Rare Hybrid Perineurioma and Granular Cell Tumor: A Pediatric Case.

We present a case of a 13-year-old patient with a distinct tumor with both granular cell and perineurial elements, located on the lower lip. The patient presented with a long-standing lip mass that was clinically felt to most likely represent a mucocele. Following surgical excision, histopathological examination revealed a well-circumscribed tumor composed of granular cells with positive S100 protein staining and spindled cells positive for EMA and GLUT-1, confirming mixed neuroectodermal and perineurial origin. This is the first case documenting a perineurial-granular cell hybrid tumor in a patient under 18 years old, and the first to be reported in the head and neck. This case expands our understanding of hybrid PNSTs, emphasizing the importance of considering diverse clinical presentations, especially in the context of rare pediatric occurrences in atypical locations.

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.

Socioeconomic Influence on Surgical Management and Outcomes in Patients with Craniosynostosis - A Systematic Review.

OBJECTIVE: Disparities in insurance and socioeconomic status (SES) may impact surgical management and subsequent postoperative outcomes for patients with craniosynostosis. This systematic review summarizes the evidence on possible differences in surgical care, including procedure type, age at surgery, and differences in surgical outcomes such as complications, length of hospital stay, and child development based on SES. DESIGN: The databases Scopus, PubMed, and CINAHL were searched between May and July 2022. Following PICO criteria, studies included focused on patients diagnosed with craniosynostosis; corrective surgery for craniosynostosis; comparison of insurance, income, or zip code; and surgical management of postoperative outcomes. RESULTS: The initial search yielded 724 articles. After three stages of screening, 13 studies were included. Assessed outcomes included: type of procedure (6 articles), age at time of surgery (3 articles), post-operative complications (3 articles), referral delay (2 articles), length of stay (2 articles), hospital costs (2 articles), and child development (1 article). Of the studies with significant results, insurance type was the main SES variable of comparison. While some findings were mixed, these studies indicated that patients with public medical insurance were more likely to experience a delay in referral, undergo an open rather than minimally-invasive procedure, and have more complications, longer hospitalization, and higher medical charges. CONCLUSIONS: This study demonstrated that SES may be associated with several differences in the management of patients with craniosynostosis. Further investigation into the impact of SES on the management of patients with craniosynostosis is warranted to identify possible interventions that may improve overall care.

Predicting Failure of Conservative Airway Management in Infants with Robin Sequence: The EARN Factors.

OBJECTIVE: Evaluate infants with Robin Sequence (RS) who were successfully treated with conservative airway measures alone vs. those who failed and eventually underwent surgical airway intervention after a protracted course of conservative management. DESIGN: Retrospective review of prospectively gathered database. SETTING: Large tertiary care institution. PATIENTS: Infants diagnosed with RS (n = 122) who underwent primary airway management at a single institution from 1994-2020. MAIN OUTCOME MEASURE: Patient demographics, nutritional and respiratory status, laboratory values, and polysomnographic results were compared between patients who were discharged after successful conservative airway management (Group 1, n = 61) and patients that underwent surgical airway intervention after failing a prolonged course of conservative management (Group 2, n = 61). Receiver operating characteristic (ROC) curve analysis was done to assess continuous variables that may predict failure of conservative airway management. RESULTS: 122 infants with RS were investigated. While several variables were significantly different between groups, the following polysomnographic EARN factors, with cut points, were identified as most predictive of failed conservative airway management: ETCO2 (max) > 49 mmHg, AHI > 16.9 events/hour, OAHI REM >25.9 events/hour, OAHI Non-REM > 23.6 events/hour. CONCLUSIONS: We identified factors in infants with RS that were associated with severe UAO that failed to improve despite weeks of conservative airway management. Our results may expedite earlier definitive treatment of these critical patients and reduce risks for known complications of prolonged UAO.

Free flap transfer with supermicrosurgical technique for soft tissue reconstruction: A systematic review and meta-analysis.

