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Case 2.12
Published in Monica Fawzy, Plastic Surgery Vivas for the FRCS(Plast), 2023
How would you choose where to place the ear in patients with anotia?Ear placement is based on:the distance from the root of the helix to the orbit,the distance from the lobule to the oral commissure, andthe angle determined by the axis of the ear to the nasal dorsum.These are measured on the normal side if there is one or that of a parent in patients with bilateral anotia.Alternatively, specific acetate templates (such as those described by Nagata and Magritz) can be used with various ear sizes. These are sized and aligned based on the positions of the eyebrow and alar of the nose on the unaffected side.
Use of Dermatologics during Pregnancy
Published in “Bert” Bertis Britt Little, Drugs and Pregnancy, 2022
In a study based on TIS information in Canada, Italy, and Israel, 53 infants exposed in early pregnancy to isotretinoin were identified (Garcia-Bournissen et al., 2008). Induced abortions were undertaken in 24 among 43 followed-up pregnancies (56 percent). Among 14 live-born infants, there were two with congenital malformations (anotia and heart defect). Bérard et al. (2007) in Canada reported 90 women pregnant during isotretinoin therapy. Among them 76 terminated the pregnancy (84 percent). There were three spontaneous abortions, one neonatal death due to obstetric trauma, and 10 live-born infants, among them one with congenital malformations of the face and neck. A study from the Berlin TIS (Schaefer et al., 2010) identified 91 isotretinoin-exposed pregnancies of which 69 (76 percent) were terminated. Five spontaneous abortions occurred and 18 live infants were born, one of which had a small ventricular septum defect.
Otorhinolaryngology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Chris Jephson, C. Martin Bailey
These range from minor abnormalities such as ‘bat ears’, as a result of abnormal folding of the cartilage, through varying degrees of microtia (Fig. 19.19), to complete absence of the pinna (anotia) with atresia of the external auditory meatus. Such anomalies can occur in isolation or in combination with other craniofacial or genetic syndromes, and there may be not only a cosmetic deformity but also a conductive hearing loss. The facial nerve may follow an abnormal course with these congenital ear anomalies.
Post-marketing surveillance for the safety of the 9-valent human papillomavirus vaccine: a retrospective real-world study in China
Published in Expert Review of Vaccines, 2023
Ruogu Meng, Rui Ma, Jianmei Wang, Peipei Liu, Zuoxiang Liu, Bingjie He, Zhike Liu, Yu Yang, Siyan Zhan
According to previous studies and local disease composition [14,16], the following autoimmune diseases were selected as outcomes for the study: Graves’ disease, Hashimoto’s thyroiditis, systemic lupus erythematosus (SLE), type 1 diabetes (T1DM), multiple sclerosis, optic neuritis, and uveitis. Adverse pregnancy outcomes included stillbirth and 23 major congenital anomalies that were selected according to the Chinese National Maternal and Child Health Surveillance Manual 2013 and included anencephaly, spina bifida, encephalocele, congenital hydrocephalus, cleft palate, cleft lip, cleft palate with cleft lip, microtia/anotia, other malformations of outer ear, esophageal atresia or stenosis, rectoanal atresia or stenosis, hypospadias, exstrophy of urinary bladder, talipes equinovarus, polydactyly, syndactyly, limb reductions, congenital diaphragmatic hernia, exomphalos, gastroschisis, conjoined twins, down syndrome, and congenital heart diseases [17].
Posterior semi-circular canal electrode misplacement in Goldenhar’s syndrome
Published in Cochlear Implants International, 2021
Ashish Castellino, Pabina Rayamajhi, Rahul Kurkure, Mohan Kameswaran
Goldenhar Syndrome may manifest in its mildest form as unilateral microtia, which has been suggested as a mandatory feature, to more severe forms involving facial, cardiac, renal and skeletal systems (Rollnick & Kaye, 1983). Its incidence ranges from 1/3500–1/5600 with a male female ratio of 3:2 (Gorlin et al., 2002). Features include facial asymmetry in 65% (Bartoshesky et al., 1986) with reduction in size and flattening of the temporal, maxillary and malar bones, epibulbar dermoids in 35% (Baum & Feingold, 1973) and cervical vertebral fusions in 60% (Avon & Shively, 1988). Pinna abnormalities range from anotia to mildly dysmorphic ears and are seen in over 65% (Coccaro et al., 1975). External auditory canals may range from narrow to atretic, with ear defects usually being asymmetrical (Bennun et al., 1985). Hypoplasia or agenesis of the ossicles, aberrant facial nerves, patulous or absent Eustachian tubes and skull base anomalies have been reported in greater than 50% with conductive hearing loss more common than sensorineural (Bassila & Goldberg, 1989; Sando & Ikeda, 1986).
Antenatal Diagnosis of Fetal Retinoid Syndrome at 20 Weeks of Gestation: A Case Report
Published in Fetal and Pediatric Pathology, 2018
Hakan Erenel, Aysegul Ozel, Funda Oztunc, Osman Kizilkilic, Nil Comunoglu, Seyfettin Uludag, Riza Madazli
Retinoids are a specific family of vitamin A derivatives including several subtypes such as first, second and third generation. Retinol, retinal, tretinoin (retinoic acid), isotretinoin, and alitretinoin are first generation retinoids. Etretinate and its metabolite acitretin are second-generation retinoids (4). Adapalene, bexarotene, and tazarotene are third generation retinoids (5). Retinoids are used in several conditions such as acne, psoriasis and cancer. One of these retinoids is isotretinoin (13-cis-retinoic acid), which was licensed in the United States in 1982 under the brand name Accutane (2). In 1985, Lammer et al. summarized outcomes of 154 pregnant patients with the exposure to isotretinoin (2). After excluding elective and spontaneous abortions, analysis of 21 malformed infants showed that most common craniofacial malformation was microtia or anotia. Maldevelopment of facial bones and calvarium, micrognathia, cleft palate, flat and depressed nasal bridge, hypertelorism, cerebellar hypoplasia, agenesis of the vermis were described cranial anomalies. They also reported several forms of conotruncal malformations such as transposition of great vessels, tetralogy of Fallot, double-outlet right ventricle, truncus arteriosus communis and supracristal ventricular septal defect in conjunction with abnormalities of thymic morphogenesis (ectopia, hypoplasia, aplasia) (2). In our case, fetal anomalies were observed in most of the above-mentioned regions.