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Neonatal Teeth

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161. Benign Lesions of the Ovaries (Follow-up)

from all 3 germ layers. Benign teratomas (also known as mature teratomas or dermoid cysts) are likely to contain more of the recognizable organic structures, such as thyroid, bronchial, and central nervous system tissue. [ ] In dermoid cysts, ectodermal structures such as hair, teeth, and skin predominate. Treatment In most instances, simple excision of the solid tumors is adequate therapy, particularly for women of reproductive age. Laparoscopic treatment of benign cystic teratomas of the ovaries (...) . The updated French guidelines indicate that transvaginal pelvic ultrasonography is the first-line modality for evaluating presumed benign ovarian tumors in adult women. [ ] Transvaginal ultrasonography is limited with regard to its role in assessing masses in neonates, children, and virginal adolescents. Color-coded Doppler ultrasonography improves the diagnostic accuracy of B-mode ultrasonography. Ultrasonography is easy, rapid, and able to provide critical information for the evaluation of an adnexal

2014 eMedicine.com

162. Tuberous Sclerosis (Diagnosis)

, isolated renal cyst(s), angiomyolipomas (AMLs), and renal cell carcinomas Dental findings: Pitting of the dental enamel is invariably present in the permanent teeth of patients with TSC [ ] ; gingival fibromas occur in 70% of adults with TSC, in 50% of children with mixed dentition (primary and permanent teeth), and in 3% of children with only primary teeth Gastrointestinal findings: Hamartomas and polyposis of the stomach, intestine, and colon may occur Hepatic findings: Hepatic cysts and hepatic AMLs (...) symptoms. Various organ systems are affected maximally at different points in life. Cardiac involvement occurs during the intrauterine or neonatal period. Rhabdomyomas tend to regress over time. Epilepsy, autism, and developmental delays manifest themselves from infancy to adolescence. Polycystic kidney disease usually is apparent in infancy or early childhood. AMLs may develop at any time from childhood into adult life. Lymphangiomyomatosis typically presents in the third or fourth decade of life

2014 eMedicine.com

163. Traumatic Ulcers (Diagnosis)

, malposed, or malformed teeth, as well as the premature eruption of teeth, can contribute to the formation of surface ulcerations. Poorly maintained and may also cause trauma. Next: Pathophysiology Nocturnal parafunctional habits, such as bruxism (ie, grinding of the teeth) and thumb sucking, may be associated with the development of traumatic ulcers of the buccal mucosa, the labial mucosa, the lateral borders of the tongue, and the palate. In addition, local irritants such as fractured or malposed (...) teeth and ill-fitting dentures may cause mucosal ulcers of the buccal mucosa, the lateral and ventral surfaces of the tongue, and the alveolar mucosa overlying the osseous structures. Healing of the ulcerated mucosa is usually delayed when the lesions overlie the maxillary or mandibular alveolar process. Ulcerations may be the result of voluntary, self-induced, and deliberate acts by patients with physical or psychological symptoms who are seeking medical attention. Butler et al report a patient

2014 eMedicine.com

164. Snoring and Obstructive Sleep Apnea, Physiologic Approach

for approximately 50% of total sleep in the neonatal period, 20-30% of total sleep in childhood, and 20% of total sleep throughout the rest of life. Delta sleep decreases steadily from childhood to old age, but REM sleep remains fairly constant. The continuity of sleep declines with age. The sleep of the elderly is often disrupted by many arousals (sometimes hundreds) and may lead to symptoms of poor sleep quality. The need for sleep does not decrease with age. Understanding the normal sleep cycle is helpful (...) advancing splints and found they improved apnea slightly. [ ] Subjective reports, such as reduction of daytime somnolence, were greater than objective measures. Coruzzi focused on the effectiveness of oral appliances and suggested that personalized oral jaw-positioning devices improve SDB. [ ] In turn, this may reduce cardiac variability, a marker for cardiovascular risk. Minimal risks to the teeth are present when making the impression at fitting. Occasionally, the jaw-thrust device and CPAP must

