Simultaneous Liver and Kidney Transplantation in a Patient With Telomere Biology Disorder: A Case Study.

Organ transplantation is the primary therapy for organ failure caused by telomere biology disorder (TBD). We describe the first documented case of simultaneous liver and kidney transplantation (SLKTx) for TBD, although the diagnosis of TBD was reached only three months following SLKTx. The patient was born prematurely, displayed growth retardation, and developed chronic kidney and liver diseases. His pre-SLKTx autoimmune, metabolic, and viral assessments were negative, and persistent pancytopenia (bone marrow cellularity 70-80%) was attributed to renal disease-associated bone marrow changes. Following SLKTx, he was discharged with stable graft function on tacrolimus and prednisolone. Although mycophenolate mofetil was discontinued on the second postoperative day, his pancytopenia persisted. Despite extensive evaluations, including drug, immune, nutritional, and viral assessments, all results were negative. A bone marrow biopsy conducted three months post-transplant revealed significant hypocellularity (40-50%). Whole genome sequencing revealed a likely pathogenic variant of the TINF2 gene. The patient was subsequently treated with danazol. At the nine-month follow-up post-SLKTx, he exhibited stable graft function and improved cell counts while maintaining triple-drug immunosuppression. Given the lack of uniform diagnostic criteria for TBD, healthcare providers must be vigilant with patients presenting with multi-organ failure and persistent cytopenias. Effective pre-transplant screening for TBD can lead to timely diagnoses, better management, and improved post-transplant outcomes.

Impact of an antimicrobial stewardship and monitoring of infection control bundle in a surgical intensive care unit of a tertiary-care hospital in India.

OBJECTIVES: Antimicrobial stewardship (AMS) in resource-limited settings lacks models that can be readily adapted to their settings. Here we discuss the impact of a combined strategy of AMS and monitoring of infection control practices in a tertiary-care centre of a developing country. METHODS: This study was undertaken in the surgical unit of a tertiary-care hospital over an 8-month period. In the first 2 months (baseline phase), prospective audit and feedback alone was undertaken, while in the next 6 months (intervention phase) this was supplemented with strategies such as antimicrobial timeout, correction of doses and bundle approach for prevention of hospital-acquired infections. RESULTS: A total of 337 patients were included (94 in the baseline phase and 243 in the intervention phase). There was a decrease in days of therapy per 1000 patient-days (1000PD) (1112.3 days vs. 1048.6 days), length of therapy per 1000PD (956 days vs. 936.3 days) and defined daily doses (DDD) per 1000PD for most antimicrobials. A decrease in double cover for Gram-negative infections (9.6% vs. 2.9%) but an increase in double anaerobic cover (4.2% vs. 7.4%) was observed. There was a decrease in the incidence of ventilator-associated pneumonia per 1000 ventilator-days in the intervention phase (46.4 vs. 35.4), whereas central line-associated bloodstream infections per 1000 central line-days remained the same (14.7 vs. 14.8). CONCLUSION: This study shows that implementation of routine AMS activities with monitoring of infection control practices can help decrease overall antimicrobial use. With furtherance of measures to control infection, antimicrobial use may be further curtailed.

Outcomes of Endoscopic Dilation in Patients with Esophageal Anastomotic Strictures: Comparison Between Different Etiologies.

BACKGROUND AND AIMS: This retrospective study was aimed at assessing the efficacy of endoscopic dilation for esophageal anastomotic strictures, and to compare response between caustic anastomotic strictures (CAS) and non-caustic anastomotic strictures (NCAS). MATERIALS AND METHODS: Patients with anastomotic strictures (enrolled during January 1996-December 2015) were analyzed. Short- and long-term outcomes of dilation, in terms of clinical success, refractory, and recurrent strictures were compared between NCAS and CAS. Patients with refractory and recurrent strictures were managed with adjunctive therapy including intralesional steroids. Factors predicting refractoriness at start of dilation and reasons for more than ten lifetime dilations were also evaluated. RESULTS: Of the 142 patients, 124 (mean age-44.02; males-74) underwent dilation. Clinical success was achieved in 113 (91.3%) patients requiring a median [Interquartile range (IQR)] of 4 (2-10) sessions. The number of dilations to achieve clinical success, refractory strictures, and recurrent strictures, and the use of adjunctive therapy were significantly higher for CAS than for NCAS. Intralesional steroid use decreased periodic dilation index (PDI) significantly in CAS. Caustic etiology and starting dilation diameter of < 10 mm were found to be predictors of refractoriness, with the former alone being an independent predictor of more than ten lifetime dilations. No patient had free perforation; however, five required revision surgery. CONCLUSION: Patients with CAS fared worse than those with NCAS in terms of number of dilations, refractoriness, recurrence of strictures, and need of adjunctive therapy. Endoscopic dilation can successfully ameliorate dysphagia due to anastomotic strictures in a majority of patients.

