Dens invaginatus is a rare developmental anomaly of the teeth that is caused by the infolding of enamel organs or the penetration of their proliferations into dental papillae before calcification has occurred. The presence of double dens invaginatus is extremely rare. This paper describes the use of cone beam computed tomography in the evaluation of a maxillary lateral incisor with double dens invaginatus and periapical periodontitis. The tooth was treated through microscopic root canal therapy. The tooth was free of clinical symptoms, and the periradicular lesion narrowed during the follow-up period of 1 year.
Humans ; Dental Pulp Cavity/abnormalities* ; Dens in Dente/pathology* ; Incisor/pathology* ; Root Canal Therapy ; Periapical Periodontitis/pathology*

A full-term female infant was admitted at 5 hours after birth due to heart malformations found during the fetal period and cyanosis once after birth. Mmultiple malformations of eyes, face, limbs, and heart were noted. The whole-exome sequencing revealed a pathogenic heterozygous mutation, c.2428C>T(p.Arg810^*), in the BCOR gene. The infant was then diagnosed with oculo-facio-cardio-dental syndrome. He received assisted ventilation to improve oxygenation and nutritional support during hospitalization. Right ventricular double outlet correction was performed 1 month after birth. Ocular lesions were followed up and scheduled for elective surgery. The possibility of oculo-facio-cardio-dental syndrome should be considered for neonates with multiple malformations of eyes, face, and heart, and genetic testing should be performed as early as possible to confirm the diagnosis; meanwhile, active ophthalmic and cardiovascular symptomatic treatment should be given to improve the prognosis.
Female ; Humans ; Infant ; Infant, Newborn ; Male ; Abnormalities, Multiple/therapy* ; Cataract/genetics* ; Cyanosis ; Proto-Oncogene Proteins ; Repressor Proteins/genetics* ; Heart Defects, Congenital/genetics*

Palatal radicular groove is a developmental malformation of maxillary incisors, lateral incisors in particular, which often causes periodontal destruction. This paper reports a case of combined periodontal-endodontic lesions induced by palatal radicular groove, which was initially misdiagnosed as a simple periapical cyst. After root canal therapy and periapical cyst curettage, the course of disease was prolonged, resulting in the absence of buccal and maxillary bone plates in the affected tooth area. After the etiology was determined, the affected tooth was extracted and guide bone tissue regeneration was performed at the same time, followed by implantation and restoration at the later stage, leading to clinical cure. The palatal radicular groove is highly occult, and the clinical symptoms are not typical. If the abscess of the maxillary lateral incisor occurs repeatedly, and the abscess of the maxillary lateral incisor has not been cured after periodontal and root canal treatment, cone-beam computed tomographic and periodontal flap surgery should be considered.
Humans ; Incisor ; Radicular Cyst ; Abscess ; Tooth Root/abnormalities* ; Root Canal Therapy ; Maxilla ; Cysts

Infraocclusion is a phenomenon that the relative occlusal growth of a tooth stops after the period of active eruption and then the tooth becomes depressed below the occlusal plane. Infraocclusion occurred more commonly in children and the mostly affected teeth were the primary mandibular second molars. The occlusal problem caused by infraocclusion may progressively worsen with age. This review summarizes the etiology, diagnosis and treatment of infraoccluded second primary molars, so as to provide reference for the dental clinicians.
Child ; Humans ; Malocclusion/therapy* ; Molar ; Tooth Abnormalities ; Tooth Eruption ; Tooth, Deciduous

OBJECTIVE: To investigate the morbidity of congenital heart defects(CHDs) in children with anorectal malformation, and to summarize appropriate treatment. METHODS: The clinical data and echocardiographic findings of 155 children with congenital anorectal malformations from the Third Affiliated Hospital of Zhengzhou University during January 2016 and October 2019 were reviewed. According to the surgical findings of anorectal malformations, the patients were categorized as the high/intermediate group and the low group; the CHDs were classified as minor CHDs and major CHDs. Multiple logistic regression was used to analyze the correlation of wingspread classification, and extracardiac malformations with the severity of CHDs. RESULTS: Out of 155 children with anorectal malformations, 47 (30.3%) had different types of cardiac structural malformations, including 18 cases of minor CHDs (11.6%) and 29 cases of major CHDs (18.7%). Sixty children (38.7%) had extracardiac malformations, of which 38 cases (24.5%) had a single extracardiac malformation, 15 cases (9.7%) had multiple extracardiac malformations, 6 had trisomy 21 syndrome, and 1 had VATER syndrome. Multivariate logistic regression analysis showed that wingspread classification of anorectal malformation and extracardiac disorders were independent risk factors for major CHDs. The probability of major CHDs in children with high/intermediate anorectal malformation was 4.709 times higher than that with low anorectal malformation ( CONCLUSIONS The morbidity of major CHDs is higher in severe cases with high/intermediate anorectal malformation and acute cases without fistula or with obstructed fistula and cases with multiple congenital disorders. Echocardiography can define the type and severity of CHDs, which are useful to develop the optimal diagnosis and treatment plan for children with anorectal malformation.
Abnormalities, Multiple ; Anorectal Malformations/therapy* ; Child ; Heart Defects, Congenital/mortality* ; Humans ; Retrospective Studies