2014 eMedicine Surgery

165. Tuberous Sclerosis (Overview)

, isolated renal cyst(s), angiomyolipomas (AMLs), and renal cell carcinomas Dental findings: Pitting of the dental enamel is invariably present in the permanent teeth of patients with TSC [ ] ; gingival fibromas occur in 70% of adults with TSC, in 50% of children with mixed dentition (primary and permanent teeth), and in 3% of children with only primary teeth Gastrointestinal findings: Hamartomas and polyposis of the stomach, intestine, and colon may occur Hepatic findings: Hepatic cysts and hepatic AMLs (...) symptoms. Various organ systems are affected maximally at different points in life. Cardiac involvement occurs during the intrauterine or neonatal period. Rhabdomyomas tend to regress over time. Epilepsy, autism, and developmental delays manifest themselves from infancy to adolescence. Polycystic kidney disease usually is apparent in infancy or early childhood. AMLs may develop at any time from childhood into adult life. Lymphangiomyomatosis typically presents in the third or fourth decade of life

2014 eMedicine.com

166. Traumatic Ulcers (Overview)

, malposed, or malformed teeth, as well as the premature eruption of teeth, can contribute to the formation of surface ulcerations. Poorly maintained and may also cause trauma. Next: Pathophysiology Nocturnal parafunctional habits, such as bruxism (ie, grinding of the teeth) and thumb sucking, may be associated with the development of traumatic ulcers of the buccal mucosa, the labial mucosa, the lateral borders of the tongue, and the palate. In addition, local irritants such as fractured or malposed (...) teeth and ill-fitting dentures may cause mucosal ulcers of the buccal mucosa, the lateral and ventral surfaces of the tongue, and the alveolar mucosa overlying the osseous structures. Healing of the ulcerated mucosa is usually delayed when the lesions overlie the maxillary or mandibular alveolar process. Ulcerations may be the result of voluntary, self-induced, and deliberate acts by patients with physical or psychological symptoms who are seeking medical attention. Butler et al report a patient

2014 eMedicine.com

167. Toxicity, Salicylate (Overview)

of salicylate-containing teething gels in infants. [ ] On the basis of a literature review, Greene et al estimated the allowable daily intake of methyl salicylate to be 11 mg/kg/d. [ ] Ingestion of topical products containing salicylates, such as Ben-Gay, salicylic acid (keratolytic), and oil of wintergreen or methyl salicylate, can cause severe salicylate toxicity. One teaspoon of 98% methyl salicylate contains 7000 mg of salicylate, the equivalent of nearly 90 baby aspirins and more than 4 times (...) the potentially toxic dose for a child who weighs 10 kg. Salicylate toxicity has been reported with the topical use of salicylate-containing teething gels in infants. [ ] On the basis of a literature review, Greene et al estimated the allowable daily intake of methyl salicylate to be 11 mg/kg/d. [ ] A comprehensive review of the existing medical literature on methyl salicylate poisoning has determined that it is a relatively common source of pediatric exposures. [ ] In younger children, most

2014 eMedicine.com

168. Atrial Septal Defect (Overview)

mutations. Ellis van Creveld syndrome is an autosomal recessive disorder associated with skeletal dysplasia characterized by short limbs, short ribs, postaxial polydactyly, dysplastic nails and teeth, and a common atrium, occurring in 60% of affected individuals. [ ] Mutations in the cardiac transcription factor NKX2.5 have been attributed to the syndrome familial ASD associated with progressive atrioventricular block. [ , , ] This syndrome is an autosomal dominant trait with a high degree of penetrance (...) . . Thuny F, Di Salvo G, Belliard O, et al. Risk of embolism and death in infective endocarditis: prognostic value of echocardiography: a prospective multicenter study. Circulation . 2005 Jul 5. 112(1):69-75. . Tonni G, Ferrari B, Defelice C, Centini G. Neonatal porencephaly in very low birth weight infants: ultrasound timing of asphyxial injury and neurodevelopmental outcome at two years of age. J Matern Fetal Neonatal Med . 2005 Dec. 18(6):361-5. . Vick GW 3rd, Murphy DJ Jr, Ludomirsky A, et al

