A Unique Case Report of Meningeal Hamartoma Within Alveolar Cleft.

This case report demonstrates a rare finding of a pediatric patient with Morning Glory anomaly and Moyamoya Disease with a palatal meningeal hamartoma discovered as a mass within a previously repaired incomplete cleft of the alveolus. Oral meningeal hamartomas are exceedingly rare with only two palatal cases described and none within a cleft palate or alveolus. These findings prompt a review of oral hamartomas with meningeal subclassification. Further discussion describes the relationship of the proposed origins of meningeal hamartomas within the setting of cleft palate development.

Subglossopalatal Membrane With Associated Cleft Palate, Cardiovascular, and Neurologic Anomalies.

ABSTRACT: Subglossopalatal membrane (or subglossopalatal synechia) is a rare clinical entity that can lead to respiratory distress and feeding difficulty due to oral obstruction. Here, the authors present a case of subglossopalatal membrane with associated cleft palate and cardiovascular and neurologic anomalies that was treated with surgical excision and lip-tongue adhesion. Etiology of these membranes is believed to be intrauterine fetal insult. Membranes should be treated with excision, whereas taking care to ensure patency of the airway. Presence of a subglossopalatal membrane should prompt thorough examination for additional congenital anomalies.

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

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

Comparison of Pre- and Postoperative Sleep Studies in Patients Undergoing Sphincter Pharyngoplasty.

OBJECTIVE: To determine if sphincter pharyngoplasty changes sleep study parameters on patients undergoing surgery for velopharyngeal insufficiency (VPI). DESIGN: Retrospective chart review on patients undergoing sphincter pharyngoplasty for VPI with pre- and postoperative polysomnography completed. SETTING: Institutional study at a tertiary pediatric hospital. PATIENTS: All patients who underwent sphincter pharyngoplasty over a 20-year period were reviewed; all patients with both pre- and postoperative sleep studies were collected for evaluation. INTERVENTIONS: Sphincter pharyngoplasty for patients with VPI. MAIN OUTCOME MEASURE: Sleep study parameters collected include apnea-hypopnea index (AHI), obstructive and central apneas, hypopneas, and mixed events. The preoperative values were compared to postoperative values. RESULTS: There were 98 patients collected with sleep studies for review. Of these, 32 patients had both pre- and postoperative sleep studies. The AHI increased from 1.8 preoperatively to a postoperative value of 4.8 ( P = .004). The number of obstructive events per night went from 4.6 to 17.6 postoperatively ( P = .04). The number of hypopneas increased from 4.0 to 13.6 ( P = .003). The other parameters were not statistically different, central events decreased from 4.8 to 2.1 ( P = .086), and mixed events were essentially unchanged from 0.2 to 0.5 ( P = .17) events per night. CONCLUSIONS: Patients undergoing sphincter pharyngoplasty for VPI may experience an increase in their postoperative AHI, obstructive events per night, and hypopneas. Sleep studies should be performed both pre- and postoperatively to assess the need for intervention or change in management.

Effect of Preoperative Molding Helmet in Patients With Sagittal Synostosis.

BACKGROUND: In our practice, the authors found that molding helmet used for plagiocephaly preoperatively, in patients with sagittal synostosis, decreased bathrocephaly, forehead bossing, and improved posterior vertex, as well as Cephalic Index (CI). This prompted us to investigate the impact of preoperative molding helmet in patients with sagittal synostosis. METHODS: A prospective study was performed on patients undergoing surgical correction of sagittal synostosis, over a 5-year period. Patients were categorized into 2 groups. "No Helmet group" only had surgical correction, and "Helmet group" had preoperative molding helmet, prior to surgical correction. Cephalic Index for the 2 groups was compared using t-test. RESULTS: There were 40 patients in the No Helmet group and 18 patients in the Helmet group. For No Helmet group, mean CI at presentation, immediately preoperative, and postoperatively was 0.70 (±0.045), 0.70 (±0.020), and 0.80 (±0.030), respectively, and for Helmet group, it was 0.69 (±0.023), 0.73 (±0.036), and 0.83 (±0.036), respectively. There was no statistically significant difference between CI of the 2 groups at presentation (P = 0.45). Comparison of postoperative CI did show a statistically significant difference between the groups (P = 0.01). For Helmet group, on comparison of CI at presentation and preoperative CI (after helmet therapy), a statistically significant improvement in CI was observed (P = 0.0004). CONCLUSION: Our results suggest that preoperative molding helmet can decrease bathrocephaly, forehead bossing, and improve posterior vertex as well as CI, prior to surgery and thus can be used as a valuable adjunct in patients with sagittal synostosis.