Adult ; Amenorrhea ; Congenital Abnormalities ; Drug Therapy ; Dysgerminoma ; Female ; Humans ; Karyotype ; Uterus

OBJECTIVE: To describe the characteristics of the clinical presentation, diagnosis, surgical methods, and outcomes of patients with otogenic cerebrospinal fluid (CSF) leakage secondary to congenital inner ear dysplasia. METHODS: A retrospective review was performed of 18 patients with otogenic CSF leakage secondary to inner ear dysplasia who underwent surgery in our group from 2007 to 2017 and had a follow-up of at least 4 months. The average length of follow-up was three years. The characteristics of the clinical presentations of all patients, such as self-reported symptoms, radiographic findings, surgical approaches and methods of repair, position of the leakage during surgery, and postoperative course, including the success rate of surgery, are presented. RESULTS: The patients presented mostly with typical symptoms of meningitis, severe hearing impairment, and CSF otorrhea or rhinorrhea. All 18 patients had at least one previous episode of meningitis accompanied by a severe hearing impairment. The preoperative audiograms of 17 patients showed profound sensorineural hearing loss, and one patient had conductive hearing loss. Twelve patients presented with an initial onset of otorrhea, and two had accompanying rhinorrhea. Six patients complained of rhinorrhea, two of whom were misdiagnosed with CSF rhinorrhea and underwent transnasal endoscopy at another hospital. High-resolution computed tomography (HRCT) images can reveal developments in the inner ear, such as expansion of a vestibular cyst, unclear structure of the semicircular canal or cochlea, or signs of effusion in the middle ear or mastoid, which strongly suggest the possibility of CSF otorrhea. The children in the study suffered more severe dysplasia than adults. All 18 patients had CSF leakage identified during surgery. The most common defect sites were in the stapes footplates (55.6%), and 38.9% of patients had a leak around the oval window. One patient had a return of CSF otorrhea during the postoperative period, which did not re-occur following a second repair. CONCLUSIONS CSF otorrhea due to congenital inner ear dysplasia is more severe in children than in adults. The most common symptoms were meningitis, hearing impairment, and CSF otorrhea or rhinorrhea. HRCT has high diagnostic accuracy for this disease. The most common fistula site was around the oval window, including the stapes footplates and the annular ligament.
Adolescent ; Adult ; Cerebrospinal Fluid Otorrhea/therapy* ; Child ; Child, Preschool ; Ear, Inner/abnormalities* ; Humans ; Infant ; Middle Aged ; Retrospective Studies ; Young Adult

The purpose of this study was to examine dental complications and to evaluate the effects of initial treatment age, treatment modalities, and treatment duration on the disorder after radiation and chemotherapy in pediatric cancer patients.For 93 children with clinical and radiographic data, the number of teeth, the morphology of teeth, the shape of the roots, and development status of the dentition were evaluated.Dental development disorders were found in 61.3% of the children. The mostly found abnormality was root deformity with the prevalence of 31.2%. In children submitted to the therapy before the age of 6, the number of missing teeth (p = 0.029) and microdontia (p = 0.003) were greater compared to the children who started to receive the treatment after the age of 6. The combination of radiation and chemotherapy showed significantly greater incidences of missing teeth (p = 0.030), microdontia (p = 0.046), and root deformity (p = 0.009) when compared with the sole application of chemotherapy. When the children were submitted to anticancer therapy for 18 months or longer duration, greater number of missing teeth (p = 0.032), microdontia (p = 0.011), root deformity (p = 0.025), and total number of teeth affected (p = 0.036) were observed compared with duration less than 18 months.The number of dental abnormalities increased when the children were treated at earlier ages, with combination of radiation and chemotherapy, and for longer period of time.
Child ; Congenital Abnormalities ; Dentition ; Drug Therapy ; Humans ; Incidence ; Prevalence ; Tooth

