Pediatric Cranioplasty Patients With Hostile Reconstructive Environments: Split Calvarial Versus Prosthetic Implant.

INTRODUCTION: Pediatric cranial defects can be preceded by prior infection, radiation therapy, failed prior cranioplasty, or cerebrospinal fluid leak, leading to a complex reconstructive environment. The primary aim of this study was to investigate differences in outcomes between pediatric patients with hostile reconstructive environments who received split-calvarial autologous grafts as opposed to prosthetic grafts in cranioplasty. METHODS: We performed an institutional review board-approved retrospective chart review of 51 patients younger than 18 years who underwent cranioplasty with a hostile setting between 1998 and 2020. Patients were then stratified into prosthetic (45%) and autologous groups (54%). The primary outcome measured was postoperative complication, defined as requirement of a subsequent surgery or revision. RESULTS: Overall, there were no significant differences in age, sex, type of hostile setting, etiology of cranial defect, or side of the cranial defect between the 2 groups. Complication rate among the 2 graft groups was 18%. However, there were no significant differences in complications, defined as infection, failure or resorption of the graft, wound breakdown or necrosis, resulting bone defect, or hematoma, between the 2 populations. There was a significant difference in etiology between patients with complications, with patients who required a cranioplasty due to previous hemicraniectomy being nearly 5 times as likely to face a complication ( P = 0.045). CONCLUSIONS: In our study, there was no significant difference observed in complications between prosthetic and split-thickness autologous grafts in pediatric patients with hostile settings. It does, however, seem that patients who had a previous hemicraniectomy are more likely to face complications as a result of cranioplasty.

The utility and post-operative evolution of head circumference in nonsyndromic single-suture craniosynostosis: a prospective study in Vietnamese children.

PURPOSE: Head circumference (HC) is an important clinical tool for following head growth in children with craniosynostosis (CS). The purpose of this study is to quantify the usefulness of HC along continuum of CS care, from diagnosis to pre- and post-operative (pre-op, post-op) follow-up in Vietnamese children. METHODS: A prospective cohort of 54 nonsyndromic single-suture CS patients undergoing open surgery from January 2015 to January 2020 was collected at Children's Hospital 2, Vietnam. HC z-score on admission was compared with World Health Organization (WHO) standards to evaluate for utility in initial diagnosis. Pre-op and post-op HC were compared to demonstrate the evolution of head growth following reconstruction. RESULTS: Nonsyndromic single-suture CS was more predominant in males (79.6%) than in females (20.4%). The mean HC z-score was - 0.38 [Formula: see text] 1.29 similar to normal WHO standards regardless of which sutural involvement. The HC z-score increased above + 1 standard deviation (SD) significantly at 3 months of follow-up (p < 0.001); however, the trajectory gradually decreased after the first year of surgery. One patient (1.8%, 1/54) demonstrated restenosis and delayed intracranial hypertension (DIH) 4 years after reconstruction. CONCLUSIONS: The HC in nonsyndromic single-suture CS children presents similarly to the values of healthy children. Additionally, HC reliably increased after reconstruction and gradually normalized over subsequent years. This indicator is consistent in Southeast Asian populations and should be used to follow all patients to assess the normal progression of post-op head growth and as a useful indicator of suspected recurrent synostosis.

Comorbidity Trends in Patients Requiring Sternectomy and Reconstruction: Updated Data Analysis From 2005 to 2020.

