Early Familial Experiences With Microtia: Psychosocial Implications for Pediatric Providers.

This study focuses on early experiences of families with a child with microtia to better inform their ongoing care by pediatric providers. Parents and children (n = 62; mean age of 6.9 ± 3.9 years) with isolated microtia participated in semistructured interviews in Spanish (66.1%) or English (33.9%). Qualitative analysis of responses used open coding to identify themes. Parents reported stressful informing experiences of the diagnosis with multiple negative emotions. Parents and children generally reported not understanding microtia etiology, while some families identified medical, religious, and folk explanations. Parental coping included learning about surgeries, normalization, perspective taking, and support from family, providers, religion, and others with microtia. Family communication centered on surgery and reassurance. Pediatricians of children with microtia need to understand families' formative psychosocial experiences to better promote positive family adjustment through clarifying misinformation, educating families about available treatment options, modeling acceptance, psychosocial screening, and providing resources.

Teasing in younger and older children with microtia before and after ear reconstruction.

This study prospectively measured teasing and emotional adjustment before and after ear reconstruction in younger and older children with microtia. Participants with isolated microtia (n = 28) were divided into two groups by age at surgery, with a younger group aged 3-5 years (n = 13) with a mean age of 4.0 (0.71) years at the time of surgery and an older group aged 6-10 years old (n = 15) with a mean age of 7.87 (1.30) years. Children and their parents were interviewed preoperatively and a year after surgery about teasing and emotions about their ear(s). Teasing began between the ages of 2.4-4.8 years. A third of the younger group and all of the older group reported preoperative teasing. Before surgery, the older group reported higher rates of negative emotions about their ear(s) and teasing was correlated for all ages with feeling sad, worried, and mad about their ear(s). After surgery, teasing and negative emotions significantly decreased with increased happiness about their ear(s). Postoperative teasing was correlated with trying to hide their ear(s). There were significant interactions from before to after surgery based on surgery age for frequency of teasing, sadness, and feeling mad, with the older group showing relatively greater change postoperatively. Teasing and negative emotions about their ear(s) decreased for all ages after surgery, with a potential protective factor seen in younger surgery age.

Complications after Total Porous Implant Ear Reconstruction and Their Management.

Microtia reconstruction using porous polyethylene implants has become an established alternative to autologous costal cartilage techniques. Few surgeons are trained in porous implant ear reconstruction (PIER), leading to a relative lack of understanding of the nuances of this type of surgery. The risks of exposure, infection, and fracture of the implant have further discouraged surgeons from performing PIERs. Meticulous technique and proper management of complications are critical to the success of surgeries involving porous implants (Medpor, Su-Por). There are a limited number of articles in the literature that report the management of complications of porous implant auricular reconstruction. The purpose of this work is to present a comprehensive review of the management of complications with PIER based on over 10 years of experience with this surgical technique.

Pre and post-operative psychological functioning in younger and older children with microtia.

OBJECTIVES: Microtia ranges from a smaller ear to the absence of the external ear and has been associated with psychosocial distress. Traditional ear reconstruction takes place beginning at age six. Use of an alloplastic implant allows for earlier surgery starting at age three, which may reduce potential negative psychological effects. However, few studies have examined psychosocial outcomes of ear reconstruction with groups that include young children. METHODS: Children (N = 23) with microtia and their parents completed two microtia-related scales, negative emotions and microtia social awareness, and the Behavioral Assessment System for Children - Second Edition (BASC-2) subscales of anxiety, depression, and social skills before surgery and one year after surgery. Participants (74% male) were three to nine years old with a mean age of 6.13 ± 2.10 years and were grouped by age at surgery, three to six years (n = 11) or seven to ten years (n = 12). The sample identified as Latino (96%) or "other" (4%). RESULTS: Pre and postoperative scores by age group were compared using two-way repeated measures analyses of variance. Children and parents reported significantly less negative emotion and microtia social awareness following surgery, with an interaction for parental report of older children showing higher negative emotion preoperatively. Older children also had higher scores of depression and anxiety before surgery and both groups reported significant decreases following surgery, along with improved social skills. Older children showed significantly greater gains in social skills. CONCLUSION: All participants and their parents reported improved psychological functioning postoperatively. However, older children may be at greater risk of psychological concerns given the longer time they have to cope with the impact of microtia on self-image and exposure to social stressors. Undergoing reconstructive surgery earlier may be a protective factor for children with microtia.

Congenital malignant melanoma: a case report with cytogenetic studies.