2014 eMedicine.com

169. Anetoderma (Overview)

, Bradley RR, Winkelmann RK. Familial anetoderma. J Am Acad Dermatol . 1987 Feb. 16(2 Pt 1):341-5. . Thomas JE, Mehregan DR, Holland J, Mehregan DA. Familial anetoderma. Int J Dermatol . 2003 Jan. 42(1):75-7. . Zellman GL, Levy ML. Congenital anetoderma in twins. J Am Acad Dermatol . 1997 Mar. 36(3 Pt 1):483-5. . Goujon E, Beer F, Gay S, Sandre D, Gouyon JB, Vabres P. Anetoderma of prematurity: an iatrogenic consequence of neonatal intensive care. Arch Dermatol . 2010 May. 146(5):565-7. . Karrer S (...) syndrome. J Am Acad Dermatol . 2007 May. 56(5):881-2. . Hodak E, David M. Primary anetoderma and antiphospholipid antibodies--review of the literature. Clin Rev Allergy Immunol . 2007 Apr. 32(2):162-6. . Goujon E, Beer F, Gay S, Sandre D, Gouyon JB, Vabres P. Anetoderma of prematurity: an iatrogenic consequence of neonatal intensive care. Arch Dermatol . 2010 May. 146(5):565-7. . Herrero-Gonzalez JE, Herrero-Mateu C. Primary anetoderma associated with primary Sjogren's syndrome. Lupus . 2002. 11(2):124

2014 eMedicine.com

170. Ellis-van Creveld Syndrome (Diagnosis)

, with progression from the proximal to distal parts of the limbs; shortening of the middle and distal phalanges; polydactyly affecting the hands (unilateral or bilateral) and, sometimes, feet; and hidrotic ectodermal dysplasia primarily affecting the nails, hair, and teeth. Dental problems are frequent, and natal teeth may be present. [ ] In the neonatal period, the leading causes of death are cardiac anomalies and thoracic dysplasia (with a narrow chest wall), with the latter causing severe respiratory (...) ; hence, the occurrence is independent of the affected patient's sex. Age Clinical findings, such as disproportionate extremities, narrow thorax, polydactyly, cardiac defects, and hidrotic ectodermal dysplasia affecting the nails, hair and teeth, are observed and diagnosable at birth. Previous Next: Prognosis A poor prognosis is declared in the neonatal period. Congenital cardiac anomalies and thoracic dysplasia (with a narrow chest wall) causing severe respiratory compromise are leading causes

2014 eMedicine Pediatrics

171. DiGeorge Syndrome (Diagnosis)

include the following (see the images below) [ ] : Retrognathia or micrognathia Long face High and broad nasal bridge Narrow palpebral fissures Small teeth Asymmetrical crying face Downturned mouth Short philtrum Low-set, malformed ears Hypertelorism Dimple on the tip of the nose Congenital heart defects, a cleft palate or incompetence of the soft palate, and immune deficiencies are common. Patients may have short stature and occasional instances of growth hormone deficiency. Renal, pulmonary (...) and cardiovascular abnormalities in 22q11.2DS include the following: Radiography Magnetic resonance imaging (MRI) Computed tomography (CT) scanning Echocardiography Angiography and magnetic resonance angiography (MRA) See for more detail. Management Congenital heart defect If a heart murmur and or other signs of a heart defect are present, consult a cardiologist right away, especially in the neonatal period. Hypocalcemia Begin calcium supplementation after proper tests (simultaneous serum calcium and serum PTH

2014 eMedicine Pediatrics

172. Disorders of Bone Mineralization (Diagnosis)

of vitamin D in breast milk (4-100 IU/L). In premature infants, insufficient amounts of calcium and phosphorus may cause nutritional rickets. Furthermore, reserves of vitamin D in the neonate highly depend on the mother's vitamin D status. Infants with low or no sun exposure may develop rickets, particularly if they have dark skin, because of decreased vitamin D production by the skin after exposure to UV light. Maternal hypovitaminosis D may cause congenital rickets in infants. In infants, clinical (...) features of and hyperphosphatemia include seizures, apnea, and tetany. In children, clinical features of rickets include the following (see the images below): Delayed motor milestones Hypotonia Enlargement of wrists Progressive bowing of long bones Rachitic rosary Harrison sulcus Violin case deformity of the chest Late closure of anterior fontanelle Parietal and frontal bossing Craniotabes Craniosynostosis Delay in teeth eruption Enamel hypoplasia Decreased bone mineral density Myopathy with normal