INTRODUCTION: Comorbidity trends after median sternectomy were studied at our institution by Vasconze et al (Comorbidity trends in patients requiring sternectomy and reconstruction. Ann Plast Surg. 2005;54:5). Although techniques for sternal reconstruction have remained unchanged, the patient population has become more complex in recent years. This study offers insight into changing trends in this patient population. METHODS: A retrospective review was performed of patients who underwent median sternectomy followed by flap reconstruction at out institution between 2005 and 2020. Comorbidities, reconstruction method, average laboratory values, and complications were analyzed. RESULTS: A total of 105 patients were identified. Comorbidities noted were diabetes (27%), immunosuppression (16%), hypertension (58%), renal insufficiency (23%), chronic obstructive pulmonary disease (16%), and tobacco utilization (24%). The most common reconstruction methods were omentum (45%) or pectoralis major flaps (34%). Thirty-day mortality rates were 10%, and presence of at least 1 complication was 34% (hematoma, seroma, osteomyelitis, dehiscence, wound infection, flap failure, and graft exposure). Univariate analysis demonstrated that sex (P = 0.048), renal insufficiency, surgical site complication, wound dehiscence, and flap failure (P < 0.05) had statistically significant associations with mortality. In addition, body mass index, creatinine, and albumin had a significant univariate association with mortality (P < 0.05). CONCLUSIONS: Similar to the original study, there is an association between renal insufficiency and mortality. However, the mortality rate is decreased to 10%, likely because of improved medical management of patients with increasing comorbidities (80% with greater than one comorbidity). This has led to the increased use of omentum as a first-line option. Subsequent wound dehiscence and flap failure demonstrate an association with mortality, suggesting that increasingly complex patients are requiring a method of reconstruction once used a last resort as a first-line option.

Outcomes of Cranioplasty Strategies for High-Risk Complex Cranial Defects: A 10-Year Experience.

INTRODUCTION: Although the literature contains reports of the risks and complications of calvarial vault reconstruction for acquired defects, there are few publications addressing the specific patient population who require such reconstructions in cases preceded by prior infection, radiation, massive associated soft tissue trauma, and so on. We define such clinical presentations as a hostile environment for large surface area reconstruction. Our objective is to compare the safety and efficacy of autologous bone and alloplastic reconstruction in hostile cranial defects. METHODS: An institutional review board-approved retrospective review of patients who underwent cranioplasty of a hostile site at the University of Alabama at Birmingham between January 2008 and December 2018 was performed. The patients were stratified into 3 groups based on the type of implant used: autogenous (bone), alloplastic (polyetheretherketone [PEEK], titanium, polymethyl methacrylate), or mixed (combination of bone and prosthetic). The primary outcome metric was a complication in the year after cranioplasty, identified by implant failure, necrosis, or infection. Statistical analysis included t tests and χ2 tests where appropriate using SPSS. RESULTS: There were 55 total cases in this period: 27 autogenous, 23 alloplastic, and 5 mixed. The purely autogenous group had the highest complication rate (44%), and the alloplastic group had the lowest complication rate (38%), which was not statistically different between the 3 groups (P = 0.121). When stratified by specific material used for reconstruction (27 bone, 14 PEEK, 10 titanium, and 5 polymethyl methacrylate), overall complication rate was statistically significant (P = 0.009, χ2 test), with PEEK implants having the lowest complication rate (21%). CONCLUSIONS: This analysis interestingly found that in the setting of hostile cranial defects, cranioplasties would benefit from the use of prosthetic implants instead of autologous bone grafts, not only for avoidance of donor site morbidity but also for a decrease in overall complications.

Nonsyndromic craniosynostosis in Vietnam: initial surgical outcomes of subspecialty mentorship.

OBJECTIVE: There is a global deficit of pediatric neurosurgical care, and the epidemiology and overall surgical care for craniosynostosis is not well characterized at the global level. This study serves to highlight the details and early surgical results of a neurosurgical educational partnership and subsequent local scale-up in craniosynostosis correction. METHODS: A prospective case series was performed with inclusion of all patients undergoing correction of craniosynostosis by extensive cranial vault remodeling at Children's Hospital 2, Ho Chi Minh City, Vietnam, between January 1, 2015, and December 31, 2019. RESULTS: A total of 76 patients were included in the study. The group was predominantly male, with a male-to-female ratio of 3.3:1. Sagittal synostosis was the most common diagnosis (50%, 38/76), followed by unilateral coronal (11.8%, 9/76), bicoronal (11.8%, 9/76), and metopic (7.9%, 6/76). The most common corrective technique was anterior cranial vault remodeling (30/76, 39.4%) followed by frontoorbital advancement (34.2%, 26/76). The overall mean operative time was 205.8 ± 38.6 minutes, and the estimated blood loss was 176 ± 89.4 mL. Eleven procedures were complicated by intraoperative durotomy (14.5%, 11/76) without any damage of dural venous sinuses or brain tissue. Postoperatively, 4 procedures were complicated by wound infection (5.3%, 4/76), all of which required operative wound debridement. There were no neurological complications or postoperative deaths. One patient required repeat reconstruction due to delayed intracranial hypertension. There was no loss to follow-up. All patients were followed at outpatient clinic, and the mean follow-up period was 32.3 ± 18.8 months postoperatively. CONCLUSIONS: Surgical care for pediatric craniosynostosis can be taught and sustained in the setting of collegial educational partnerships with early capability for high surgical volume and safe outcomes. In the setting of the significant deficit in worldwide pediatric neurosurgical care, this study provides an example of the feasibility of such relationships in addressing this unmet need.

