An atypical presentation of talon cusp: Double talon cusps

Talon cusp is an uncommon developmental anomaly resulting in an extra cusp or cuspal projection on an anterior tooth consisting of normal enamel, dentin, and varying degrees of pulp tissue. Talon cusp shows varied presentations of which the palatal talon cusp has been the most common of all. The management requires an extensive understanding of this clinical entity and the complications associated with its occurrence. In this case series, we are reporting double talon cusps which is a rarity itself, involving the palatal aspect of maxillary central incisors

A rare case of discontinuous type splenogonadal fusion in a 13 year old boy: a case report and review of literature

Splenogonadal fusion (SGF) is a rare developmental anomaly in which an abnormal connection between the splenic tissue and gonads or mesonephric derivatives is present. This entity often presents with scrotal mass, inguinal hernia, or cryptorchidism. Less than 200 cases have been reported since it was first described in 1883. It can be of continuous and discontinuous type based on the presence of a band of connecting splenic tissue. Report a rare case of discontinuous type of SGF in an adolescent male presenting as nonpalpable testis. On evaluation, ultrasonography (USG) and magnetic resonance imaging of abdomen and pelvis, his left scrotal testis was atrophied and right intra-abdominal undescended testis. This is the first reported case of SGF from Bosnia and Herzegovina. Laparoscopy was demonstrated to be the only accurate exploratory procedure for the diagnosis and surgical treatment of SGF with non‑palpable testis.

Pancake kidney with single draining ureter: a rare developmental anomaly

Pancake kidney is one of rare form of congenital developmental anomaly of kidney. Very rarely it is drained by a single ureter. We are reporting a case of pancake kidney with a single draining ureter. The diagnosis was made during the routine workup for abdominal pain and UTI in a 12 year old male child. Usually pancake kidney may be associated with other congenital malformation but in our case we did not find any. Pancake kidney is managed surgically as well as conservatively depending upon the presenting symptoms and problem. This case was managed conservatively for recurrent UTI.

Hemimelia tibial izquierda con pie equino varo aducto bilateral y ausencia del tercer rayo de la mano izquierda. Reporte de Caso / Left tibial hemimelia with bilateral clubfoot and absence of the third ray of the left hand. Case Report

La hemimelia tibial se puede presentar en una gran variedad de espectros, desde la hipoplasia tibial hasta la ausencia completa de la tibia con o sin compromiso adjunto cuadricipital, ligamentario, patelar, fibular y/o femoral; esto ha dado lugar a múltiples clasificaciones con implicaciones anatómicas y terapéuticas. Esta enfermedad se ha descrito desde 1841, sin embargo, es la deficiencia más rara en las extremidades inferiores, siendo la más común la deficiencia fibular. Presentamos un paciente con diagnóstico antenatal de pie equino varo bilateral, agenesia de la tibia izquierda y comunicación aurículo ventricular (CIA) con cariotipo normal. Al nacer presenta fascies normales. Se confirma con radiografías la ausencia del tercer rayo de la mano izquierda y la ausencia de la tibia izquierda con ensanchamiento del peroné, tipo 5C en la clasificación de Paley, y pie equino varo aducto bilateral. Nivel de Evidencia: IV

Tibial hemimelia is a broad spectrum disorder, from tibial hypoplasia to complete absence of the tibia with or without quadricipital, ligament, patellar, fibular and / or femoral attachment. This has led to multiple classifications, with their anatomical and therapeutic implications. Although this disorder has been described since 1841, it is the rarest deficiency in the lower extremities, with the most common being fibular deficiency. The case is presented on a patient with an antenatal diagnosis that included bilateral congenital talipes equinovarus, agenesis of the left tibia, and atrioventricular communication with normal karyotype. The facies were normal at birth. Radiographs confirmed the absence of the third ray of the left hand and the absence of the left tibia, with widening of the fibula, type 5C in Paley classification, and bilateral congenital talipes equinovarus. Evidence Level: IV

Prevalence of Developmental Anomalies of Teeth in a Group of Indian Children A Cross Sectional.