Rare anatomic variations of the right hepatic biliary system.

PURPOSE: To report rare and clinically significant anatomic variations in the biliary drainage of right hepatic lobe. METHODS: Unique variations in the extra- and intrahepatic biliary drainage of right hepatic lobe were observed in 6 cadaveric livers during dissection on 100 formalin-fixed en bloc cadaveric livers. RESULTS: There was presence of aberrant drainage of right segmental and sectorial ducts in four cases and of accessory right posterior sectorial duct in two cases. CONCLUSIONS: We encountered some extensively complicated biliary drainage of right hepatic lobe, unsuccessful recognition of which can lead to serious biliary complications during hepatobiliary surgeries and biliary interventions.

The segmental branching of the hepatic arteries in the liver: a cadaveric study.

The purpose of this work was to evaluate the sectorial and segmental arterial branching (second- and third-order branching) in the right and left hemilivers, as knowledge of this branching should lead to improved vascular mapping for various endovascular procedures in the liver. The study was conducted on 100 formalin-fixed adult cadaveric livers. The arterial anatomy of the liver was dissected from the origin of the hepatic arteries to their segmental branches. Conventional segmental branching of both the right and the left hepatic arteries was seen in only 25% of the livers. In the remaining livers, the segmental branching of one or both of the hepatic arteries was different from that given in anatomy texts. The branching of the right and left hepatic arteries was anatomically classified into seven and six patterns, respectively, mainly on the basis of their sectorial and segmental anatomy. The present study details the highly diverse segmental arterial anatomy of the liver. The information provided here has important implications for procedures that involve selective catheterization of segmental arteries.

Closed loop of main duct of pancreas: a rare variant configuration.

During routine dissection, duplication of main pancreatic duct extending from body to head region of pancreas was observed in a 78-year-old formalin fixed male cadaver. Main pancreatic duct gave a prominent branch which joined back the parent duct in head resulting in the formation of a closed loop. This gave the appearance of focal duplication in the form of a closed loop, an unusual variant. This was an incidental finding. Such cases usually remain asymptomatic; however, if undetected may be the cause of postoperative pancreatic fistula following pancreaticobiliary surgery. Knowledge of variable anatomy of pancreatic duct system became important to reduce the risk of postoperative complication and during various endoscopic guided procedures like drainage of pseudocyst or placement of stent into the duct.

Transitional cell carcinoma of ureter simulating left colonic malignancy-a rare case.

We reported a case of ureteric tumor, an uncommon disease presenting with colonic obstruction. This 52-year-old lady presented with history of colicky pain left lower abdomen for 5 months. Colonoscopy revealed circumferential nonnegotiable stricture at 25 cm from anal verge, and colonoscopic biopsy was inconclusive. Contrast-enhanced computed tomography (CECT) showed a growth involving sigmoid colon and left ureter with proximal left hydroureteronephrosis with nonfunctioning left kidney. Guided fine needle aspiration cytology (FNAC) showed features of an adenocarcinoma. The patient was operated with a diagnosis of carcinoma colon and underwent low anterior resection with left nephroureterectomy. Histopathology of resected specimen revealed ureteric transitional cell carcinoma with colon infiltration. She had smooth postoperative recovery.

Zygomycetes peritonitis in ambulatory peritoneal dialysis: Case report and review of literature.

