Living domino liver transplantation in an adult with congenital absence of portal vein.

Congenital absence of the portal vein (CAPV) is a rare malformation of the splanchnic venous system. Although CAPV is usually detected in the pediatric age group, our patient was a 35-year-old woman. She had been diagnosed with CAPV in 1996 when she was 27 years old. In 1998, she was placed on hemodialysis due to chronic renal failure. After several episodes of encephalopathy in 2002, liver transplantation (LT) was recommended to her and her family. Since there was no suitable living donor candidate, she was put on the waiting list for a deceased donor liver transplant in Japan. In 2004, her ammonia level increased to around 300 microg/dl, and she went into a coma lasting for three days. After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. Her portal vein, which had drained directly into the inferior vena cava (IVC), was transected together with a cuff of the IVC wall and anastomosed to the graft liver portal vein in an end-to-end fashion. In conclusion, liver transplantation proved to be a safe and effective way to save this patient and improve her quality of life.     

Repair of bile duct defect with full-thickness vascularized jejunal patch

The use of a vascularized jejunal patch for the reconstruction of bile duct injuries is presented. The method has been used in 1 patient with a common bile duct stricture and in 1 patient with a noncircumferential bile duct defect. The procedure has the advantages of technical simplicity, primary mucosal coverage, lasting elasticity, and minimal risk for stricture formation.

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Resumen Se presenta el uso de un parche vascularizado de yeyuno para la reconstrucción de lesiones del canal colédoco. Se reseca un segmento móvil del yeyuno lo suficientemente amplio para cubrir el defecto, asegurándose de que existe buena irrigación para el segmento, visualizando los vasos por transiluminación con luz ordinaria. La continuidad del intestino es restablecida mediante anastomosis término-terminal. Se utiliza la parte mesentérica del segmento intestinal, el cual es ascendido por vía retrocólica. Se sutura mucosa con mucosa con una sola capa de puntos separados de ácido poliglicólico 5-0. Se coloca un tubo-en-T para drenaje, el cual es retirado a las 6 semanas. El método ha sido utilizado en un caso de estenosis del colédoco y en un caso con un defecto circunferencial del mismo canal biliar. El procedimiento tiene la ventaja de la simplicidad técnica, la cobertura primaria de la mucosa, la elasticidad permanente y un mínimo riesgo de que se produzca estenosis.

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Résumé L'emploi d'un patch jéjunal bien vascularisé pour traiter les blessures de la voie biliaire fait l'objet de l'article. La méthode a été utilisée pour un cas de sténose de la voie biliaire principale et un cas de plaie n'intéressant pas la totalité de la circonférence du canal biliaire. Elle a pour avantages sa simplicité technique et le fait qu'elle assure une couverture muqueuse, sa persistante élasticité et le fait qu'elle présente un risque réduit de sténose.

     

Ueber primäre Hydronephrose

Ohne Zusammenfassung     

Congenital hydrocephalus and L1 disease: a case report (2009: 7b)

L1 disease is the most common genetic cause of congenital hydrocephalus. Mutations in the L1CAM gene are associated with an overlapping clinical spectrum of four X-linked neurological conditions, characterized by hydrocephalus, mental retardation, lower limb spasticity and adducted thumbs. Brain anomalies are frequently present in L1 disease. We describe these anomalies by reporting a case of a male newborn presenting with congenital hydrocephalus along with corpus callosum agenesis and enlargement of the massa intermedia. These findings, in association with the presence of clasped thumbs, raised the suspicion of L1 disease, which was confirmed by the detection of a mutation in the L1CAM gene. In cases of congenital hydrocephalus, recognition of the brain anomalies associated with L1 disease may contribute to pursuing the genetic analysis needed for the diagnosis and genetic counseling.     

Complications of temporomandibular joint arthroscopy: a retrospective analytic study of 670 arthroscopic procedures.

PURPOSE: Temporomandibular joint (TMJ) arthroscopy has been considered a safe surgical procedure in the treatment of TMJ derangement. However, it is not exempt from complications. This study evaluates the complications of arthroscopy in patients with internal derangement of TMJ. PATIENTS AND METHODS: Five hundred consecutive patients (670 joints) with TMJ derangement who underwent arthroscopy between 1995 and 2004 were retrospectively analyzed. All the patients were classified as II to V in the Wilkes classification. Lysis and lavage, electrocautery of the posterior ligament, injection of corticoids, injection of ethanolamine, myotomy of lateral pterygoid muscle attachments, myotomy and electrocautery, motor debridement, injection of sodium hyaluronate, and meniscal suture were performed in different patients. RESULTS: Complications were recognized during or immediately after the surgery. They were observed in 5 of 341 (1.26%) arthroscopies of the right TMJ and 4 of 329 (1.21%) arthroscopies of the left TMJ. A 1.34% complication rate was found in the whole series. No blood clots within the external auditory canal were observed. Bleeding within the superior TMJ space was observed in 57 cases (8.5%), 36 of them in the right TMJ and 21 in the left TMJ, but they were not considered as true complications. Lacerations of the external auditory canal were found in 2 cases (0.3%), with no cases of perforation of the tympanic membrane. Lesion of the auriculotemporal nerve was observed in a case. Paresia of the facial nerve was found in 4 cases (0.6%). Alteration of visual accuracy of the ipsilateral eye was also observed in a patient immediately after the surgery. CONCLUSION: Special care must be taken to reduce complications within the upper joint space by means of an adequate instrumentation and by paying attention to essential points of the arthroscopic technique.