Introduction: Developmental anomalies of the tooth areroutinely encountered by paediatric dental professionalsand may present with esthetic and or functional problems.The reported literature has shown a wide variation in theprevalence of these conditions across the globe. Study aimedto assess the prevalence of various developmental anomaliesof tooth in a sample of Indian paediatric populationMaterial and Methods: A total of 3000 children in the agegroup of 6 to 13 years visiting the OPD of pedodontics andpreventive dentistry of BIDSH, Patna were examined clinicalyand radiologicaly when required and the data was tabulated.Descriptive statistics methods were used using SPSS.Results: Prevalence of dental anomalies was 4.86%.in thestudy population and Enamel hypoplasia, hypodontia andtalon's cusp were the most frequently encountered anomalies.The presence of developmental anomalies was a statisticalysignificant finding.Conclusion: Early detection of developmental anomaliesmay prevent future complications and eliminate the need forcomplex therapeutic intervention.

Hemimelia del peroné y la tibia, más que un hueso ausente: presentación de casos / Fibular and tibial hemimelia, more than just an absent bone: case presentations

El acortamiento congénito del miembro inferior hace parte de un grupo de trastornos poco frecuentes, entre los que se encuentran la deficiencia femoral focal proximal y la hemimelia de peroné o de la tibia. Pueden presentarse desde una forma leve de hipoplasia hasta ausencia completa del hueso con grados variables de acortamiento, siendo evidente el acortamiento de la extremidad desde el nacimiento. El artículo revisa este tema y presenta los casos de hemimelia de tibia y peroné del Hospital Universitario San Ignacio en los últimos años, se describen los principales hallazgos imaginológicos en radiografía simple y en resonancia magnética.

Congenital lower extremity shortening is part of a group of infrequent disorders, together with proximal femoral deficiency, tibial and fibular hemimelia. It can manifest as a mild form of hypoplasia to a complete lack of the bone, with variable degrees of shortening and with an evident start since birth. In this pictorial review, we will describe cases of fibular and tibial hemimelia seen at San Ignacio University Hospital in the last years. The main imaging findings are described in X ray and MRI.

A Case of Ectopic Liver Attached to the Gallbladder Wall.

The liver lies mainly in the right upper quadrant of the abdomen where it is protected by the thoracic cage and diaphragm. Externally, the liver is divided into two anatomical lobes and two accessory lobes.During autopsyof a 25- year oldmale, an ectopic liver tissue was found attached to the serosa of gallbladder. Morphological variations of liver are irregularities in form, occurrence of one or more accessory lobe. The exact reason for the origin of ectopic liver in man isstill unknown. It may also simulate tumor.

Developmental anomalies affecting the morphology of teeth - a review

Introduction:The development of tooth is a complex process wherein there is series of interactions between the ectoderm and ectomesenchyme. The role of genes in determining the shape and form of a specific tooth has already been defined, the alterations in which can lead to a variety of anomalies in regards to number, size, form, shape, structure, etc. Objective: To review the literature on the developmental anomalies of teeth. Literature review: The developmental anomalies affecting the morphology exists in both deciduous & permanent dentition and shows various forms such as gemination, fusion, concrescence, dilacerations, dens evaginatus, dens invaginatus, enamel pearls, taurodontism or peg laterals. These anomalies have clinical significance concerning esthetics, malocclusion and more importantly predisposing the development of dental caries and periodontal diseases. Conclusion: Knowledge of various diagnostic criteria for identification of these developmental anomalies is significant for early diagnosis and pertinent treatment.

Congenital Foramen In the Body of Sternum.