2014 eMedicine Pediatrics

173. Evaluation of the Pediatric Surgical Patient (Diagnosis)

process. An icteric sclera suggests hepatic or biliary dysfunction. Otitis media can be excluded if tympanic membranes that are clear and if visible landmarks are found. Finding an erythematous oropharynx or inflamed nasal turbinates with associated rhinorrhea is common in upper respiratory tract infections. A quick dental examination to identify loose teeth is important in children scheduled to undergo surgery. Chest wall Breast tissue is commonly observed in infant boys and girls. This is normal (...) of the abdomen may also be a clue to guide diagnosis. A scaphoid abdomen in a neonate or infant may suggest a diaphragmatic hernia but may be normal in a thin child. Intestinal obstruction, an abdominal mass, or ascitic fluid may cause abdominal distention. Second, listen for bowel sounds. Be patient because up to 2 minutes may pass before bowel sounds are heard. The absence of bowel sounds may suggest peritonitis. The character of the bowel sounds is also important; high-pitched sounds are consistent

2014 eMedicine Pediatrics

174. Seizure Disorders in Pregnancy (Follow-up)

acid. [ ] In addition, carbamazepine is also associated with a risk of cardiac anomalies; thus, for patients taking this drug, a fetal echocardiogram is recommended at 20-22 weeks' gestation. Trimethadione In 1975, a cluster of findings, including epicanthal folds, low-set ears, microcephaly, short stature, and irregular teeth, was ascribed to trimethadione. [ ] A review of 57 pregnancies published in 1977 revealed either malformations or fetal loss 87% of the time. [ ] Currently, trimethadione (...) in newborns suggest that the inhibition of vitamin K–dependent clotting factors (ie, II, VII, IX, X) secondary to increased vitamin K metabolism and the inhibition of placental transport of vitamin K results from AED use. Historically, most patients on AEDs received oral vitamin K supplementation at the end of pregnancy. However, a study of 204 neonates born to mothers taking AEDs who did not received vitamin K supplementation showed no evidence of coagulopathy. [ ] Upon delivery, clotting studies can

2014 eMedicine.com

175. Sleep Stage Scoring (Follow-up)

silence during REM sleep may not be of help in distinguishing it from the preceding or subsequent sleep stages. Compounding the problem of interpreting EMG channels is intrusion of artifact into the signal. Some examples include cyclic jaw movements, teeth grinding (bruxism), or steady high-amplitude noise generated by increased pressure on an electrode (eg, as caused by lying on the chin). Additionally, muscle artifact spilling over into cortical leads is not an unusual finding. ECG signal (...) are useful in the diagnosis and management of sleep disorders. For example, increased sleep latency may be increased in primary insomnia and decreased in obstructive sleep apnea, whereas Stage R latency may be decreased in narcolepsy. Sleep architecture changes with advancing age. The percentage of stage R is greatest in neonates, where it may encompass 50% of total sleep time. This proportion decreases gradually to 20-25% at adolescence, with further slight decrease after age 65. Greater sleep

2014 eMedicine.com

176. Mucopolysaccharidoses Types I-VII (Diagnosis)

syndrome type A or type B leads to the accumulation of keratan sulfate and chondroitin-6-sulfate in the connective tissue, the skeletal system, and the teeth. 5q11-q13 (Maroteaux-Lamy syndrome) Xq27.3-q28 (Hunter syndrome) A new mutation has been reported, making a total of 15 different mutations that can cause premature stop codons in the alpha-L-iduronidase gene ( IDUA ), and the biochemistry of these mutations has been investigated. Natural stop codon read-through is dependent on the fidelity (...) Med Genet A . 2011 Jun. 155A(6):1329-35. . Dodsworth Ch, Burton B K,. Increased incidence of neonatal respiratory distress in infants withmucopolysaccharidosis type II (MPS II, Hunter syndrome). Molecular Genetics and Metabolism . 2013. . Wijburg FA, Wegrzyn G, Burton BK, Tylki-Szymanska A. Mucopolysaccharidosis type III (Sanfilippo syndrome) and misdiagnosis of idiopathic developmental delay, attention deficit/hyperactivity disorder or autism spectrum disorder. Acta Paediatr . 2013 May. 102(5

