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1/12. A rare case of completely isolated duplication cyst of the alimentary tract.

    A rare case of a gastrointestinal cystic duplication in a 7-day-old infant is described. The duplication diagnosed antenataly at 25 weeks of gestation was found during surgery to be separated from the gastrointestinal tract, hanging on a vascular pedicle, with no connection to the mesentery. The duplication was excised, and postoperative follow-up of 14 months was uneventful. The possible pathogenesis of this malformation is discussed.
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2/12. Separation of omphalopagus conjoined twins using combined caudal epidural-general anesthesia.

    PURPOSE: To describe the anesthetic management of newborn omphalopagus conjoined twins undergoing a series of diagnostic and surgical procedures which culminated in successful separation at one month of age. CLINICAL FEATURES: Evaluations of the extent of shared organ systems were carried out without the need for anesthesia. The twins were anesthetized twice, once for insertion of skin expanders, and later for surgical separation. Various airway management techniques were utilized. To facilitate surgical separation, caudal epidural catheters were inserted in an effort to provide both operative and post-operative analgesia. In addition, prior to the induction of anesthesia, the extent of cross-circulation between twins was assessed. CONCLUSION: Caudal epidural catheters can be used to provide both operative and post-operative analgesia. Early extubation, another benefit of regional analgesia, was not achieved because both twins developed respiratory failure in the immediate postoperative period. Testing for the extent of cross-circulation between twins proved valuable, allowing for detailed scripting of the complex induction sequence and airway management.
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keywords = operative
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3/12. anal canal duplication in infants and children--a series of 6 cases.

    The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea. Fistulography revealed a tubular structure in five cases and a cystic structure in one case, behind the normal anal canal, in one case communicating with it. A presacral sacrococcygeal teratoma was found in two children and in one case it was visualised by preoperative US in an infant with a lumbosacral myelomeningocele. Surgical excision was performed by a perineal approach in 5 cases, by a combined sacral and perineal approach in the last case, because of the associated teratoma. Non-invasive preoperative investigations, consisting of a pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved for special indications. Surgical treatment restores a normal perineal aspect, without sequelae, and avoids complications like those described in other types of digestive duplications: infection, ulceration, bleeding, malignant changes during later adult life. Associated anomalies are frequently described in the literature, especially presacral tumours (16%) and anorectal malformations (21%); they can influence the management, the surgical approach and the functional prognosis.
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ranking = 2
keywords = operative
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4/12. Intralingual foregut duplication cyst in a newborn.

    Congenital lingual cystic masses are challenging entities that can be detected prenatally or discovered in various forms after birth. We report what we believe is only the ninth case of an intralingual foregut duplication cyst that was lined with gastric mucosa. The preoperative evaluation of lingual cystic masses in newborns can include palpation, high-resolution sonography, computed tomography (CT), or magnetic resonance imaging (MRI). However, CT and MRI can pose a risk to the infant because they require sedation in order to properly position the patient for imaging. In our patient, we found that high-resolution sonography was most useful in revealing the nature of the mass as a nonvascular cystic lesion and in delineating its extension. We excised this lesion via bipolar cautery, which we prefer to cold-knife or CO2 laser surgery.
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5/12. Laparoscopic treatment of a gastric duplication cyst: report of a case.

    Gastric duplications are rare benign tumors that are mainly discovered in relatively young patients. Although laparoscopic surgery, a less invasive surgical procedure, should be used for this condition, its application in the treatment of this disease has not yet been reported. A 14-year-old girl visited our hospital with the chief complaint of repeated episodes of upper abdominal pain. Because diagnostic imaging revealed a cystic tumor in the dorsal stomach, laparoscopic surgery was performed. The cystoma was intraoperatively found to contiguous with the posterior wall of the gastric cardia and was thus diagnosed to be a gastric duplication. With the intraoperative aid of gastroscopy, we were able to remove the cystoma from the continuous gastric wall safely without causing either a perforation or any other injury to the stomach. Gastric duplication is a congenital disease, and thus the majority of such cases are diagnosed in childhood. A laparoscopic resection of a duplicated stomach is less invasive and esthetically superior to other methods, and therefore it is considered to be a useful therapeutic modality.
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6/12. Hypoplasia of the right hepatic lobe combined with a floating gallbladder.

    Agenesis or hypoplasia of the right hepatic lobe combined with a floating gallbladder is an extremely rare condition. We report a case of hypoplasia of the right hepatic lobe, discovered in a 65-year old female. This was an incidental finding at CT scan for staging of a right colonic cancer. The CT evidenced the presence of a hypoplastic right lobe, while the left lobe was diffusely enlarged. Furthermore, the gallbladder was described as floating with partially calcified walls. The diagnosis of this rare anomaly was confirmed intraoperatively. The patient underwent right hemicolectomy and cholecystectomy. Biopsies were taken from both right and left hepatic lobes, revealing the presence of normal hepatic parenchyma. Since all causes of acquired atrophy of the liver had been ruled out, we considered this case to be of congenital origin.
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keywords = operative
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7/12. Alimentary tract duplications in infants and children.

    This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pecs, hungary, and at the Department of Pediatric Surgery of the Medical Academy of Dresden, germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 percent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoraco-abdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.
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keywords = operative
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8/12. Annular pancreas in two consecutive siblings: an extremely rare case.

    Annular pancreas is the rare congenital anomaly where the pancreas forms a full or incomplete ring around the second segment of the duodenum, causing various degrees of stenosis or atresia. It is estimated that it appears in 1 out of 12 000-15 000 births of living neonates and until now, in the literature, only 6 cases have been reported among individuals of the same family. We present the case of two siblings, a boy and a girl, with annular pancreas from consecutive pregnancies of the same couple. Both neonates had a prenatal diagnosis of duodenal obstruction and they underwent duodenoduodenal, proximal transverse to distal longitudinal anastomosis. Furthermore, the girl had a mobile ascending colon. Their postoperative condition was perfect. The case we are reporting is an addition to the other 6 cases of familial presentation of annular pancreas and is similar to one of them. In these families, a total of 16 persons present this congenital anomaly while 14 are seemingly healthy. Twelve of the affected persons are female and 4 male. In conclusion, it can be stated that female individuals seem to have a greater propensity to transmit the disease to their descendants, compared to males, suggesting the possible action of an autosomal recessive sex-influenced gene. The recording of such rare family cases should be encouraged, in order to fully recognize a possible type of inherited transmission.
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keywords = operative
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9/12. Colonic duplication: diagnostic dilemma.

    Colonic duplication is a rare congenital anomaly which presents as a diagnostic and therapeutic challenge. We report a 5-year-old boy with colonic duplication who required multiple hospital admissions, multiple diagnostic tests, and evaluation by various clinical specialists before the diagnosis was made intraoperatively. He was known to have left renal hypoplasia, low spinal abnormalities, hypoplasia of the left hemipelvis, and mild hypoplasia of the left lower limb, all of which were considered to be a variant of caudal regression syndrome.
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keywords = operative
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10/12. Computed tomography and ultrasonography of carcinoma in duplication cysts.

    The CT and ultrasound findings of malignancy arising in walls of alimentary tract duplication cysts are previously unreported. We describe two cases where identification of both cyst and tumor nodule and separation of the mass from other visceral organs was achieved. We review the English literature reporting this unusual condition and suggest that CT and ultrasound are of great value in accurate preoperative diagnosis.
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ranking = 1
keywords = operative
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