Spiridon Vernadakis

Secondary hyperoxaluria: a risk factor for kidney stone formation and renal failure in native kidneys and renal grafts

Secondary hyperoxaluria is a multifactorial disease affecting several organs and tissues, among which stand native and transplanted kidneys. Nephrocalcinosis and nephrolithiasis may lead to renal insufficiency. Patients suffering from secondary hyperoxaluria, should be promptly identified and appropriately treated, so that less renal damage occurs. The aim of this review is to underline the causes of hyperoxaluria and the related pathophysiologic mechanisms, which are involved, along with the description of seven cases of irreversible renal graft injury due to secondary hyperoxaluria.

Liver transplantation in patients with liver metastases from neuroendocrine tumors: A systematic review

Background: Liver transplantation to treat neuroendocrine tumors, especially in the setting of diffuse liver involvement not amenable to operative resection remains controversial. We sought to perform a systematic review of the current literature to summarize data on patients undergoing liver transplantation with neuroendocrine tumors liver metastases as the indication. Methods: A systematic review was conducted in accordance to the Preferred Reporting Items for Systematic reviews and Meta‐Analysis guidelines. Eligible studies were identified using 3 distinct databases through March 2017: Medline (PubMed), ClinicalTrials.gov, and Cochrane library, Cochrane Central Register of Controlled Trials using a search algorithm: “(neuroendocrine or NET) and transplantation and liver.” Results: From the 1,216 records retrieved, 64 studies were eligible. Overall, 4 studies presented data from registries, namely the European Liver Transplant Registry and the United Network for Organ Transplantation/Organ Procurement and Transplantation Network databases, 3 were multicenter studies. The largest cohort of data on patients undergoing liver transplantation for neuroendocrine tumors liver metastasis indication were from single center studies comprising a total of 279 patients. Pancreas was the primary tumor site for most patients followed by the ileum. Several studies reported that more than half of patients presented with synchronous disease (55.9% and 57.7%); in contrast, metachronous neuroendocrine tumors liver metastasis ranged from 17.7% to 38.7%. Overall, recurrence after liver transplantation ranged from 31.3% to 56.8%. Reported 1‐, 3‐, and 5‐year overall survival was 89%, 69%, and 63%, respectively. Several prognostic factors associated with worse long‐term survival including transplantation >50% liver tumor involvement, high Ki67, as well as a pancreatic neuroendocrine tumors versus gastrointestinal neuroendocrine tumors tumor location. Conclusion: Liver transplantation may provide a survival benefit among patients with diffuse neuroendocrine tumors metastases to the liver. However, due to high recurrence rates, strict selection of patients is critical. Due to the scarcity of available grafts and the lack of level 1 evidence, the recommendations to endorse liver transplantation for extensive liver neuroendocrine tumors metastases warrants ongoing deliberations.

Liver transplantation for unresectable colorectal liver metastases: A systematic review

The use of liver transplantation (LT) for liver metastases attempted in the early 1990s was associated with poor perioperative outcomes and unacceptably low overall survival. Recently, there has been renewed interest in LT as a treatment option for colorectal liver metastases (CLM) in countries where organ supply is high. To date, no meticulous analysis about the efficacy, safety and outcomes of LT in CLM patients has been published. We present the first systematic review on the subject.

The Role of Intraoperative Nerve Monitoring (IONM) in Thyroidectomy: Where Do We Stand Today?

