Adult ; Branchial Region ; abnormalities ; Branchioma ; congenital ; diagnosis ; Diagnostic Errors ; Female ; Fistula ; congenital ; diagnosis ; Humans

We report a case of congenital lacrimal sac fistula located on the temporal side of the lateral canthus. A systemically healthy 1-year-old girl came to the outpatient clinic with a complaint of tearing on the temporal side of the right lateral canthus since birth. On examination, a small orifice was found in the skin on the temporal side of the lateral canthus. There was no evidence of inflammation or swelling within the opening. Surgeons carried out an operation under general anesthesia. They passed a probe through the lacrimal orifice and advanced it toward the lacrimal sac. Next, they introduced saline to the inferior punctum and found that it drained to the lateral fistula. The lower lid stretched as the dissected fistula was pulled. After the operation, the patient was free of the symptom. This paper is to report a case of congenital lacrimal sac fistula located on the temporal side of the lateral canthus.
Diagnosis, Differential ; *Eye Abnormalities ; Female ; Fistula/*congenital/diagnosis ; Humans ; Infant ; Lacrimal Apparatus/*abnormalities ; Lacrimal Apparatus Diseases/*congenital/diagnosis

OBJECTIVETo summarize the clinical characteristics, diagnosis and surgical in-treatment results of congenital coronary artery fistulas (CAF) in adults.

METHODSFourteen patients (8 men, 6 women), aged from 18 to 60 years with a mean of 32 +/- 13 years, underwent surgical correction of CAF between March 1985 and April 2002. Eleven of the 14 patients (78.57%) were symptomatic. The diagnosis of CAF was made by echocardiography or angiocardiography preoperatively. The fistulae originated from the right, left and double coronary arteries in 10 (71%), 3 (21%) and 1 (7%) patient(s), respectively. The fistulae drained into the right ventricle (8 patients), left ventricle (4), right atrium (1) and pulmonary artery (1), respectively. The diameter of fistulae ranged from 0.30 to 1.80 cm with a mean of (1.16 +/- 0.49) cm. There were 6 CAF patients associated with coronary artery aneurysms and 4 CAF patients with other coexisting cardiac defects. The distal fistulae were closed in 10 patients with cardiopulmonary bypass (CPB) and 4 patients without CPB. The coexisting defects were corrected simultaneously.

RESULTSThere was no early and late death. One patient had low cardiac output syndrome and cured during early postoperative period. Twelve patients (85.71%) were followed up for a mean period of 3.35 +/- 4.28 years without myocardial ischemia or infarction and recurrent fistulae. Heart function was improved to NYHA functional class I in 11 patients and class II in 1 patient.

CONCLUSIONSAll adult patients with CAF who have demonstrable hemodynamic and cardiovascular morphological changes should be surgically treated as early as possible. The appropriate surgical management and reliable myocardial protection are key points of good surgical results.

Adolescent ; Adult ; Coronary Vessel Anomalies ; diagnosis ; surgery ; Female ; Fistula ; congenital ; diagnosis ; surgery ; Humans ; Male ; Middle Aged

Adolescent ; Adult ; Ear Diseases ; congenital ; diagnosis ; surgery ; Female ; Fistula ; diagnosis ; surgery ; Humans ; Male ; Neck ; Young Adult

To discuss the diagnosis and therapy of congenital vesicovaginal fistula (VVF). We reported 1 case of pregnancy and congenital VVF and summarized the pathogenesis and characters. Congenital VVF is extremely rare and characterised by continuous urinary leakage,cyclical hematuria with severe suprapubic pain. It is always associated with other urinary tract abnormalities, urinary tract infection and stone. The pathogenesis is related with genetic, environment, drugs, radiation and incomplete fusion of mullerian. The diagnosis relies on clinical manifestation and radiological examinations.Surgical resection is the key to treatment. Pregnancy and congenital VVF is extremely rare and always misdiagnosised associated with other urinary tract abnormalities. Early diagnosis and making sure the location, size and relationship with other tissues are important. Reasonable preoperative preparation, surgical and postoperative surgical care are the key for treatment.
Adult ; Female ; Humans ; Pregnancy ; Pregnancy Complications ; diagnosis ; Vesicovaginal Fistula ; congenital ; diagnosis

Pulmonary arteriovenous fistula (PAVF) is abnormally dilated vessels that provide a right-to-left shunt between pulmonary artery and pulmonary vein and is clinically divided into simple and complex type. Here, we report four cases of surgically resected sporadic PAVFs presenting various clinical and histologic spectrums. Cases 1 (a 57-old-female) and 2 (a 54-old-female) presented as incidentally identified single aneurysmal fistulas and the lesions were surgically removed without complication. On the other hand, case 3 (an 11-old-male) showed diffuse dilated vascular sacs involving both lungs and caused severe hemodynamic and pulmonary dysfunction. Embolization and surgical resection of the main lesion failed to relieve the symptoms. Case 4 (a 36-old-male) had a localized multiloculated cyst clinically mimicking congenital cystic adenomatoid malformation. Microscopically, the lesion consisted of dilated thick vessels, consistent with the diagnosis of fistulous arteriovenous malformation/hemangioma.
Aneurysm ; Arteriovenous Fistula* ; Arteriovenous Malformations ; Cystic Adenomatoid Malformation of Lung, Congenital ; Diagnosis ; Fistula ; Hand ; Hemodynamics ; Lung ; Pulmonary Artery ; Pulmonary Veins