BACKGROUND: Supermicrosurgery is a technique that allows microsurgeons to accomplish dissections and anastomoses of vessels and nerve fascicles with diameters of ≤0.8 mm. Considering the potential benefits of this technique and limited literature synthesizing the outcomes of supermicrosurgery, the aim of this study was to summarize the available evidence of reconstructive supermicrosurgery and to estimate the success rate. METHODS: We conducted a comprehensive search across PubMed, Scopus, Embase, and Web of Science. We included patient-based studies reporting on procedures for soft-tissue reconstruction with free flaps specifying the use of supermicrosurgery. We excluded studies reporting on lymphatic surgery, solely peripheral nerve surgery, and tissue replantation. Our primary endpoint was to calculate the flap success rate. Pooled estimates were calculated using a random-effects meta-analytic model. RESULTS: Forty-seven studies reporting outcomes of 698 flaps were included for qualitative synthesis. Overall, 15.75% of flaps were used for head and neck, 4.4% for breast and trunk, 9.3% for upper limb, and 69.2% for lower limb reconstruction. The most used flap was the superficial circumflex iliac artery perforator flap (41.5%). The overall flap success rate was 96.6% (95%CI 95.2%-98.1%). The cumulative rate of partial flap loss was 3.84% (95%CI 1.8%-5.9%). The overall vascular complication rate resulting in complete or partial flap loss was 5.93% (95%CI 3.5%-8.3%). CONCLUSIONS: Supermicrosurgery displays a high success rate. Further studies are necessary to explore the true potential of supermicrosurgery. This technique reformulates the boundaries of reconstructive surgery due to its extensive application.

Pharyngeal Arches, Chapter 1: Normal Development and Derivatives.

The pharyngeal arches form the cornerstone of the complex anatomy of the face and neck. These embryologic structures are the foundation of face and neck development, and anomalous growth can result in craniofacial abnormalities. Surgeons who manage head and neck pathology and pathoanatomy will invariably encounter conditions associated with aberrant pharyngeal arch anatomy, and a thorough understanding of the normal and pathological development of these important structures is paramount to accurate diagnosis and treatment. This manuscript is the first of a three-part educational series that addressed the pharyngeal/branchial arch embryology, development, nomenclature, and normal anatomy (Part I), pathologic anomalies of ear and neck derived from abnormal development of the arches (Part II), and different types of orofacial clefts, including Tessier clefts (Part III).

Defining Age-related OSA Features in Robin Sequence Using Polysomnographic-based Analyses of Respiratory Arousal Responses and Gas-exchange Parameters.

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, <1-year old), (b) non-RS older children (1-2 years old) with severe OSA (obstructive AHI (OAHI) of ≥10 events; n = 12), and (c) control infants and children (0-2 years old) without sleep apnea (OAHI ≤1.5/h, n = 12). We examined OSA sleep-stage specific and position-specific indexes, and the relationship between OSA severity and respiratory arousal indexes (OAHI/respiratory arousal indexes). 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.

Timing and Duration of Facial Nerve Dysfunction After Mandibular Distraction Osteogenesis for Robin Sequence.

OBJECTIVE: Collect data from craniofacial surgeons to analyze mandibular distraction osteogenesis (MDO) protocols, and facial nerve dysfunction (FND) to characterize this common, but poorly documented complication after MDO in infants with Robin Sequence (RS). DESIGN, SETTING, AND PARTICIPANTS: A 16-question anonymous survey designed through REDCap was digitally distributed to members of the American Cleft Palate-Craniofacial Association and International Society of Craniofacial Surgery (ISCFS). MAIN OUTCOME MEASURE(S): Demographic information, MDO perioperative variables, surgeon experience with FND after MDO for patients with RS, and the timing and duration of FND were analyzed. RESULTS: Eighty-four responses were collected, with 80 included for analysis. Almost two-thirds of respondent surgeons reported FND as a complication of MDO in patients with RS (51, 63.8%); 58.8% (n = 47) transient FND and 5% (n = 4) with permanent facial nerve palsy only. Both transient and permanent FND was documented by 13 (16.3%) respondents. Among respondents, FND was observed immediately following initial device placement/osteotomies in 45.1%, during distraction in 45.1%, during consolidation in 19.6%, and following device removal in 43.1%. Twenty-five of these respondent surgeons reported resolution of FND between 1 and 3 months (53.2%, n = 25). CONCLUSIONS: FND after MDO in patients with RS was noted by most respondents in this survey study. While most surgeons noted temporary FND, one-fifth reported long-term dysfunction. FND was documented most commonly following device placement/osteotomies or during active distraction. Further research should seek to establish risk factors associated with FND and identify surgical and perioperative prevention strategies.