Reconstructive Management of Gunshot Wounds to the Frontal Sinus: An Urban Trauma Center's Perspective.

INTRODUCTION: In the last decade, we have seen a steady increase in the incidence of frontal sinus trauma due to gunshot wounds and a decrease in motor vehicle trauma. Penetrating gunshot wounds to the frontal sinus present a unique challenge to the reconstructive surgeon because they require careful consideration of the management principles of plastic surgery. Despite previous reviews on frontal sinus trauma, there are no studies examining the management techniques of frontal sinus fractures due specifically to gunshot wounds. In this study, we aim to retrospectively evaluate the use of a variety of tissue flaps in intervention and associated outcomes. METHODS: A retrospective chart review was completed on all patients with gunshot wound(s) to the frontal sinus from January 2010 to January 2018 at a single institution. The patients were classified based on the fracture pattern (anterior vs posterior table vs both), degree of displacement, presence of nasofrontal outflow tract injury, and evidence of cerebrospinal fluid leak. Patients were then stratified according to the type of reconstruction performed (cranialization, obliteration and need for free flap) and evaluated for major and minor complications after reconstruction. RESULTS: In this study, we present outcome data from 28 cases of frontal sinus trauma due to gunshot wounds. There was a statistically significant difference (P = 0.049) in the type reconstructive strategy employed with each type of flap, with pericranial flaps primarily used in cranialization, temporal grafts were more likely to be used in obliteration, and free flaps were more likely to be used in cranialization. The overall major complication rate was 52% (P = 0.248), with the most common acute major complication was cerebrospinal fluid leak (39%) and major chronic was abscess (23.5%). CONCLUSIONS: This report explores the management of frontal sinus trauma and presents short-term outcomes of treatment for penetrating gunshot wounds at a tertiary referral center.

About the One Hemi-Uvula Repair Technique During Palatoplasty.

This is a response to a letter replying to our paper discussing the significance of the uvula during palatoplasty and our surgical technique of uvula repair. The hemi-uvula repair technique was developed independently in our department. In our publication, we fully cited the 2 studies by Rossell-Perry et al about uvula repair and acknowledged them to be the first to publish the concept of using one hemi-uvula. Upon further study, the mistake of omitting 2 references from the list, although they were cited in the text, was found to have occurred during the transition between publishers of this journal (corrected in Erratum). We highlighted some of the differences in the studies and techniques, which are already discussed in our paper, for further clarification.

Incidence of delayed intracranial hypertension in children with isolated sagittal synostosis following open calvarial vault reconstruction.

PURPOSE: Delayed intracranial hypertension (DIH) occurs most frequently in children with syndromic or multi-suture synostosis after surgical correction. The rarity of DIH in children with isolated non-syndromic sagittal synostosis (ISS) warrants follow-up evaluation by large craniofacial centers until skeletal maturity. This study reports the incidence of DIH in children following open repair for ISS by our center's craniofacial team. METHODS: A single-center retrospective study of patients who underwent open calvarial vault remodeling (CVR) for ISS at our institution between November 2000 and November 2012 was performed. Syndromic and multi-suture synostosis patients were excluded. Demographic and follow-up data were extracted from the medical record for analysis until July 2017. RESULTS: One hundred five patients with ISS were identified who had undergone CVR in the aforementioned timeframe. Average age at initial surgery was 11.7 ± 15.32 months. Mean follow-up in our craniofacial clinic was 4.94 ± 3.53 years, with 69 patients (65.7%) having follow-up in craniofacial clinic ≥ 3 years and 74 (70.5%) having follow-up ≥ 3 years in any clinic at our institution. Four patients (3.8%) had intracranial pressure (ICP) monitors placed for symptoms concerning for DIH, one of which (0.95%) had confirmed DIH and underwent a second surgical procedure at 7.4 years of age. The patient presented late initially, having his first operation at 1.56 years of age. CONCLUSION: One patient out of 105 (0.95%) developed DIH, confirmed by ICP monitoring, and required reoperation. The occurrence of DIH, albeit rare, remains an important topic to include in parental discussions and mandates long-term follow-up in this population.

