We performed a retrospective analysis of all patients with NSSC undergoing cranial vault remodeling at our center from March 1999 to March 2011. Only patients with complete preoperative CT scans available for review were included. Staff pediatric neurosurgeons were blinded to patient diagnosis and official radiology report, analyzed the CT images, and documented the site of synostosis and any other findings. Of the 231 patients, 80 met the inclusion criteria. Sites of synostosis included sagittal (51 patients), coronal (17 patients), metopic (11 patients), and frontosphenoidal (1 patient). Clinical diagnosis correlated with radiographic site of fusion in all patients except the patient with frontosphenoidal synostosis. Incidental findings were documented in more than 50% of the patients including prominent extra-axial cerebrospinal fluid (n = 36, 45%), ventriculomegaly (n = 5, 6.25%), choroid fissure cyst (n = 2), cavum septum pellucidum (n = 2), Chiari malformation (n = 1), and prominent perivascular space (clinically nonsignificant finding, n = 1). Incidental findings required additional follow-up or management in 5 patients (6.25%). Our findings support the use of preoperative imaging in this population to identify intracranial anomalies that cannot be discerned by clinical examination. Whereas many findings were not clinically important, some required additional attention.