We present a case of CAPD peritonitis caused by zygomycetes infection. A 46-year-old male patient presented with refractory peritonitis requiring catheter removal. He had persistence of fever and an ultrasonography abdomen done revealed loculated collections. An initial pigtail drainage followed by open laparotomy was performed. Intra-operative peritoneal and omental biopsy revealed large areas of necrosis with broad aseptate fungal hyphae consistent with zygomycosis. He was managed with 3 gm of intravenous amphotericin and is doing well at 6 months of follow-up.

Pseudobowel perforation following endoscopic retrograde cholangiopancreatography.

We report two cases of pneumoperitoneum following endoscopic retrograde cholangiopancreatography for retained common bile duct (CBD) stones. These post-cholecystectomy patients underwent sphincterotomy, CBD clearance, and "T" tube removal at the same time. Post-procedure, both of the patients developed pneumoperitoneum. Pneumoperitoneum developed as a result of air traversing from the duodenum to the peritoneum through the ruptured "T" tube tract. "T" tube removal in the same sitting as sphincterotomy and CBD clearance may lead to pneumoperitoneum, which can be managed conservatively.

Large pelvic masses of obscure origin: urologist's perspective.

BACKGROUND: Large pelvic masses pose unique diagnostic and therapeutic challenges due to varied aetiology, paucity of characteristic imaging features, lack of therapeutic algorithms and surgical difficulties in resection inside the narrow confines of the pelvis with close proximity of vital structures. METHODS: Records of 22 patients with large pelvic masses in the last six years were analysed. Their demographic and clinical features were noted, along with imaging features, preoperative biopsy, surgical procedure, intraoperative difficulties, complications, adjuvant therapy and outcome. RESULTS: There were 14 men and 8 women with a median age of 45 years. Presenting symptoms were abdominal mass, pain, lower urinary tract symptoms, urinary retention and constipation. Imaging was mostly unable to determine the organ of origin of the tumour. Histopathology revealed pelvic fibromatosis (2), chondrosarcoma (1), liposarcoma (1), haemangioendothelioma (1), lymphangioma (2), fibroleiomyoma (1), leiomyosarcoma (3), schwannoma (4), malignant nerve sheath tumour (1), rectal gastrointestinal stromal tumour (1), retrovesical hydatid cysts (3), sacral chordoma (1) and Ewing's sarcoma (1). In 5 patients complete excision was not possible because of extension into vital structures. Fifteen patients were alive at 1-5 years of follow-up. CONCLUSIONS: Urologists, being the 'gatekeepers of the pelvis', are usually involved in the management of large pelvic masses. Good outcome can be achieved with careful surgical planning.

Comparison of observational and controlled clinical trials of diltiazem in the treatment of chronic anal fissure.

Diltiazem has been extensively studied in the treatment of chronic anal fissures, but efficacy in clinical practice is not fully established. The aim of the present study was to evaluate the safety and efficacy of topical application diltiazem in observational studies as well as in controlled clinical trials in the treatment of chronic anal fissures. A systematic literature search was carried out from 1966 to 31 December, 2007 on PubMed, Medline, Embase and Cochrane database, using the appropriate search words. We found six observational studies with 392 patients and five controlled clinical trials with 289 patients in which topical diltiazem treatment was given. Efficacy was found to be very high in observational studies (56.88%), whereas it was found to be modest in controlled clinical trials (29.41%). In observational studies, most of the patients reported complete healing of fissures within 6-12 weeks, whereas in controlled trials healing was reported within 8 weeks, with tolerable adverse effects of diltiazem. On the basis of the above studies, it can be concluded that topical application of diltiazem is useful in the treatment of chronic anal fissure, but to fully establish its efficacy, larger prospective double-blind study is required in the near future.

Solitary skeletal metastasis in carcinoma gallbladder: two case reports.

We report 2 cases of carcinoma gallbladder with only solitary skeletal metastasis. To the best of our knowledge there are only 3 case reports of carcinoma gallbladder with skeletal metastasis in the English literature.

Effect of pneumoperitoneum on intracranial pressure during supratentorial craniotomy: a case report.