Outcome of Palate Re-repair with Radical Repositioning of the Levator Muscle Sling as a First-Line Strategy in Postpalatoplasty Velopharyngeal Incompetence Management Protocol.

BACKGROUND: Palatal re-repair aims to improve velar function by retropositioning the levator muscles. Although it has become a popular procedure, very few studies document its efficacy. To date, this is the largest series reported to clarify its indications and efficacy. METHODS: One hundred eighty-three consecutive cleft patients presenting with velopharyngeal incompetence and evidence of abnormally oriented levator muscles underwent palate re-repair (regardless of the gap size) performed by a single surgeon from 2000 to 2015. Perceptual speech assessment was performed using the Pittsburgh Weighted Speech Score. Other patients' demographic data were collected. RESULTS: Complete records of 111 patients were available. Eighteen cases were syndromic (18.9 percent). Postoperatively, there was highly significant improvement (p < 0.001) in nasal emission (from 2.24 to 0.64), nasality (from 3.44 to 1.27), articulation (from 5.32 to 2.01), and total score (from 11.29 to 4.11). Speech became normal/borderline normal, improved or did not improve in 66.7, 24.3, and 9 percent of patients, respectively. An initial diagnosis of isolated cleft palate, Caucasians, intravelar veloplasty in the primary repair, older patients, and nonsyndromic cases were associated with better outcome. There were no reported cases of postoperative fistula or new obstructive sleep apnea. CONCLUSIONS: This large series study provides confirmatory evidence of the effectiveness and safety of the re-repair procedure. It is recommended as a first-line procedure in all velopharyngeal incompetence cases with abnormally oriented levator muscles regardless of gap size, even if the primary operation included prior muscle dissection. The pharyngoplasty rate could be significantly reduced with the current protocol. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

The Significance of Uvula After Palatoplasty: A New Technique to Improve the Aesthetic Outcome.

OBJECTIVE: This study aims to examine the importance of the uvula as a part of palatoplasty outcome and to assess the aesthetic results of the conventional versus a new technique for uvuloplasty. DESIGN/PARTICIPANTS: The study included 2 groups of patients undergoing palatoplasty. Group I consisted of 20 cleft palate patients repaired with the conventional uvula repair, combining the 2 hemi-uvulae. Group II consisted of 20 patients repaired with our new technique, sacrificing one hemi-uvula and centralizing the remaining one. The aesthetic outcome was assessed in both groups. A questionnaire was distributed to the families of both groups to assess their concern about the uvula after palate repair. SETTING: Cleft unit at a tertiary care center. RESULTS: Sixty-five percent of parents considered the uvula as important functionally and aesthetically after palate repair whereas 35% either did not care or were not sure about its importance. Results of the aesthetic outcome of the 2 techniques for uvula reconstruction showed that uvula was absent in 4 cases in group I versus 1 in group II ( P > .05), small in 8 cases of group I versus 4 in group II ( P > .05), bifid in 5 cases of group I versus none in group II ( P < .05), became deviated in no case of group I versus 4 in group II ( P > .05), and was satisfactory in 3 cases of group I versus 11 in group II ( P < .05). CONCLUSIONS: Among the respondents, the uvula was a significant concern to the parents of cleft patients and should be given more attention during repair. The described technique had better aesthetic outcome over the conventional one of combining the 2 hemi-uvulae.

Time course of improvement after re-repair procedure for VPI management.