Objective. Intraoperative nerve monitoring (IONM) gains daily ground, being used during thyroidectomy to help the surgeon identify the recurrent laryngeal nerve (RLN). But its value still remains debatable among endocrine surgeons. In this review article, we aim to present current knowledge on the subject. Data sources. The MEDLINE/PubMed database was searched for publications with the medical subject heading “Intraoperative Nerve Monitoring (IONM)” and keywords “thyroidectomy,” “recurrent laryngeal nerve (RLN)” or “vocal cord paresis or vocal cord paralysis (VCP).” The search was conducted both on basis of the MESH tree and as a text search. Study selection/Data extraction. We restricted our search to English till July 2012. In this review, we only included 34 articles and abstracts that were available in English. Conclusions. RLN IONM adds a functional dimension to surgery that aids, but does not replace, visual identification. For the time being, intraoperative visual identification of the nerve and preoperative and postoperative laryngoscopic assessment of vocal cord function remain the gold standard of RLN management in thyroid surgery. Whether IONM will become a standard of care or not depends on the familiarization of each surgeon with this technique as well as on the publication of studies involving large series of thyroidectomies, where the evaluation of IONM will have significant statistical power.

Hoarseness after thyroidectomy: blame the endocrine surgeon alone?

OBJECTIVEHoarseness is a postoperative complication of thyroidectomy, mostly due to damage to the recurrent laryngeal nerve (RLN). Hoarseness may also be brought about via vocal cord dysfunction (VCD) due to injury of the vocal cords from manipulations during anesthesia, as well as from psychogenic disorders and respiratory and upper-GI related infections. We reviewed the literature aiming to explore these potential surgical and non-surgical causes of hoarseness beyond thyroidectomy and the role of the endocrine surgeon. Is he/she alone to blame?METHODs/MATERIALThe MEDLINE/PubMed database was searched for publications with the medical subject heading “hoarseness” and keywords “thyroidectomy”, “RLN”, “VCD” or “intubation”. We restricted our search till up to May 2013.RESULTSIn our final review we included 80 articles and abstracts that were accessible and available in English. We demonstrated the incidence of hoarseness stemming from surgical and non-surgical causes and also highlighted the role of intubation as a potential cause of injury-related VCD.CONCLUSIONSHoarseness is a relatively common complication of thyroidectomy, which can be attributed to many factors including surgeon’s error or injuries during intubation as well as to other non-surgical causes. However, compared to procedures such as cervical spine surgery, mediastinal surgery, esophagectomy and endarterectomy, thyroidectomy would seem to be a procedure with a relatively low rate of recurrent laryngeal nerve palsies (RLNPs). It is often difficult to determine whether the degree of hoarseness after thyroidectomy should be attributed only the surgical procedure itself or to other causes, for example intubation and extubation maneuvers. The differential diagnosis of postoperative hoarseness requires the use of specific tools, such as stroboscopy and intra- and extralaryngeal electromyography, while methods like acoustic voice analysis, with estimation of maximum phonation time and phonation frequency range, can distinguish between objective and subjective deterioration in the voice. The importance of medical history should be also emphasized.

A Question Seeking for an Answer: Use of T-tube in the Era of Liver Transplantation With Grafts From Extended Criteria Donors and Donors After Cardiac Death.

To the Editor: W e read with great interest the recent publication of López-Andújar et al1 presenting their results of a prospective and randomized clinical trial referring to the incidence and severity of biliary complications due to liver transplantation (LT) after choledochocholedochostomy for the biliary reconstruction with or without a temporary T-tube. Biliary complications including leaks; strictures; and ischemic-type biliary lesions, stones, and sphincter of Oddi dysfunction still remain the Achilles’ heel of the procedure because they contribute to its morbidity and mortality. There is still controversy about the usefulness of T-tube for end-to-end biliary anastomosis to reduce the incidence of biliary complications. More specifically, there is ongoing debate regarding the use of a T-tube for biliary reconstruction in LT. There is emerging literature that suggests the application of T-tube because it provides easy access to the biliary tree, lowers the pressure in the biliary system, aids in monitoring the quality and output of bile, and might reduce the incidence of anastomotic stricture2 without the increased rate of complications compared with the non–T-tube approach.3 Moreover, a previous meta-analysis conducted by our group on this subject4 evaluated the outcomes after duct-to-duct biliary reconstruction with or without T-tube in LT. We demonstrated nonsuperiority of performing duct-to-duct anastomoses with T-tube as far as anastomotic bile leaks or fistulas, need for postoperative choledochojejunostomies, and need for dilatation and stenting of anastomotic strictures is concerned. The evidence gathered in our study discourages the routine use of T-tubes in biliary reconstruction in LT.2