Laparoscopic surgery has become the method of choice for many procedures previously performed by open techniques. However, its use in patients with a potentially decreased intracranial compliance warrants caution. We report a case of combined glioma excision and laparoscopic cholecystectomy procedures with evaluation of the effects of pneumoperitoneum on ICP and operative field. The clinical implications, safety and recommendations of conducting laparoscopic surgeries in neurosurgical patients are discussed. Performing pneumoperitoneum in patients with marginal intracranial compliance needs interdisciplinary discussions and ICP monitoring is mandatory.

Traumatic diaphragmatic hernia: tertiary centre experience.

BACKGROUND: Traumatic diaphragmatic hernia (TDH) resulting from traumatic diaphragmatic rupture (TDR) may not be easily detected and can lead to significant morbidity and mortality. PATIENTS AND METHODS: A retrospective case note analysis was performed of all patients treated for TDR at a major teaching hospital between March 2003 and March 2008. The aetiological factors, associated injuries, management and outcome were analysed. RESULTS: Twenty-seven patients were studied (24 males, 3 females) and their ages ranged from 16 to 72 years (median 35 years). TDR was left-sided in 85% and right-sided in 15%. Aetiology was blunt trauma in 81% and 19% had penetrating injury. Associated injuries were present in 81%. The most common approach for repair was transabdominal (89%); additional thoracotomy was needed in 11%. Herniation of abdominal contents was present in 85% and herniation of more than one organ was present in 57%. The diaphragmatic rent was repaired primarily in 89% using nonabsorbable sutures. Post-operative pulmonary complications occurred in 52% of patients. Three patients (11%) died. CONCLUSION: Left-sided blunt traumatic diaphragmatic rupture was more common than right-sided rupture. The most commonly herniated organs were the stomach and colon. Most ruptures could be repaired by an abdominal approach, which also allowed a complete exploration of the abdominal organs. Careful attention should be given to associated intra-abdominal injuries. Most of the defects were repaired directly using nonabsorbable sutures.

Coexistent hepatic and pulmonary epitheloid hemangioendothelioma.

Epitheloid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin known to arise in soft tissue, liver and lung. We describe a case of coexistent hepatic and pulmonary epitheloid hemangioendothelioma, proven on autopsy, and review the histological and radiological features of epitheloid hemangioendothelioma. The coexistence of hepatic with pulmonary EHE has been reported in only a few cases. Large confluent masses, peripheral location with capsular retraction, hypertrophy of uninvolved liver, invasion of portal and hepatic veins, enhancing margins and delayed enhancement and dense calcification are the typical features which provide a clue to diagnosis of hepatic EHE. In patients with both hepatic and pulmonary EHE it is difficult to say whether the tumor arose primarily in the lung or liver, or began simultaneously in both organs.

Morphology of liver.

BACKGROUND: Liver size is related to body surface area. The present study was conducted to assess liver size and to find its relationship with body parameters. Also, the external surface of liver was studied for presence of fissures, which may demarcate the vascular segments and the blood vessels beneath them and may help the surgeon for resection of liver segment. MATERIAL AND METHODS: Apparently normal liver specimens were obtained from 50 cadavers aged 19-65 years. Measurements of liver diameters were taken with the help of Vernier Calipers. Volume of liver was taken by water displacement method. Means and standard deviation of data were calculated and liver dimensions were co-related with body parameters. RESULTS: The mean maximum transverse diameter was 199.4 + 24.5 mm and maximum vertical diameter was 149.5 + 18.7 mm. Significant correlations were observed between Maximum anteroposterior diameter and body mass index (p < 0.05);Vertical diameter at falciform ligament and body weight (p < 0.01);Highly significant correlation was seen between liver volume and body surface area (P < 0.001). The fissures showed underlying veins draining into the inferior vena cava in 40% cases. CONCLUSIONS: A significant increase in the body parameters with a corresponding increase in the liver dimensions was found. Study of morphology of liver can help the surgeons to dissect in proper planes to make the resection safe. The surface of liver showed 1-3 fissures distributed on all lobes in 70% specimens demarcating the vascular segments, which may help the surgeon during resection of liver.