OBJECTIVES: Palatal re-repair aims to improve velar function by retro-positioning the levator veli palatini muscles. The surgery includes extensive dissection, leading to tissue edema and scar formation which may need time to remodel. Together with the change of muscle orientation and tension, it is expected that a period of time is needed to reach the final functional performance. This study attempts to determine how much time is required to reach the optimum performance of the palate after re-repair. METHODS: A retrospective chart review identified consecutive cleft patients with VPI who underwent palate re-repair procedure by a single surgeon from 2000 to 2015 and achieved normal or borderline normal VP function. Only patients who had regular postoperative follow-up visits for speech assessments until resolution of speech abnormalities were included. The percent of patients cured at each time point following surgery was recorded. RESULTS: Forty-five patients met the inclusion criteria. The mean age at surgery was 6.6 ± 3.2 years. Speech abnormalities had resolved in 44.5% of patients within the first 6 months after surgery, 62.2% after up to 1 year, 75.6% after up to 2 years and 88.9% after up to 3 years post re-repair palatoplasty. The remaining 11.1% continued to improve after 3 years up to 6 years. CONCLUSIONS: Re-repair procedures may take a longer time than previously thought for the final outcome to manifest. Close monitoring of improvement with continued speech therapy is recommended before deciding to move to the next surgical step in management.

Total Palatal Mobilization and Multilamellar Suturing Technique Improves Outcome for Palatal Fistula Repair.

BACKGROUNDS: The success rate of the surgical repair of palatal fistula after palatoplasty is often unsatisfactory. This study is a review of 15 years of single surgeon's experience with the evolution of a reliable surgical technique with high success rate. METHODS: This is a retrospective chart review of consecutive cleft cases undergoing repair of palatal fistula from 2000 to 2015. The study included 37 consecutive fistula repair cases with wide elevation and mobilization of the palatal tissues and nasal and oral layer repair. Group 1 (n = 20) were treated earlier in the study using either midline, von Langenbeck, or 2-flap palatoplasty with 3-layer suturing. Group 2 (n = 17) were treated through a Dorrance-type incision and additional repair of the oral periosteum for a total of 4-layer suturing. RESULTS: The overall fistula closure rate was 94.6% (90% in group 1 and 100% in group 2). The difference in outcome between the 2 groups was statistically insignificant (P > 0.05). Most patients (83.8%) had concomitant velar muscle retropositioning for treatment of velopharyngeal incompetence. CONCLUSIONS: Fistula repair using wide mobilization of the entire palate through previous repair incisions and multilamellar suturing technique has a very low fistula recurrence rate. Addition of the fourth layer of suturing and the use of a Dorrance-type incision further improves the outcome. This approach provides wide tissue release and access to tissue layers for better repair and tension-free closure. Combining intravelar veloplasty with fistula repair is safe and allows management of the fistula and its possible consequences on palatal function in a single procedure.

IRF6 Sequencing in Interrupted Clefting.

In a retrospective review of patients seen at the University of Alabama at Birmingham Cleft and Craniofacial Center, four patients with rare interrupted clefting were identified who had undergone genetic testing. Each of these patients had a typical cleft lip, with intact hard palate and cleft of the soft palate. Given this picture of mixed clefting, IRF6 sequencing was done and was negative for mutations in all four patients. As genetic testing for single-gene mutations and exome sequencing become clinically available, it may be possible to identify novel mutations responsible for this previously unreported type of interrupted clefting.

Effectiveness and safety of autologous fat grafting to the soft palate alone.

BACKGROUND: Posterior pharyngeal augmentation is an accepted method of treating velopharyngeal insufficiency (VPI). Techniques using autologous fat harvest, preparation, and grafting are well described. Based on the complications from retropharyngeal injection, we performed augmentation of the nasal surface of the palate to reduce hypernasality with decreased risks. METHODS: After Institutional Review Board approval, a chart review from 2010 to 2013 identified 46 patients with cleft palate, subjective and nasoendoscopic evidence of VPI treated with autologous fat grafting to the soft palate. Speech evaluation of velopharyngeal function was compared before and after autologous fat grafting. RESULTS: A total of 61 autologous fat grafting procedures were performed in 46 patients. The average age of the study population is 5.59 ± 2.05 years. The majority underwent a single procedure (32/46 or 69.6%), 13 of 46 patients (28.2%) had 2 fat grafting procedures and only 1 patient (2.2%) had 3 fat grafting procedures. The fat was injected primarily in the soft palate. The recorded volume of fat grafted averaged 2.4 ± 1.1 mL. Average operative time was 39 ± 12.55 minutes. There were no local or donor site complications. Four patients were lost to follow-up. Of 34 patients with adequate speech follow-up, including Pittsburgh Weighted Speech Scale (PWSS) assessment, the average preoperative score of 8.17 ± 3.59 was reduced to 5.17 ± 3.14 postoperatively. Although 26 of 34 patients (76.5%) had an improvement in their PWSS score, only 13 of 34 patients (38.23%) saw an improvement in their PWSS category. CONCLUSIONS: Autologous fat grafting to the soft palate is a safe operation with minimal risks. Speech outcomes are subjectively enhanced in the majority of patients, with a full PWSS category improvement seen in 40% of the cases. Patient selection criteria to optimize results are provided.