Background:The knowledge of sternal variations is important as this bone is usually chosen for bone marrow aspiration.One such developmental anomaly is the presence of foramina, of varying dimensions, in manubrium or body or xiphisternum.Usually, the defect occurs in the lower third of sternum,in the body, as a single midline foramen (oval or round) and is usually asymptomatic and can be demonstrated only by CT scanning. The observation and knowledge of these foramina are of utmost importance in clinical, forensic and acupuncture techniques.Hence, this observation could be a useful one. Materials and methods: During a routine osteology class in the Department of Anatomy,Kanyakumari Government Medical College,an adult sternum of unknown sex and age was found to have a foramen in the lower part of body at the level of articular facets for fifth costal cartilages. Results:The following observations were made: (1)The sternum presented an oval foramen in the lower part of the body. (2) It was located in midline at the junction between third and fourth sternebrae.(3) Its margins were smooth and measured 5mm×6mm. Conclusion:Embryologically,sternum is formed by the craniocaudal fusion of two sternal bars,which are condensations of mesenchymal tissue which get converted to pre-cartilage before fusion. Any defect in this fusion may cause cleft sternum or sternal foramina(single or multiple). The single sternal foramen presented here could have clinical implications. Its knowledge helps in preventing fatal cardiac tamponade following needle insertion during bone marrow biopsy or acupuncture performed in this region. Forensic misinterpretation of this defect is also possible.

Bilateral fusion in primary mandibular teeth.

Fusion has been described as a developmental anomaly characterized by the union of two adjacent teeth. Few cases of this rare anomaly in primary dentition have been reported in Indian population. A rare case of bilateral fusion between primary mandibular lateral incisors and canines is presented in this report.

Endodontic and post-endodontic management of a fused molar.

Treatment of fused teeth needs special care and attention to the bizarre anatomy. This paper describes root canal treatment of a fused carious tooth presenting with apical periodontitis. It is a rare case of fusion of the mandibular second molar with a paramolar. There is no literature regarding placement of crown over endodontically treated fused teeth. In this case, the fused teeth were endodontically treated and restored by a porcelain fused to metal crown.

Primary Double Tooth: A Case Report And Review of Literature.

A number of developmental anomalies can be encountered in clinical situations, which directly or indirectly can affect the treatment plan. These anomalies in the primary dentition can be of immense clinical significance since they can have a bearing on the permanent dentition. Here we report a case of fusion of a deciduous maxillary central incisor with its adjacent supernumerary tooth, presenting with the complication of dentoalvelolar abscess. Clinical observation along with periapical radiographs were used to arrive at a diagnosis. This case report emphasizes the need to attempt simple, esthetic conservative procedures before complex treatment alternatives are contemplated. Also, a review of literature is presented.

Bilateral fusion of primary mandibular lateral incisors and canines: A report of a rare case / Fusão bilateral de dentes decíduos incisivo e canino inferior: relato de um caso raro

Purpose: Synodontia or fusion is a developmental anomaly of shape of tooth formed by union of two independently developing primary or secondary teeth. Prevalence of tooth fusion is estimated at 0.5-2.5% in the primary dentition and less in permanent dentition. The bilateral type of fusion in the primary dentition is rare and is about 0.02%. This paper describes a rare case of bilateral fusion of primary mandibular lateral and canine teeth. Case description: An 8 year old girl had a complaint of unusually large sized teeth in her mandible. After physical examination and use of periapical radiographs and study models bilateral fused teeth in the mandibular lateral incisor and canine region was diagnosed. Conclusion: The bilateral fusion of primary mandibular lateral and canine teeth is a rare condition and should be carefully evaluated to diagnose any associated pathology.

Objetivo: Sinodontia ou fusão é uma anomalia de desenvolvimento da forma do dente formado pela união de dois dentes decíduos ou permanentes em desenvolvimento de forma independente. A prevalência de fusão dental é estimada em 0,5 a 2,5% na dentadura decídua e menor na permanente. O tipo de fusão bilateral na dentadura decídua é rara e aproximadamente de 0,02%. Este artigo descreve um caso raro de fusão bilateral de dentes decíduos incisivos laterais e caninos inferiores. Descrição do caso: Uma menina de 8 anos de idade tinha uma queixa clínica de dentes de tamanho grande anormal em sua mandíbula. Após exame físico e uso de radiografias periapicais e modelo de estudo, a fusão bilateral de dentes decíduos incisivos laterais e caninos inferiores foi diagnosticada. Conclusão: A fusão bilateral de dentes decíduos incisivos laterais e caninos na mandíbula é uma condição rara e deve ser cuidadosamente avaliada para diagnosticar quaisquer patologias associadas.