Congenital absent sternum is a rare birth defect that requires early intervention for optimal long-term outcomes. Descriptions of the repair of absent sternum are limited to case reports, and no preferred method for management has been described. Herein, we describe the use of porcine acellular dermal matrix to reconstruct the sternum of an infant with sternal infection following attempted repair using synthetic mesh. The patient was a full-term male with trisomy 21, agenesis of corpus callosum, ventricular septal defect, patent ductus arteriosus, right-sided aortic arch, and congenital absence of sternum with no sternal bars. Following removal of the infected synthetic mesh, negative pressure wound therapy with instillation was used to manage the open wound and provide direct antibiotic therapy. When blood C-reactive protein levels declined to ≤2 mg/L, the sternum was reconstructed using porcine acellular dermal matrix. At 21 months postoperative, the patient demonstrated no respiratory issues. Physical examination and computed tomography imaging identified good approximation of the clavicular heads and sternal cleft and forward curvature of the ribs. This case illustrates the benefits of negative pressure wound therapy and acellular dermal matrix for the reconstruction of absent sternum in the context of infected sternal surgical site previously repaired with synthetic mesh.
Acellular Dermis ; Agenesis of Corpus Callosum ; Aorta, Thoracic ; C-Reactive Protein ; Congenital Abnormalities ; Down Syndrome ; Ductus Arteriosus, Patent ; Early Intervention (Education) ; Head ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Male ; Methods ; Negative-Pressure Wound Therapy ; Physical Examination ; Ribs ; Sternum ; Surgical Mesh ; Thoracic Surgery ; Wounds and Injuries

STUDY DESIGN: Randomized controlled single-blinded clinical trial. PURPOSE: To evaluate the efficacy of Schroth therapy on thoracic curve angle, pain, and self-perceived body image (SPBI) of the back in Scheuermann's patients in comparison with the efficacy of classic anti-gravitation exercises. OVERVIEW OF LITERATURE: Scheuermann disease is the most common cause of hyperkyphosis of the thoracic and thoracolumbar spine during adolescence. However, very few studies evaluated the effect of exercises on the progression of kyphosis in Scheuermann patients. Schroth three-dimensional exercise therapy was found in several studies to be effective in the treatment of adolescent scoliosis; however, we found no randomized controlled trials that evaluated the efficacy of this method in Scheuermann patients. METHODS: A total of 50 young adults (males and females) with Scheuermann's disease were randomly divided into either the experimental group (Schroth therapy treatment, n=25) or the control group (classic anti-gravitation exercises, n=25). Participants in both the groups were provided a course of individual treatment sessions during few weeks, with one appointment per week. They were required to perform the exercises daily throughout the study period (12 months) and fill their performance in a research log. We evaluated the thoracic Cobb angle (main outcome measure), pain, SPBI, flexion of the shoulder (supine), flexion of the shoulder (standing), kyphotic deformity measured using inclinometer, and L5 kyphosis apex line (L5-KAL) as well as administered the Scoliosis Research Society-22 Questionnaire for the participants before the treatment, after 6 months, and 1 year postoperatively. These results were then compared. RESULTS: In the mixed analysis of variance, the main effect of time was significant in the thoracic kyphosis (F [1]=5.72, p=0.02), and in the L5-KAL (F [1]=5.76, p=0.02). The main effect of time on the kyphotic deformity, measured using an inclinometer, did not reach the significance level; however, it showed the tendency (F [1]=2.80, p=0.07). In the group-by-time interaction, a significant difference was found in the thoracic kyphosis (F [1]=4.91, p=0.03) and in the kyphotic deformity, measured using an inclinometer (F [1]=4.05, p=0.02). Thus, the Schroth therapy group showed significantly greater improvement than the classic anti-gravitation exercises group. CONCLUSIONS: The present findings indicate that back exercises in general, and Schroth therapy in particular, is an effective treatment for preventing and significantly improving the thoracic Cobb angle and symptomatic representation in Scheuermann's patients.
Adolescent ; Body Image ; Congenital Abnormalities ; Exercise ; Exercise Therapy ; Humans ; Kyphosis ; Methods ; Physical Therapy Modalities ; Posture ; Quality of Life ; Randomized Controlled Trials as Topic ; Scheuermann Disease ; Scoliosis ; Shoulder ; Spine ; Young Adult