Renal paratransplant hernia. An uncommon variant of internal hernia. Are we aware of it

of Internal Hernia. Are We Aware of It? P aratransplant hernia (PH) consists of an unusual variant of internal hernia, which was first reported by Kyriakides et al. (1) as entrapment of bowel through a defect in the peritoneum covering the transplanted kidney. It is an uncommon but potentially fatal complication of renal transplantation. To our knowledge, nine cases have been previously reported (2, 3). We presented the very first case of Richter hernia as a variant of renal PH. A 53-year-old man presented to our department with 48-hr symptoms of abdominal pain, distension, and nausea. Three months ago, he underwent kidney transplantation from a deceased donor because of a 6-year history of uremia. His body temperature was normal and no signs of peritonitis were present. Plain abdominal x-ray demonstrated widespread small bowel obstruction (SBO) and multiple air-fluid levels. A nasogastric tube was inserted and an enema administered to release the distension. However, over the next 2 days, the patient’s condition did not improve. Abdominal computed tomography (CT) scan revealed that most small bowel loops were dilated and filled with fluid, without being able to identify cause of the obstruction. An exploratory laparotomy was performed. A necrotic segment of partially obstructed small bowel approximately 8 cm long was found herniated within a peritoneal defect at the site of allograft implantation (Fig. 1). The so-called Richter hernia was diagnosed. Viable bowel was anastomosed by side-to-side fashion. The peritoneal defect was closed with nonabsorbable sutures. The patient had a sound postoperative course and was discharged at the eighth postoperative day.

Liver transplantation for liver malignancies in Wilson's disease: two novel cases.