Aprotinin and transfusion requirements in pediatric craniofacial surgery.

OBJECTIVE: To evaluate transfusion requirements in children receiving aprotinin during craniofacial surgery. BACKGROUND: Pediatric craniofacial procedures may involve massive blood loss. Aprotinin may decrease perioperative blood loss and transfusion requirements. METHODS: Patients (aged 1 month to 3 years) who had major reconstructive craniofacial surgery received intraoperative aprotinin (13 patients) or placebo (13 patients). Administered colloids and blood products were recorded. RESULTS: Patients in the aprotinin and placebo groups had similar mean age, body weight, body surface area, operative time, and length of hospital stay. Mean volumes of total colloids (aprotinin group: 70 ± 40 ml; and placebo group: 120 ± 80 ml; P ≤ 0.05) and packed red blood cells (aprotinin group: 380 ± 90 ml; and placebo group: 550 ± 200 ml; P ≤ 0.004) were less in the aprotinin group than in the placebo group. Mean urine output during surgery was greater in the aprotinin group than in the placebo group (320 ± 200 ml vs 150 ± 70 ml, respectively; P ≤ 0.003). Mean blood urea nitrogen and serum creatinine values after surgery were similar between the groups. Complications of aprotinin included anaphylaxis (one patient) and rash (one patient); no deaths occurred. CONCLUSIONS: Aprotinin was associated with decreased packed red blood cell transfusion requirements in children undergoing craniofacial surgery, with no renal toxicity or death. Aprotinin is no longer available for clinical use in the USA because of adverse effects in adults; re-evaluation of aprotinin is warranted for children scheduled to undergo surgery involving potentially high blood loss.

Endoscopy in Craniosynostosis Surgery: Evolution and Current Trends.

Over the past 30 years, advances in endoscopic technology and advancing interest in the benefits of minimally invasive approaches for craniofacial surgery have resulted in these techniques becoming a part of the standard of care in the treatment of craniosynostosis. In this review, we discuss the evolution and adoption of endoscopic-assisted strip craniectomy procedures. In addition to reviewing the studies describing various nuances and modifications to minimally invasive strip craniectomy, attention to comparisons in outcomes between traditional or open cranial vault reconstructions and endoscopic-assisted techniques is highlighted for different craniosynostosis diagnoses.

Pediatric dog bite outcomes: infections and scars.

BACKGROUND: There is little consensus on the management of dog bite victims. Few studies have examined long-term patient outcomes. This study was designed to evaluate two outcomes: infection and unfavorable scar formation. METHODS: A retrospective study of dog bite cases from January 2013 to May 2016 was conducted at our level I pediatric trauma center. Forty-five patients were identified who received definitive repair and had long-term follow-up for reasons other than rabies vaccination. Variables recorded were wound characteristics including presence of tissue loss, location in the hospital of the wound repair procedure, personnel performing the repair, postrepair infection, and a binary assessment of unfavorable scar formation. RESULTS: Unfavorable scarring was not significantly related to either repair location or personnel. Rate of infection was not significantly related to repair location. However, infection rate was significantly related to personnel performing the repair (p=0.002), with 8 of 11 (73%) infections after repair by emergency physicians compared with surgeons. DISCUSSION: The presence of infection was significantly related to bedside repair by emergency physicians. The data are suggestive of differences in wound preparation and repair technique between emergency department and surgical personnel. Standardizing technique could reduce infectious complications and long-term morbidity associated with repairing dog bites and other contaminated wounds. A robust and practical classification system for dog bite wounds would be helpful in stratifying these wounds for research comparison and healthcare triage. LEVEL OF EVIDENCE: The level of evidence for this retrospective study is level III.

Axillary osmidrosis in Apert syndrome: management with an arthroscopic shaver technique.

Apert syndrome is a congenital condition characterized by craniosynostosis, syndactyly, and maxillary hypoplasia. Previous authors have outlined the management of craniofacial and extremity anomalies associated with this syndrome; however, there is a paucity of literature regarding the treatment of the cutaneous manifestations of Apert syndrome. Axillary osmidrosis, a chronic skin condition characterized by an excessive, axillary malodor resulting from apocrine gland dysfunction, can be particularly severe in patients with Apert syndrome. Herein, we describe a pediatric patient with Apert syndrome and severe axillary osmidrosis managed by an arthroscopic shaver technique of axillary glandular debridement and aspiration.