Facial Nerve Dysfunction After Mandibular Distraction Osteogenesis in Patients with Robin Sequence: A Systematic Review and Meta-Analysis.

OBJECTIVE: Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO. DESIGN AND SETTING: According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: "distraction osteogenesis" and "Robin Sequence". A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it. RESULTS: Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00 mm/day and total distraction length ranged from 13.00 to 22.3 mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04). CONCLUSION: This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND.

Botulinum Toxin to Improve Scar Quality in Cleft Lip Repair: A Systematic Review.

OBJECTIVE: Cleft lip repair (CLR) can be complicated by hypertrophic scar or keloid. Botulinum toxin type A (BTA) may improve postoperative scarring by reducing muscle tension and cytokine activity at the scar site. This systematic review analyzes the available evidence regarding the effect of BTA on scar quality after CLR. DESIGN: The search was conducted in 6 different databases in accordance with PRISMA guidelines (PubMed, Scielo, Embase, Scopus, Web of Science, and Cochrane) using "botulinum toxin" and "cleft lip" as keywords. SETTING: Academic hospital. PATIENTS: Exclusive to patients who underwent CLR and BTA injection. OUTCOME MEASURES: Mean visual analog scores (VAS), mean Vancouver scar scale (VSS), scar width, and BTA or CLR-related complications. RESULTS: Five studies for a total of 216 patients met inclusion criteria. Four studies reported on primary CLR during infancy while 1 study recruited older patients seeking revision. All patients had BTA (range: 1-2 units/kg) injected in the orbicularis oris muscle. One study documented BTA injections in additional perioral muscles. All 4 studies that measured scar width and had a saline control arm found a significant decrease in width with BTA injection. Improvement of VAS and VSS with BTA was reported in 3 of 5 studies and 2 of 5 studies, respectively. There were no reports of complications associated with BTA or CLR. CONCLUSION: The existing studies support the use of BTA injection to improve scar quality following CLR with low concern for complication. Further investigations with a greater number of patients are necessary.

Administration of Intravenous Dexmedetomidine and Acetaminophen for Improved Postoperative Pain Management in Primary Palatoplasty.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Safety and Efficacy of Single-Dose Ketorolac for Postoperative Pain Management After Primary Palatoplasty: A Prospective Cohort Study With Historical Controls.

OBJECTIVE: To analyze safety and efficacy of single-dose ketorolac after primary palatoplasty (PP). DESIGN: Consecutive cohort of patients undergoing PP, comparing to historical controls. Setting: A large academic children's hospital. PATIENTS, PARTICIPANTS: A consecutive cohort of 111 patients undergoing PP (study n = 47) compared to historical controls (n = 64). INTERVENTIONS: All patients received intraoperative acetaminophen, dexmedetomidine, and opioids while the study group received an additional single dose of ketorolac (0.5 mg/kg) at the conclusion of PP. MAIN OUTCOME MEASURES: Safety of ketorolac was measured by significant bleeding complications and need for supplementary oxygen. Efficacy was assessed through bleeding, Face Legs Activity Cry Consolability (FLACC) scale, and opioid dose. RESULTS: Length of stay was similar for both groups (control group 38.5 hours [95% CI: 3.6-43.3] versus study group 37.6 hours [95% CI: 31.3-44.0], P = .84). There were no significant differences in all postoperative FLACC scales. The mean dose of opioid rescue medication measured as morphine milligram equivalents did not differ between groups (P = .56). Significant postoperative hemorrhage was not observed. CONCLUSIONS: This is the first prospective study to evaluate the safety and efficacy of single-dose ketorolac after PP. Although lack of standardization between study and historical control groups may have precluded observation of an analgesic benefit, analysis demonstrated a single dose of ketorolac after PP is safe. Further investigations with more patients and different postoperative regimens may clarify the role of ketorolac in improving pain after PP.

Administration of Single-Dose Antibiotic Does Not Decrease Oronasal Fistula Rates After Primary Palatoplasty.

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.