Multidisciplinary staged surgical management of bifrontal meningoencephalocele with long-term follow-up.

The authors report on an infant with a bifrontal encephalocele that was associated with multisuture craniosynostosis, spasticity, and a progressively severe epilepsy. They describe the initial presentation, genetic screening results, staged multidisciplinary operative plans, clinical course, complications, and long-term surgical and developmental follow-up. To their knowledge, the comprehensive surgical management of this type of complicated congenital cranial anomaly has not been previously described. Surgical management was staged and multidisciplinary and required careful attention to all 3 components of the condition: 1) hydrocephalus, 2) frontal meningoencephalocele, and 3) epilepsy.

IRF6 mutations in mixed isolated familial clefting.

Mutations in the interferon regulatory factor 6 (IRF6) gene are known to cause van der Woude syndrome (VWS), a common syndromic form of oro-facial clefting characterized by the familial occurrence of mixed clefting (cleft lip with or without a cleft palate and cleft palate alone in the same family) and lower lip pits. As lip pits are not present in all cases of VWS, IRF6 mutations can cause a phenotype identical to non-syndromic clefting. However, recent studies failed to identify IRF6 mutations in sporadic and familial non-syndromic clefting, concluding that testing for IRF6 was not warranted for sporadic or familial non-syndromic clefting. Here we report on two families that demonstrate familial mixed clefting in which mutations in IRF6 were identified, suggesting that IRF6 testing does have a role in familial, non-syndromic OFC.

Further delineation of the Kapur-Toriello syndrome.

The Kapur-Toriello syndrome (KTS) is a rare multiple congenital anomaly syndrome, with presumed autosomal recessive inheritance based on the initial report of affected siblings. Here we report on a female with multiple anomalies, including cleft lip and palate, coloboma, mental retardation with cerebral atrophy, and imperforate anus who, upon re-evaluation at 30 months, was recognized to have a columella that extended below the nares. This distinctive finding prompted the diagnosis of KTS. This is the 5th report of KTS, and the second female. Interestingly, both female cases also manifest an ano/rectal malformation, suggesting that this should be considered a component manifestation of this rare syndrome.

Retrospective review of 99 patients with secondary alveolar cleft repair.

PURPOSE: The purpose of the present review was to evaluate the protocol and technique used in a large population of patients with cleft lip and palate when secondary grafting is performed during the early mixed dentition stage, as determined by eruption of the central incisor. In the United States, most investigators have recommended alveolar grafting at the 9- to 11-year age range or before eruption of the permanent canines. MATERIALS AND METHODS: An institutional review board-approved chart review of 99 patients undergoing alveolar cleft bone grafting during a 7-year period at a single institution was performed. Data were collected regarding demographics, operative time, length of hospitalization, follow-up time, complications, and additional procedures performed. The cases were divided by patient age into 2 groups: group 1, aged 6 to 8 years (n = 61); and group 2, aged 9 years and older (n = 38). Statistical analysis was performed for various comparisons in the study. RESULTS: The average operative time for groups 1 and 2 was 86 and 103 minutes, respectively. The complication rate, length of stay, and follow-up time between the 2 groups was not statistically significant at the P = .05 significance level. CONCLUSIONS: We recommend earlier bone grafting at or before the eruption of the central incisor, rather than delaying until the cuspid tooth root is 25% formed. We believe this will provide better bone support for the dentition, a decreased burden of treatment for the patient, and improved quality of life. Performing the procedure at this time can lead to decreased operative times, with comparable postoperative outcomes.