Congenital Granular Cell Tumor: Case Report and Review.

Congenital granular cell tumors are infrequently occurring masses occurring on a neonate's gingiva/alveolus. These lesions are benign with no noted malignant transformation, and treatment of excision is based on its effect on the neonate's respiratory ability and/or nutritional intake. The purpose of this review is to discuss a case of a congenital granular cell tumor and its treatment and review of the literature including demographics, histopathology, and operative treatment.

Newborn With Enlarged Erythematous Mass on Back: Case Report and Review of Medical Literature.

Newborn skin nodules are usually benign and self-resolving skin condition. Differential diagnosis of such lesions include cysts, hemangioma, abscess, cellulitis, sclerema neonatorum, subcutaneous fat necrosis, neurofibromatosis, benign tumors, or malignant tumors such as rhabdomyosarcoma, infantile fibrosarcoma, or neuroblastoma. We report a case of congenital subcutaneous fat necrosis in a 7-day-old baby presenting with multiple erythematous mass on back.

Effect of molding helmets on intracranial pressure and head shape in nonsurgically treated sagittal craniosynostosis patients.

OBJECTIVE Cranial vault expansion is performed in pediatric patients with craniosynostosis to improve head shape. Another argument for performing total cranial vault reconstruction is the potential reduction in the harmful effects of elevated intracranial pressure (ICP) that are associated with craniosynostosis. Alternatively, molding helmets have been shown to improve the cranial index (CI) in patients with sagittal synostosis without surgery. However, it is unknown if the use of molding helmets without surgery contributes to adverse changes in ICP. The effect of molding helmets on ICP and CI in patients with sagittal synostosis was investigated. METHODS A prospective cohort study of 24 pediatric patients with sagittal synostosis who planned to undergo total cranial reconstruction was performed from 2011 to 2014 at the Children's Hospital of Michigan. A preoperative molding helmet was used in 13 patients, and no molding helmet was used in 11 patients. End-tidal carbon dioxide, patient positioning, level of sedation, type of anesthetic, and the monitoring site at the time of intraoperative recording were regulated and standardized to establish the accuracy of the ICP readings. CI and head circumference were monitored for each patient. RESULTS The mean duration of the preoperative use of the molding helmet was 17 weeks (range 7-37 weeks). Under controlled settings, the average intraoperative ICP was 7.2 mm Hg (range 2-18 mm Hg) for patients treated with a preoperative molding helmet and 9.5 mm Hg (range 2-22 mm Hg) for patients with no preoperative molding helmet. ICP was not significantly different between the 2 groups, suggesting that the use of a molding helmet in this population is safe. The average CI at the time of helmet placement was 0.70 (range 0.67-0.73), and this improved to an average of 0.74 (range 0.69-0.79) after using the molding helmet for a mean of 17 weeks. CONCLUSIONS ICPs were not significantly different with the use of a preoperative molding helmet, refuting the prevailing thought that molding helmets would be detrimental in children who have craniosynostosis. The use of molding helmet in this population of patients improves head shape and does not adversely affect ICP.

Resorbable PLLA-PGA plate and screw fixation in pediatric craniofacial surgery: clinical experience in 1883 patients.

The need to provide rigid bony fixation in the surgical treatment of craniofacial deformities has inspired an on-going evolution of surgical innovations and implants. Because of the young age of many treated craniosynostosis patients and the unique pattern of cranial vault growth, the extensive implantation of metal devices is potentially problematic. The use of resorbable plate and screw devices offers all of the benefits of rigid fixation without many of their potential risks. Since the introduction of resorbable plate and screw devices in 1996, tens of thousands of craniofacial patients have received implants, but long-term results from a large series have yet to be reported. A combined prospective and retrospective analysis was done on 1883 craniosynostosis patients under 2 years of age treated by 12 surgeons from seven different geographic locations over a 5-year period who used the same type of resorbable bone fixation devices (poly-L-lacticpolyglycolic copolymer). Specifically, the incidence of postoperative infection, fixation device failure, occurrence of delayed foreign-body reactions, and the need for reoperation resulting from device-related problems were determined. Technical difficulties and trends in device use were also noted. From this series, significant infectious complications occurred in 0.2 percent, device instability primarily resulting from postoperative trauma occurred in 0.3 percent, and self-limiting local foreign-body reactions occurred in 0.7 percent of the treated patients. The overall reoperation rate attributable to identifiable device-related problems was 0.3 percent. Improved bony stability was gained by using the longest plate geometries/configurations possible and bone grafting any significant gaps across plated areas that were structurally important. The specific types of plates and screws used evolved over the study period from simple plates, meshes, and threaded screws to application-specific plates and threadless push screws whose use varied among the involved surgeons. This report documents the safety and long-term value of the use of resorbable (LactoSorb) plate and screw fixation in pediatric craniofacial surgery in the infant and young child. Device-related complications requiring reoperation occurred in less than 0.5 percent of the implanted patients, which is less frequent than is reported for metallic bone fixation. Resorbable bone fixation for the rapidly growing cranial vault has fewer potential complications than the traditional use of metal plates, screws, and wires.