We have reviewed 12 cases of congenital T-E fistula during the period of 20 years from Jan. 1957 to Dec. 1976, who were hospitalized at Severance hospital and WonJu Christian Hospital. The diagnosis was confirmed by esophagography and surgery. And we obtained following results. 1. Ten cases out of 12 were type A according to Holinger's classification and 7 were male. 2. The most common complication in those pregnancy was hydramnios which was observed in 5 cases, 6 cases of 12 have combined anomalies; congenital heart disease (2 cases), imperforated anus (2 cases) 3. The common clinical menifestation were regurgitation after feeding, profuse oral secretion, respiratory difficulty. 4. Four out of 5 cases who had corrective surgery within 72 hours after birth were survived and 5 cases had surgical treatment after 72 hours were died. 5. The complication after surgery were esophageal stricture, aspiration peneumonia and pneumothorax.
Anal Canal ; Classification ; Diagnosis ; Esophageal Stenosis ; Fistula ; Gangwon-do ; Heart Defects, Congenital ; Humans ; Male ; Parturition ; Pneumothorax ; Polyhydramnios ; Pregnancy ; Tracheoesophageal Fistula*

Esophageal stenosis due to tracheobronchial remnants is a rare cause of congenital esophageal stenosis. The cause is thought to be esophageal sequestration of tracheobronchial remnants during embryonic separation. Errors in diagnosis are common and high index of suspicion is required for accurate diagnosis, and resection of primary site with anastomosis is recommanded. Although a rare entity, esophageal stenosis due to tracheobronchial remnants should be considered in patients with esophageal stenosis, when esophageal stenosis fails to respond to standard therapy including bougienage and balloon dilatation, and esophagoscopy shows normal mucosa on the stenotic segment. Four cases of congenital esophageal stenosis due to tracheobronchial remnants were reported, the first case without accompanied anomaly, the second case accompanied by tracheoesophageal fistula, the third case accompanied by Down syndrome, and the last case accompanied by tracheoesophageal fistula and congenital heart disease.
Diagnosis ; Dilatation ; Down Syndrome ; Esophageal Stenosis* ; Esophagoscopy ; Heart Defects, Congenital ; Humans ; Mucous Membrane ; Tracheoesophageal Fistula

PURPOSE: To assess the usefulness of MRI in preoperative diagnosis of congenital anorectal malformation MATERIALS AND METHODS: MR findings of 11 cases with surgically proved anorectal malformations were retrospectively reviewed and compared with operative findings, according to the level of atresia, the development of sphincter muscle, fistula and associated anomalies of other organs. RESULTS: Four of 11 cases were low type of anorectal atresia, 3 cases were intermediate type, and 3 cases were high type. There was one case of Currarino triad with low type of anorectal stenosis. MRI demonstrated the levels of atresia correctly in all cases and revealed fistulas in all high type of anomalies. Degrees of the development of the sphincter muscles were good in all cases of low types and fair in a case of intermediate type and an anorectal stenosis, whereas the development was poor in 2 cases of intermediate type and all 4 cases of high type. The associated anomalies in anorectal malformation were renal agenesis, congenital hip dysplasia and sacral defect with presacral teratoma in Currarino triad. CONCLUSION: MRI was a simple and useful study to confirm the level of atresia, fistula and associated anomalies in the diagnosis of the congenital anorectal malformation.
Constriction, Pathologic ; Diagnosis ; Fistula ; Hip Dislocation, Congenital ; Magnetic Resonance Imaging ; Muscles ; Retrospective Studies ; Teratoma

PURPOSE: The congenital H-type rectovestibular fistula, a fistula between the anorectum and genital tract besides a normal anus is a rare variant of anorectal deformities. This disease needs proper treatment but there are no standard of diagnosis and treatment. The purpose of this report is to review a 13-year experience of approach and management for H-type rectovestibular fistula at a single institution. METHODS: From February 2002 to August 2015, we cared for 11 patients who had congenital H-type rectovestibular fistula and reviewed their clinical presentation, accompanied anomalies, diagnostic modalities, operative technique, and postoperative progress. RESULTS: Most patients with H-type rectovestibular fistula presented with symptoms including vestibular defecation and major labial abscess. We could find the fistula tract in most of patients by fistulography using contrast dye. All of the patients had been operated. There were 2 recurrences after surgical treatment who had inflammation and infection associated with the fistula. All other patients recovered without complications. CONCLUSION: We think the operation including fistulectomy and repair of perineal body through a transanal approach can be a feasible option to the congenital H-type rectovestibular fistula. Also, combined inflammation and infection should be treated prior to surgery to reduce postoperative complications.
Abscess ; Anal Canal ; Congenital Abnormalities ; Defecation ; Diagnosis* ; Fistula* ; Humans ; Inflammation ; Postoperative Complications ; Recurrence