Repair of Pediatric Split Earlobe Deformity With Anterior Straight-Line Closure and Posterior Z-Plasty: Surgical Technique and Review of 26 Cases.

BACKGROUND: Split earlobe deformity typically results from earring-related trauma or, less commonly, from congenital malformation. Several surgical approaches to repair the earlobe have been described, with the goals of reconstructing normal contour of the earlobe, avoiding notching of the free margin, and minimizing scar visibility. METHODS: The authors reviewed 26 consecutive patients who underwent earlobe reconstruction using the senior author's novel technique, which involves anterior straight-line closure paired with a posterior Z-plasty. Baseline demographic characteristics, etiology of split earlobe, follow-up, outcomes, and rate of complications were analyzed. RESULTS: The median age at surgical repair was 8.04 (interquartile range, 4.53-12.84) years. Most patients had acquired split earlobe deformity secondary to trauma. Median follow-up was 86.5 (interquartile range, 29-385.5) days. Only 4 patients had less than satisfactory results, 3 having residual contour abnormality and 1 noting earlobe asymmetry. None of these patients desired revision. No keloid formation, dehiscence, or other postoperative complications were reported. CONCLUSIONS: The authors describe a novel and effective surgical technique that successfully reconstructs split earlobe deformity. This technique minimizes visible scarring with an anterior straight-line repair combined with posterior Z-plasty to avoid notching.

Dental Topics for Plastic Surgeons, Part Four: Common Cysts and Tumors of the Jaw.

ABSTRACT: Conditions that affect dental and periodontal structures receive sparse coverage in the plastic surgery literature, yet a working knowledge of this subject matter is important in certain areas of clinical practice and a fundamental understanding is part of plastic surgery competency tested on the in-service and written board examinations. This 4-part series was written to provide plastic surgeons with a working knowledge of dental topics that may be relevant to their clinical practice. This section, Part IV, covers common dental cyst and tumors of the jaw.

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.

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.

Dental Topics for Plastic Surgeons, Part Three: Infection and Trauma of the Dentition.

ABSTRACT: Conditions that affect dental and periodontal structures receive sparse coverage in the plastic surgery literature, yet a working knowledge of this subject matter is important in certain areas of clinical practice and a fundamental understanding is part of plastic surgery competency tested on the in-service and written board examinations. This 4-part series written to provide plastic surgeons with a working knowledge of dental topics that may be relevant to their clinical practice. This section, Part III, covers inflammatory and infectious conditions of the dentition and related structures, as well as dentoalveolar trauma.

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.

Occult Scaphocephaly: A Forme Fruste Phenotype of Sagittal Craniosynostosis.

INTRODUCTION: Latent cranial suture fusions may present with mild or absent phenotypic changes that make the clinical diagnosis challenging. Recent reports describe patients with sagittal synostosis and a normal cranial index (CI), a condition termed normocephalic sagittal craniosynostosis (NSC). The goal of this study is to evaluate the shape and intracranial volume (ICV) in a cohort of NSC patients using quantitative cranial shape analysis (CSA). METHODS: We identified 19 patients (7.5 ±â€Š2.28 years) between 2011 and 2016, who presented to our hospital with NSC. Cranial index and CSA were measured from the computed tomography image. Cranial shape analysis calculates the distances between the patient's cranial shape and its closest normal shape. Intracranial volume was measured and compared to an established age-matched normative database. RESULTS: Cranial index revealed 15 (78.9%) patients within the mesocephalic range and 4 patients (21.1%) in the brachycephalic range. Detailed CSA identified 15 (78.9%) patients with subtle phenotypic changes along the scaphocephalic spectrum (ie, subtle anterior and posterior elongation with inter-parietal narrowing) and 1 patient (5.3%) with isolated overdevelopment on the posterior part of the right parietal bone. Three patients (15.8%) had a CSA close to normal. Mean ICV was 1410.5 ±â€Š192.77cc; most patients (78.9%) fell within ±2 standard deviations. CONCLUSION: Quantitative CSA revealed that most of the patients with NSC had cranial shape abnormalities, consistent with a forme fruste scaphocephaly that could not be otherwise recognized by clinical observation or CI. Given these findings, we propose the term occult scaphocephaly to describe this condition. The associated incidence of intracranial hypertension is unknown.