Kuo-Ting Chen

The surgical technique for the complete unilateral cleft lip-nasal deformity

Over a period of 17 years, gradual modifications of the Millard rotation advancement cheiloplasty were made based on the experience of treating a large number of clefts seen in the Chang Gung Craniofacial Center. Emphasis is made on primary nasal correction and definitive repair of the lip. Some of the important aspects of this repair include the following: (1) no dissection over the maxilla; (2) primary nasal correction; (3) lining the pyriform area with an inferior turbinate mucosal flap; (4) complete wound closure of all areas to prevent contracture; (5) alar transfixion sutures for creating an alar groove and fixation of the lower lateral cartilage; (6) reconstruction of deficient vermilion on the cleft side of the Cupids bow, creating a parallel “red line” and “white skin roll”; (7) dissection of muscle and skin to minimize horizontal scars extending beneath or around the ala; (8) no “back-cuts”, using a small triangular skin flap above the =ldwhite skin roll” whenever necessary to achieve adequate rotation without distortion of the philtral column; (9) postoperative splinting with a silicone nasal conformer. Preoperative evaluation and measurement of the pathological deformity will help achieve a better result. Also, preoperative measurements are useful in evaluation of postoperative results.

Massive intratumor hemorrhage in facial plexiform neurofibroma

Plexiform neurofibromatosis is a feature of von Recklinghausens disease. Head and neck lesions may produce varying degrees of cosmetic and functional deformity. However, life‐threatening hemorrhage into facial plexiform neurofibromas has not been previously reported.

Facial elephantiasis neurofibromatosa ― excision and skin graft

A case of left facial elephantiasis neurofibromatosa was treated with near-total excision and skin graft. Results were favorable. Repositioning of canthal ligaments, preserving part of relatively normal masseter muscle and primary nasal reconstruction with forehead flap were performed. The skin graft over the myxomatous muscle was successful. Troublesome bleeding was avoided or controlled by incising the normal skin, subperiosteal dissection, hypotensive anesthesia, and chromic catgut suture ligature.

Proximal Deletion of the Long Arm of Chromosome 1: [del(1)(q23–q25)]

A patient with bilateral complete cleft of the lip and palate (CLP) had a proximal deletion of the long arm of chromosome 1 (1q). This rare chromosomal abnormality was characterized by pre- and postnatal growth retardation, psychomotor retardation, and specific craniofacial and other systemic anomalies. There is a high incidence of CLP in proximal 1q deletion syndrome, especially bilateral CLP. Twelve other cases reported in the literature having this deletion and associated anomalies were reviewed.

Early Incarcerated Diaphragmatic Hernia Following Right Donor Hepatectomy: A Case Report

Right diaphragmatic hernia after donor hepatectomy is extremely rare. The occurrence is usually late. We present a case with early occurrence complicated with small bowel strangulation. Early detection and emergency surgical repair relieved the problem quickly. Predisposing factors are discussed. To avoid such a complication is very important.

Loss of Speech After Living-Related Donor Liver Transplantation: Detection of the Lesion by Diffusion Tensor Image

INTRODUCTION Loss of speech after living-related liver transplantation is uncommon. Either immunosuppressive agents, related sequelae, or a neurological event may cause it. CASE REPORT A 46-year-old man developed dysarthria and dysphagia on the 10th day after living-related donor liver transplantation for alcoholic cirrhosis with Child-Pugh class C. Brain magnetic resonance images and electroencephalograms could not detect any lesion, but the diffusion tensor image showed a subacute lacunar infarction at right midbrain. The patients speech improved 1 month after rehabilitation. CONCLUSIONS Some unexpected neurological events, such as loss of speech, may occur after liver transplantation. The differential diagnosis becomes very important before active treatment. Magnetic resonance imaging supplemented with diffusion tensor imaging is an effective imaging study in establishing the diagnosis.

Repeated introperative cholangiography is helpful for donor safety in the procurement of right liver graft with supraportal right bile duct variants in living-donor liver transplantation.