Value of coronal CT scan for airway disease in children / 重庆医科大学学报

Objective:To explore the value of direct coronal CT scan for airway disease in children. Methods:The clinical and imaging data of the lung and airway disease of eighty-seven cases were retrospectively analyzed.Chest film(48 with chest fluo- roscopy) the cross and coronal CT scan were performed and confirmed by tracheal endoscopy operation in all cases. Results: The main X-ray signs on chest film(fluoroscopy) and the cross CT were obstructive emphysema obstructive pneumonia or at- electasis. The coronal CT displayed 68(78.2%) cases with foreign body of which 64 cases were positive and 4 cases nega- tive;3(3.4%)cases with tracheal bronchus; 2 of which with the right upper lobe bronchus arising from the tracheal 1case with the apical and posterior segment of the right upper lobe bronchus arising from the tracheal;7(8.0%) cases with tracheal- bronchus Stenosis:1 case with the cross diameter of the tracheal narrow 4 cases with the left bronchus narrow 2 cases with tracheal narrow compressed by esophagitis foreign body;2(2.3%) cases with trachea stenosis associated with tracheal bronchus: 1 case with the cross diameter of the tracheal narrow and the tracheal diverticulum above the carina the other one with the right tracheal-bronchus,then tracheal stenosis above the carina;4(4.6%) cases with bronchitis;2(2.3%) cases with bronchus granuloma.1(1.2%)cases bronchus TB. Conclusion:Axial and Direct coronal CT scanning can demonstrate the cause of some lung and airway diseases,provide evidence for the differential diagnosis in children and be helpful in planning virtual flexible bronchoscopy.

Accessory Renal Arteries Found during Dissection / 체질인류학회지

Accessory renal artery (ARA) is a kind of developmental anomaly in renal artery. It is important in respect to clinical medicine, for example primary hypertension, renovascular disease, inferior vena caval obstruction, ureteral obstruction, occurrence of other vascular anomalies such as accessory renal veins, surgical importance and renal transplantation. However, up to few research of ARA was reported in dissection of cadavers. In our dissecting theater, 12 accessory renal arteries for 10 cadavers were found during dissection the 22 cadavers from 1995 to 1996. 1. Two cases were bilateral and 8 cases were unilateral accessory renal arteries. 2. Seven cases were left and 5 cases were right accessory renal arteries. 3. Two cases originated at the abdominal aorta between celiac trunk and superior mesenteric artery, 7 cases originated between superior mesenteric artery and inferior mesenteric artery, and 3 cases originated below inferior mesenteric artery. 4. Seven cases have no branches during their courses, 4 cases have 3 branches, and a case has 2 branches. 5. Seven cases entered into renal parenchyma through renal hilum, 5 cases entered into apical and arterosuperior segments, and 6 cases entered into inferior segment.

A Case of Late Presentation of Congenital Cystic Adenomatoid Malformation of the Lung / 결핵

Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAM is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so tare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a form of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes: cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multicystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postinflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complete removal of the involved lobe. Partial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

Surgical Outcome of Familial Lens Dislocation in Two Families

The congenital lens dislocation is a uncommon genetic disease, which is inherited autosomal dominant mannner and less likely autosomal recessive. The cause of the disease has not been fully understood although there are some hypothesis based on developmental anomaly. There are controversies about the management of the disease. In these days, early surgery is usually performed to prevent the compilcation. The authors experienced five patients in two families and three of them were operated with good results. They showed autosomal dominaot inheritance pattern. So, we present five cases of uncommon congenital lens dislocation with a brief review of literature.

A Case of Rieger's Syndrome Associated with Controlled Glaucoma

Rieger's syndrome is a rare autosomal dominant developmental anomaly characterized by ocular and systemic abnomalities. These disorders are typically bilateral and usually diagnosed at birth or in the childhood. The most common ocular defects are hypoplasia of the iris, a prominent Schwalbe's line with iris strands and corectopia. A large number of patients have glaucoma due to devel-opmental defects of the anterior chamber angle structure. The authors experienced a case of Rieger's syndrome which was found in 31-year-old female, who had characteristic ocular and other systemic abnormalities. Associated glaucoma was well controlled by filtering surgery.