Acetabulum ; abnormalities ; diagnostic imaging ; Adolescent ; Adult ; Cartilage ; abnormalities ; Female ; Hip Dislocation, Congenital ; diagnosis ; surgery ; Hip Joint ; abnormalities ; surgery ; Humans ; Ligaments ; abnormalities ; Male ; Orthopedic Procedures ; Young Adult

OBJECTIVETo analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment.

METHODSWe reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed.

RESULTSThe exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the mean air-conductive threshold were decreased from (60.0±11.4)dBHL to (32.2±12.1)dBHL(P=0.000); and the mean ABG were decreased from (43.2±12.0)dB to (16.3±9.4)dB(P=0.000).

CONCLUSIONSCongenital middle ear malformations, otosclerosis, congenital cholesteatoma are the most common causes in unilateral conductive hearing loss with an intact tympanic membrane. The diagnosis rate can be improved by analyzing the clinical features. Through exploratory tympanotomy and hearing reconstruction, we can clarify the diagnosis and achieve a satisfying hearing recover.

Adolescent ; Adult ; Aged ; Audiometry ; Child ; Cholesteatoma ; congenital ; pathology ; Diagnosis, Differential ; Ear Ossicles ; pathology ; Ear, Middle ; abnormalities ; Female ; Hearing Loss, Conductive ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Middle Ear Ventilation ; Otosclerosis ; pathology ; Tympanic Membrane ; Young Adult

Rectovestibular fistula is the most common type of anomaly found in a female newborn with anorectal malformation. However, when the baby is found to have two orifices in the introitus, rectovaginal fistula is much less common and suspected. The rare differential diagnosis of Müllerian agenesis, a condition in which the rectum shifts anteriorly and the vagina is absent, is seldom considered. In many cases, the diagnosis of Müllerian agenesis is made only during definitive anorectoplasty. In view of its impact on management, a proper examination under anaesthesia, imaging studies and a diagnostic laparoscopy may be required to confirm the presence or absence of Müllerian structures in such patients. We herein describe a patient with the rare coexistence of VACTERL association and Müllerian agenesis, and discuss the management of anorectal malformations in female patients with Müllerian agenesis.
Abnormalities, Multiple ; diagnosis ; Anal Canal ; abnormalities ; surgery ; Anorectal Malformations ; Anus, Imperforate ; complications ; diagnosis ; surgery ; Child ; Diagnosis, Differential ; Esophagus ; abnormalities ; Female ; Heart Defects, Congenital ; complications ; Humans ; Infant, Newborn ; Kidney ; abnormalities ; Laparoscopy ; Limb Deformities, Congenital ; complications ; Mullerian Ducts ; abnormalities ; Rectal Fistula ; diagnosis ; Rectum ; abnormalities ; surgery ; Spine ; abnormalities ; Trachea ; abnormalities ; Vagina ; abnormalities

Lymphangioma is a congenital abnormality of the lymphatic system detected primarily in early childhood. There are rare reports of mediastinal lymphangioma in older adults. We hereby report on a 66-year-old female patient who underwent kidney transplantation 20 years previously and who developed pathologically confirmed solitary mediastinal lymphangioma 1 year ago. Chest radiography showed a mediastinal nodule, which was not observed 2 year previously, therefore she was referred to the pulmonary division. She had no symptoms, and chest computed tomography demonstrated a 25-mm, well-defined, low-density nodule located at the anterior mediastinum. The size of the nodule had increased from 25 mm to 34 mm 1 year later, and it was completely resected via video-assisted thoracic surgery. The histological diagnosis was cystic lymphangioma. Therefore, we recommend that clinicians consider cystic lymphangioma as a possible diagnosis even in older patients with a mediastinal cystic mass that shows progressive enlargement.
Adult ; Aged* ; Congenital Abnormalities ; Diagnosis ; Female ; Humans ; Kidney Transplantation* ; Kidney* ; Lymphangioma* ; Lymphangioma, Cystic ; Lymphatic System ; Mediastinal Cyst* ; Mediastinum ; Radiography ; Thoracic Surgery, Video-Assisted ; Thorax

Angiocardiography ; Anti-Infective Agents ; therapeutic use ; Aorta ; abnormalities ; surgery ; Bronchopneumonia ; diagnosis ; drug therapy ; Cardiac Surgical Procedures ; methods ; Ductus Arteriosus, Patent ; diagnosis ; surgery ; Female ; Heart Defects, Congenital ; diagnosis ; surgery ; Humans ; Infant ; Pulmonary Artery ; abnormalities ; surgery ; Tomography, Spiral Computed

Midgut malrotation includes a range of developmental abnormalities that occur during fetal intestinal rotation. Manifestations of intestinal malrotation are generally seen in the paediatric population and are uncommon in adults. Symptomatic patients may present with either acute abdominal pain due to midgut volvulus, or chronic abdominal pain due to proximal midgut partial obstruction in the presence of congenital bands. A limited number of paediatric cases of duodenal occlusion due to duodenal malrotation has been previously reported in the medical literature. We herein report the case of a 57-year-old woman who presented with duodenal obstruction due to organoaxial partial rotation of the distal duodenum associated with midgut malrotation. This is probably the first of such a case diagnosed in adulthood reported in the medical literature. Our patient underwent Roux-en-Y duodenojejunostomy and had symptomatic relief following the successful surgery.
Duodenal Obstruction ; congenital ; diagnosis ; surgery ; Duodenostomy ; Duodenum ; abnormalities ; Female ; Humans ; Middle Aged ; Sri Lanka ; Treatment Outcome