BACKGROUND Despite recent advances in preoperative diagnostic imaging and operative techniques, biliary variation of the donor still remains a challenge in the procurement of graft. The supraportal right bile duct (BD) variant including presentation as trifurcation is a potential trap for injuring the remnant bile duct of donor. METHODS Before living/related-donor liver transplantation (LRLT), cholangiogram with magnetic resonance images of each donor was performed as a routine. After exploration of the donor before hilar dissection, intraoperative chloangiography (IOC) was routinely performed. Among the supraportal right bile duct variants, if the preoperative cholangiography showed a suspected trifurcation of the bile duct, we then performed 3 sessions of IOC during liver graft procurement, including prior to hilar dissection, before the division of bile ducts and after the division. We reviewed the cholangiogram and the postoperative laboratory data of a consecutive series of 25 donors of LRLT. RESULTS There was no division injury of the remnant bile duct of all of the donors. CONCLUSIONS Repeated IOC is suggested as a routine for variants of supraportal right bile ducts especially trifurcation pattern in graft procurement to avoid the injury of donor remnant bile ducts.

Factors affecting the regeneration of liver graft after living related liver transplantation: a preliminary study.

a “ o r ADEQUATE liver graft regeneration and functional reserve are essential for good outcomes of living donor liver transplantation (LDLT). Many authors have studied graft function or predictors for survival. In contrast, the aim of our study was to evaluate the volume increase, because of the small-for size problem especially when the living donor is difficult to find a second alternative. After living donor liver transplantation, the graft usually regenerates; however, the speed of change varies. Prediction of the regeneration speed is a great challenge to surgeons especially when the graft volume is limited or the recipient is critically ill preoperatively. We performed this prospective study to investigate factors significantly affecting regeneration speed.

Case Report: Maximum-Intensity Projection and Minimum-Intensity Projection of Computed Tomography Are Helpful in the Diagnosis of Budd-Chiari Syndrome in Complicated Liver Cirrhosis

BACKGROUND Early detection of Budd-Chiari syndrome (BCS) to give the appropriate therapy in time is crucial. Angiography remains the golden standard to diagnose BCS. However, to establish the diagnosis of BCS in complicated cirrhotic patients remains a challenge. We used maximum intensity projection (Max IP) and minimum intensity projection (Min IP) from computed tomographic (CT) images to detect this syndrome in such a patient. CASE REPORT A 55-year-old man with a history of chronic hepatitis B infection and alcoholism had undergone previously a left lateral segmentectomy for hepatic epitheloid angiomyolipoma (4.6 × 3.5 × 3.3 cm) with a concomitant splenectomy. Liver decompensation with intractable ascites and jaundice occurred 4 months later. The reformed images of the venous phase of enhanced CT images with Max IP and Min IP showed middle hepatic vein thrombosis. He then underwent a living-related donor liver transplantation with a right liver graft from his daughter. Intraoperatively, we noted thrombosis of his middle hepatic vein protruding into inferior vena cava. The postoperative course was unevenful. Microscopic findings revealed micronodular cirrhosis with mixed inflammation in the portal areas. Some liver lobules exhibited congestion and sinusoidal dilation compatible with venous occlusion clinically. CONCLUSIONS We recommend Max IP and Min IP of CT images as simple and effective techniques to establish the diagnosis of BCS, especially in complicated cirrhotic patients, thereby avoiding invasive interventional procedures.

Genetic diagnosis of neurofibromatosis type 1: targeted next- generation sequencing with Multiple Ligation-Dependent Probe Amplification analysis

BackgroundNeurofibromatosis type 1 (NF1) is a dominantly inherited tumor predisposition syndrome that targets the peripheral nervous system. It is caused by mutations of the NF1 gene which serve as a negative regulator of the cellular Ras/MAPK (mitogen-activated protein kinases) signaling pathway. Owing to the complexity in some parts of clinical diagnoses and the need for better understanding of its molecular relationships, a genetic characterization of this disorder will be helpful in the clinical setting.MethodsIn this study, we present a customized targeted gene panel of NF1/KRAS/BRAF/p53 and SPRED1 genes combined with Multiple Ligation-Dependent Probe Amplification analysis for the NF1 mutation screening in a cohort of patients clinically suspected as NF1.ResultsIn this study, we identified 73 NF1 mutations and two BRAF novel variants from 100 NF1 patients who were suspected as having NF1. These genetic alterations are heterogeneous and distribute in a complicated way without clustering in either cysteine–serine-rich domain or within the GAP-related domain. We also detected fifteen multi-exon deletions within the NF1 gene by MLPA Analysis.ConclusionsOur results suggested that a genetic screening using a NGS panel with high coverage of Ras–signaling components combined with Multiple Ligation-Dependent Probe Amplification analysis will enable differential diagnosis of patients with overlapping clinical features.