Two Novel Cases Wilson disease (WD) is a rare autosomal recessive disorder of copper (Cu) metabolism that presents with hepatic, neurologic, or psychiatric disturbances. It typically begins with a presymptomatic period, during which Cu accumulation in the liver causes subclinical hepatitis and progresses to liver cirrhosis and development of neuropsychiatric symptoms. Liver disease consists of jaundice, autoimmune-type hepatitis, fulminanthepatic failure, or chronic liver disease leading to cirrhosis (1). Cirrhosis is commonly the basis of hepatocellular carcinoma (HCC) development, which has rarely been previously reported because of very few available series with long-term follow-up but perhaps should suggest increased vigilance (2). On the other hand, cholangiocarcinoma (CCC) appears to be extremely rare in this disease, with only five cases reported in the literature (2). Liver transplantation (LT) provides cure for WD in patients where medical therapy has failed or in those with advanced decompensated liver disease at initial evaluation (3). Its association with liver malignancies (LM) such as HCC and CCC has been scarcely documented. We herein report on two cases of coexistence of WD with LM, which have been successfully transplanted. The first patient is a 49-year-old woman with a 23-year history of WD and Child-Turcotte-Pugh B cirrhosis. During follow-up imaging, contrastenhanced computed tomography (CT) revealed four tumor lesions with a maximal diameter of 4.5 cm. Alphafetoprotein (AFP), carcinoembryonic antigen (CEA), and carbohydrate antigen (CA) 19-9 were within normal range. Radiologic workup was accomplished using abdominal magnetic resonance imaging, thoracic CT, and bone scintigraphy according to our protocol (4), which has ruled out extrahepatic manifestations. Computed tomographyYguided tumor biopsy set the diagnosis of multifocal HCC. The patient was listed for LT and underwent two sessions of transarterial-chemoembolization (TACE) as bridging therapy. She was transplanted 8 months later with a model for end-stage liver disease (MELD) score of 9. Histology confirmed the preoperative diagnosis of four HCC lesions with microvascular invasion (Fig. 1A). Two years after LT, she manifested a solitary lung metastasis, which was successfully resected. The second patient is a 48-yearold man with an 18-year history of WD and Child-Turcotte-Pugh B cirrhosis. During follow-up imaging, CT scan revealed two malignant focuses in segment VIII. To establish a diagnosis in that nodular cirrhotic liver, a CT-guided biopsy was performed. The presence of malignancy was confirmed without identifying its origin. AFP, CEA, and CA 19-9 were normal in repetitive measurements. Radiologic workup was accomplished by abdominalmagnetic resonance imaging, thoracic CT, and bone scintigraphy, which have ruled out extrahepatic manifestations. As the lesions were highly suggestive of HCC within the Milan criteria, he was listed for LT and underwent a session of TACE as bridging therapy. Eleven months after TACE, he was successfully transplanted with MELD score of 27. Final histology revealed, in contrast to the preoperative assumption, a bifocal CCC without vascular invasion (Fig. 1B). Follow-up studies revealed no evidence of tumor recurrence. Posttransplantation treatment included corticosteroids; tacrolimus, which was switched to m-TOR inhibitor (sirolimus) after 3 months; and mycophenolate mofetil in both patients according to the protocol of our department. Post-LT follow-up encompassed abdominal and thoracic CTevery 3months during the first year, every 6 months during the second year, and yearly thereafter. Both patients remain in good general condition 32 and 24 months after LT, respectively, and receive no chelating agent. An increased risk of LM is recognized in some forms of metabolic liver disease. Interestingly, although tumors are rare in WD (3), HCCs and CCCs have been described in several case reports as well as in recent retrospective studies of WD patients, suggesting higher frequency of these malignancies in WD patients than in the control population (2). In contrast to our patient, in most of the reported cases, the LM was incidentally diagnosed in the pathologic specimen after death for WD-induced liver failure (2). Various attempts have been made to ascertain which patients with WD should be considered for LT (3). Patients with WD should enroll close follow-up with imaging examinations, as AFP measurement alone is not reliable for exclusion of LM. Fulminant liver failure and disease refractory to medical treatments consists the current indications for LT in WD. The risk of LM is not to ignore and could consist additional indication for LT. These patients should be eventually listed earlier for LT, irrelevant to liver function status. According to our restricted experience with transplanted LM in WD, WD patients may benefit from earlier listing/LT, and LT may provide acceptable results even in the case of CCC.

Jejunal diverticulosis as the obscure cause of acute abdominal pain.

Fig 1. Multiple diverticula mainly of the proximal jejunum. A 74-YEAR-OLD MAN was admitted with a 6-hour history of severe diffuse abdominal pain of acute onset, accompanied by nausea and flatulence. On examination, he was agitated with signs of diffuse peritonitis. The laboratory analysis revealed leukocytosis (19,100 leukocytes/mm) and mild hemodynamic instability (blood pressure, 87/64 mmHg; heart rate, 109 bpm). After initial fluid recovery, the patient underwent emergency laparotomy. The patient had no previous surgical history. The exploratory laparotomy revealed the presence of multiple diverticula mainly of the proximal jejunum (Fig 1), one of which was ruptured and caused the peritonitis (Fig 2). The patient was treated with segmental resection of the jejunum carrying the ruptured diverticulum. The intestinal segment containing the perforated diverticulum was removed and an end-to-end anastomosis was done to reconstruct the intestinal transit. Histopathology report confirmed an ulcerative jejunal diverticulitis with imminent perforation and acute local peritonitis. The patient recovered well and was discharged from hospital on postoperative day 9. There were no signs of abdominal pain 6 months after the operative procedure.

Music and Surgery: A Literary Reappraisal

HISTORY The involvement of music in medicine dates back to the 6th century, when Greek philosopher Pythagoras described music as contributing to the natural harmony of the mind and body. In 1914, intraoperative music was provided to ameliorate the anxiety and fear of patients before a surgical operation. Many personalities are known to be both musicians and physicians, with Auenbrugger and Laennec providing brilliant examples. Theodor Billroth, who laid the foundation for modern abdominal surgery with his pioneering operations, was also an excellent musician.