2014 eMedicine.com

177. Mucocele and Ranula (Diagnosis)

caught between the maxillary anterior teeth and the mandibular anterior teeth during mastication or with the habit of biting one's lip. This trauma results in a crush-type injury and severance of the excretory duct of the minor salivary gland. In the palate, low-grade chronic irritation (eg, from heat and noxious tobacco products) may cause these lesions to develop. Mucoceles occur when injury to the minor salivary glands occurs usually secondary to trauma; biting one's lip; chronic inflammation (...) . . Macdonald AJ, Salzman KL, Harnsberger HR. Giant ranula of the neck: differentiation from cystic hygroma. AJNR Am J Neuroradiol . 2003 Apr. 24(4):757-61. . Osborne TE, Haller JA, Levin LS, Little BJ, King KE. Submandibular cystic hygroma resembling a plunging ranula in a neonate. Review and report of a case. Oral Surg Oral Med Oral Pathol . 1991 Jan. 71(1):16-20. . Ali MK, Chiancone G, Knox GW. Squamous cell carcinoma arising in a plunging ranula. J Oral Maxillofac Surg . 1990 Mar. 48(3):305-8

2014 eMedicine.com

178. Ectodermal Dysplasia (Diagnosis)

11, 2019 Author: Kara N Shah, MD, PhD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Ectodermal Dysplasia Overview Background The ectodermal dysplasias (EDs) comprise a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of 2 or more tissues derived from embryonic ectoderm. The tissues primarily involved are the skin and its appendages (hair follicles, eccrine glands, sebaceous glands, and, nails) and teeth (...) of cutaneous and/or oral embryonal ectoderm (ie, hair, nails, teeth, eccrine glands). In some forms, mesodermal abnormalities are also present. Characteristic features include the following: Hair defects: A reduction in the number of hair follicles in conjunction with structural hair shaft abnormalities may be seen. Structural hair shaft abnormalities may result from aberrations in hair bulb formation and include longitudinal grooving, hair shaft torsion, and cuticle ruffling. Hair bulbs may be distorted

2014 eMedicine.com

179. de Lange Syndrome (Diagnosis)

appearance by adulthood, with normalization of the dimensions. The faster changes in facial features than expected for age are typical in nearly all adolescents and adults. [ , ] During the neonatal period, respiratory and feeding difficulties ( ) predominate. The low-pitched cry frequently noted in the newborn period or in early infancy may disappear in late infancy. [ ] Self-injury is common in patients older than 12 years. Pubertal development and fertility are normal in BDLS individuals. Control (...) are neat, arched, and well defined. The nasal bridge is depressed, and the nares are upturned. Case study 1. The patient, aged 5 years, is shown; note microbrachycephaly, well-defined eyebrows, anteverted nares, long and thin upper lip, down-turned angles of mouth, and widely spaced teeth. Case study 1. Upper limb reduction anomalies are pictured with only 2 fingers present. Case study 1. Note short hypoplastic fifth finger. Case study 1. Small feet with short hypoplastic toes and syndactyly

2014 eMedicine.com

180. DiGeorge Syndrome (Diagnosis)

include the following (see the images below) [ ] : Retrognathia or micrognathia Long face High and broad nasal bridge Narrow palpebral fissures Small teeth Asymmetrical crying face Downturned mouth Short philtrum Low-set, malformed ears Hypertelorism Dimple on the tip of the nose Congenital heart defects, a cleft palate or incompetence of the soft palate, and immune deficiencies are common. Patients may have short stature and occasional instances of growth hormone deficiency. Renal, pulmonary (...) and cardiovascular abnormalities in 22q11.2DS include the following: Radiography Magnetic resonance imaging (MRI) Computed tomography (CT) scanning Echocardiography Angiography and magnetic resonance angiography (MRA) See for more detail. Management Congenital heart defect If a heart murmur and or other signs of a heart defect are present, consult a cardiologist right away, especially in the neonatal period. Hypocalcemia Begin calcium supplementation after proper tests (simultaneous serum calcium and serum PTH

2014 eMedicine.com

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