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Craniofacial Surgery
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
Benjamin Robertson, Sujata De, Astrid Webber, Ajay Sinha
Described in 1906 by Apert, this syndrome has an incidence of 1: 60 000 live births. Apert syndrome is caused by mutations in FGFR2; two-thirds of cases have the mutation Ser252Trp while the remaining one-third have the Pro253Arg mutation.36 There is an autosomal dominant pattern of inheritance although most cases of Apert syndrome occur without a family history as a result of new mutations and there is a link with advanced paternal age.37 The main features of this condition include craniosynostosis (usually bicoronal), abnormal midfacial development and fusion of the digits of the hands and feet (syndactyly) (Figure 19.6). Abnormalities of the midface and cranium are evident at birth, with brachycephaly and midface retrusion causing an anterior open bite (malocclusion). Cleft soft palate or bifid uvula is present in 30% of cases. Fixation of the stapes footplate may cause a conductive hearing loss. There may be other associated malformations and intellectual ability may vary from normal to significantly impaired.38
Large pregnancy-associated pyogenic granuloma: a case report
Published in Journal of Obstetrics and Gynaecology, 2019
Jiarui Bi, Yi Sun, Liangjia Bi, Hannu S. Larjava
A 29-year-old pregnant woman with no systemic diseases or allergies visited the dental hospital complaining of a lump behind her upper front teeth. She had received 40 mg/day progesterone injections to support her pregnancy between the 5th and 14th gestational weeks, and then with an oral administration of 4 mg/day progesterone until the 16th week of her pregnancy. She first noticed the lump at 16th week of pregnancy. Intraoral examination showed the presence of a vascular lesion in size of about 6.0 × 4.0 cm, at the hard palate next to her incisors (Figure 1(A–C)). The tumour bled with touch and sometimes spontaneously. The patient had poor oral hygiene with a lot of dental calculus and soft deposits. The gingiva showed severe inflammation with a cyanotic colour and swollen papillae. A large-in-size soft tissue tumour covered with white pseudomembrane was found at the palatal aspect of her maxillary incisors. The lesion had introduced anterior open-bite malocclusion and disturbed her speaking and mastication. After having a normal childbirth, a complete excision of the lesion was carried out under a general anaesthesia (Figure 1(D)). A post-operative care visit one week after surgery revealed a good healing of the wound site with an improvement of the masticatory and speaking functions (Figure 1(E,F)).