Although rare, congenital malignant melanoma (CMM) should be considered in the differential diagnosis of congenital skin lesions. We report a case of CMM in a 4-month-old infant presenting with an enlarging scalp mass, initially thought to be a hemangioma. Incisional biopsy of the lesion showed a compound congenital nevus with atypical cells suggestive of a proliferative nodule versus malignancy on histopathology. Subsequent excisional biopsy revealed malignant melanoma, and further workup confirmed extensive disease with distant metastases. Cytogenetic analysis of both the tumor sites showed highly abnormal karyotypes including pseudotetraploidy, telomere associations, and evidence of gene amplification, all consistent with malignancy. Fluorescence in situ hybridization demonstrated amplification of the MYC gene, with no copy number changes in CDKN2A (INK4/ARF), PTEN, or Cyclin D1. Our report details the cytogenetic and molecular studies of CMM, which provide insight into the biologic behavior of the lesions and may confirm diagnosis when histopathology is not determinant.

Ear reconstruction using a porous polyethylene framework and temporoparietal fascia flap.

Ear reconstruction is a difficult procedure requiring a framework and soft tissue covering. The traditional method uses a rib cartilage framework placed beneath scalp skin. This method has been used for 50 years despite inherent problems with both harvesting rib cartilage and using scalp for coverage. The authors describe a method using a porous polyethylene (PPE) framework covered by a large temporoparietal fascia (TFP) flap raised with the underlying subgaleal fascia (SGF). The entire implant is covered by the two-layered flap, which can be raised without any scalp incision. The skin grafts applied to the covered implant lie on the SGF. The trilaminar structure of the SGF allows the skin to move independently over the implant, resisting shear forces and reducing the probability of implant exposure. Ear reconstruction using the PPE framework was performed on 786 ears over an 18-year period. Initial complications were common. With improved implant design and complete coverage of the implant with both the TPF and SGF, exposure rate dropped to 7% with a 12-year follow-up. Implant fractures decreased to less than 3%. The PPE/TPF method allows earlier ear reconstruction in children with minimal scarring and discomfort. The reconstructed ear can closely mimic the shape and projection of the natural contralateral ear in fewer stages and with a shorter learning curve.

Atresia repair before microtia reconstruction: comparison of early with standard surgical timing.

OBJECTIVE: To compare short-term results of atresia repair when performed before versus after microtia reconstruction. STUDY DESIGN: Retrospective case review. SETTING: Tertiary otologic referral center. PATIENTS: Congenital aural atresia with or without microtia: 70 cases over 24 months. INTERVENTION: Atresia repair before Medpor microtia reconstruction (ARM) versus atresia repair after microtia reconstruction with autogenous rib (ARR) versus atresia reconstruction without microtia (AR). MAIN OUTCOME MEASURES: Surgical outcomes, short-term postoperative audiometric results (at least 4 months after surgery but within the first postoperative year), complications. RESULTS: Data from the 3 groups are as follows: ARM, 31 patients with median age 4.2 years (range, 2.5-9.3 yr); ARR, 28 patients with median age 12 years (range, 6.9-61); and AR, 11 patients with median age 5.9 years (range, 5.5-59 yr). Preoperative computed tomographic grading using the Jahrsdoerfer scale demonstrated an average score of 7.4 (range, 6-9) for the ARM group, 7.7 (range, 6-9) for the ARR group, and 8.5 (range, 8-9) for the AR group. For patients scoring 8 to 10 on the Jahrsdoerfer scale, postoperative pure-tone average 2 for each group were as follows: ARM, 28 dB hearing loss (HL); ARR, 32 dB HL; and AR, 29 dB HL. For patients scoring 7 or less, postoperative pure-tone average 2 were as follows: ARM, 42 dB HL; and ARR, 41 dB HL (AR, no patients). Surgical complications of infection and facial nerve injury were not seen in any group. Meatal stenosis was higher in the ARR group. One patient in the ARM group suffered a high-frequency sensorineural HL. No patient receiving Medpor microtia reconstruction suffered a complication due to the presence of the ear canal before microtia reconstruction. CONCLUSION: Early results of ARM compare favorably with results achieved with atresia repair after microtia reconstruction with autogenous rib cartilage and with atresia repair without microtia repair. Hearing outcome and complications in this study are also comparable with previously reported expert results. Because restoration of binaural hearing has been shown to be advantageous for auditory development and function, timing of atresia repair can be considered before microtia reconstruction on an individual case basis, provided preoperative computed tomographic evaluation shows an adequate chance of surgical success.