In hallux valgus, one of the most common conditions affecting the forefoot, the first metatarsophalangeal joint is progressively subluxed due to lateral deviation of the hallux and medial deviation of the first metatarsal. Patients usually complain of medial prominence pain, commonly referred to as "bunion pain," plantar keratotic lesions, and lesser toe deformities such as hammer toe or claw toe deformities. The etiology of hallux valgus is multifactorial. Narrow high-heeled shoes or excessive weight-bearing have been suggested to be extrinsic factors contributing to the condition, and many other intrinsic factors also exist, such as genetics, ligamentous laxity, metatarsus primus varus, pes planus, functional hallux limitus, sexual dimorphism, age, metatarsal morphology, first-ray hypermobility, and tight Achilles tendon. When we evaluate patients with hallux valgus, careful history taking and meticulous examination are necessary. On the radiographic evaluation, we routinely measure the hallux valgus angle, intermetatarsal angle, and distal metatarsal articular angle, which are valuable parameters in decision making for bunion surgery. To prevent the development and progression of hallux valgus, a soft leather shoe with a wide toe box is usually recommended. The use of a toe separator or bunion splint may help in relieving symptoms. The purpose of hallux valgus surgery is to correct the deformity and maintain a biomechanically functional foot. When we decide on an adequate surgical option, we should consider the patient's subjective symptoms, the expectations of the patient, the degree of the de-formity, and the radiographic measurements in order to correct the deformity and prevent complications after surgery.
Achilles Tendon ; Congenital Abnormalities ; Decision Making ; Diagnosis ; Flatfoot ; Foot ; General Surgery ; Genetics ; Hallux Limitus ; Hallux Valgus* ; Hallux* ; Hammer Toe Syndrome ; History ; Humans ; Intrinsic Factor ; Ligaments ; Metatarsal Bones ; Metatarsophalangeal Joint ; Shoes ; Splints ; Toes ; Weight-Bearing

OBJECTIVETo explore the diagnostic and therapeutic methods for perioperative children with congenital heart disease (CHD) with airway stenosis in pediatric intensive care unit (PICU).

METHODFiberoptic bronchoscopy was used for the diagnosis of 100 CHD cases in PICU who were clinically considered to have possible airway malformation because of complicated difficult-to-control lung infection, atelectasis and failure with the ventilator after surgery from January 2010 to October 2011. Cases who were confirmed to have severe airway stenosis by bronchoscopy and weaning from the ventilator after surgery were treated with balloon expandable stents into the desired position in the bronchoscopy.

RESULTThere were 73 cases (73%) of CHD patients with airway abnormalities, including 31 cases of severe stenosis (31%), moderate stenosis in 29 cases (29%), mild stenosis in 13 cases (13%). Nine of the 10 children in whom the mechanical ventilation was hard to be stopped after surgery because of severe airway stenosis were weaned from mechanical ventilation successfully by fiberoptic bronchoscopy, while one case died from primary disease with severe sepsis after the placement of bronchial stents.

CONCLUSIONCHD children with difficult-to-control lung infection, atelectasis and failure with ventilator after surgery are often complicated with airway abnormalities. The therapeutic bronchoscopy with airway stent can be used for cases with weaning from the ventilator because of severe airway stenosis.

Airway Obstruction ; diagnosis ; etiology ; therapy ; Bronchoscopy ; methods ; Constriction, Pathologic ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; complications ; diagnosis ; surgery ; Humans ; Infant ; Infant, Newborn ; Intensive Care Units, Pediatric ; Lung Diseases ; diagnosis ; etiology ; therapy ; Male ; Perioperative Period ; Respiration, Artificial ; Stents ; Trachea ; abnormalities ; Tracheal Stenosis ; diagnosis ; etiology ; therapy ; Treatment Outcome

OBJECTIVETo evaluate the clinical features, diagnosis, treatment, and outcome of intralobar pulmonary sequestration (ILS).

METHODSPatients who were diagnosed with ILS in our hospital between January 1988 and January 2009 were retrospectively reviewed. We recorded the clinical symptoms, imaging findings, operative technique, complications, and outcome of these patients.

RESULTSForty-seven patients (25 men and 22 women) with an average age of 32.3 years were enrolled. Forty-two patients had symptoms including cough and hemoptysis. Chest X-ray, computed tomography (CT), magnetic resonance imaging (MRI), and angiography were performed. Thoracotomy was performed in 45 patients, while thoracoscopy was performed in 2 patients. Lobectomy was the most common treatment procedure. Massive bleeding developed in 2 patients due to injury of aberrant supplying artery intraoperatively, 1 patient had atrial fibrillation, 1 patient had thrombosis of upper extremity postoperatively. All patients were confirmed the diagnosis pathologically, 4 accompanied with bronchogenic cyst, 15 with bronchiectasis, 8 with infection, 2 with aspergilloma, and 1 with carcinoid. No late complications occurred.

CONCLUSIONSILS is rare, surgery is recommended because some patients may have potential severe complications. Contrast enhanced CT and three-dimensional reconstruction is the best diagnostic method. Both thoracotomy and thoracoscopy are appropriate for the selected candidates.

Adolescent ; Adult ; Bronchopulmonary Sequestration ; diagnosis ; diagnostic imaging ; surgery ; Child ; Congenital Abnormalities ; diagnosis ; diagnostic imaging ; surgery ; Female ; Humans ; Male ; Middle Aged ; Radiography ; Retrospective Studies ; Treatment Outcome ; Young Adult

Ear Diseases ; congenital ; diagnosis ; surgery ; Ear, External ; abnormalities ; Ear, Middle ; abnormalities ; Humans