Submucosal resection of a microcystic oropharyngeal lymphatic malformation using radiofrequency ablation.

Lymphatic malformations (LMs) are uncommon congenital anomalies noted to have a prevalence of 1 per 5000 births and comprise roughly 6% of all pediatric soft tissue lesions. Recently radiofrequency ablation has been described as a surgical option for the treatment microcystic LMs in the oral cavity, more specifically the tongue. The following case describes the use of radiofrequency ablation for the submucosal removal of a large obstructing pharyngeal LM in a 4-year-old female. The mucosal sparing approach and surgical method of extirpation are discussed in detail. To the authors' knowledge this is the first description of a submucosal coblation technique being used as treatment for pharyngeal LMs.

Effect of molding helmet on head shape in nonsurgically treated sagittal craniosynostosis.

OBJECT: Sagittal craniosynostosis is traditionally considered to be a surgical condition. Poor results of simple suturectomy follow from early reclosure of the suture. A wider craniectomy or use of interposing materials has not improved the outcome. However, endoscopic suturectomy supplemented with postoperative use of a molding helmet has shown good results. Because suturectomy reunites within 8-12 weeks of surgery, the authors questioned if the improved outcome was primarily related to use of the helmet. METHODS: In 4 patients whose families opted for calvarial reconstruction when the infant was 4-6 months old, instead of endoscopic suturectomy, a molding helmet was used to minimize compensatory changes in the interim. Patients underwent 3D CT scanning to confirm craniosynostosis. Follow-up visits were made at intervals of 4 weeks for adjustment of the helmet, head circumference measurements, clinical photographs, and cranial index measurement. RESULTS: There was significant improvement in the head shape within 6 weeks of use of the molding helmet. The cranial index score improved from a mean (± SD) of 67% ± 3% to 75% ± 2%. CONCLUSIONS: These cases demonstrate that molding helmets improve head shape even without a suturectomy in patients with sagittal craniosynostosis, challenging the traditional view.

Auditory brainstem response abnormalities and hearing loss in children with craniosynostosis.

OBJECTIVES: Craniosynostosis is a devastating disorder characterized by premature closure of the cranial plates before or shortly after birth. This results in an abnormally shaped skull, face, and brain. Little is known about hearing disorders in such patients, and nothing has been published about their auditory brainstem responses. Our objective was to evaluate such patients for auditory brainstem response and hearing disorders with the long-term goal of improving patient evaluation and management. PATIENTS AND METHODS: We evaluated the auditory brainstem responses, hearing, and brain images of children with fibroblast growth factor receptor 2 craniosynostosis (n = 11). RESULTS: Prolongation of the auditory brainstem response I-to-III interpeak latency was a frequent characteristic of fibroblast growth factor receptor 2 craniosynostosis, occurring in 91% of our patients. Prolongation of the III-to-V interpeak latency was an occasional characteristic, occurring in 27% of our patients. Whenever the I-to-III interpeak latency was prolonged, wave II was always abnormal. Associated morbidities included sensorineural hearing loss (27%), recurrent otitis media (100%), and Arnold-Chiari malformation (27%). Cranial decompression improved the interpeak latencies of 2 children. CONCLUSIONS: These previously undocumented auditory brainstem response abnormalities reflect abnormal neural transmission, which could cause peripheral and central auditory processing disorders. We speculate that the major pathogenic basis of the I-to-III interpeak latency and wave II abnormalities is compression of the auditory nerve as it passes through the internal auditory meatus and posterior fossa, which would explain the auditory nerve hearing loss, tinnitus, and vertigo that affect these children. Awareness of these abnormalities could lead to important advancements in the auditory and neurosurgical assessment and management of this overlooked patient group. We provide recommendations for the improved assessment and management of these patients. In particular, we recommend that auditory brainstem response diagnostics become standard clinical care for this patient group as the best way to detect auditory nerve compression.