Good outcomes of living donor liver transplant in primary sclerosing cholangitis: an experience from North India.

BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. In the absence of effective medical therapy, liver transplant is the definitive treatment for advanced stage. However, recurrence of PSC after liver transplant is of concern which can lead to graft failure and may require retransplant. There are limited data on outcomes of living donor liver transplant (LDLT) in PSC. Also, in LDLT as donors are genetically related there can be an increased risk of recurrence. We conducted this retrospective study to analyze the outcomes of LDLT in PSC at a tertiary liver transplant center in north India. METHODS: We conducted a retrospective analysis of 3213 transplant recipients who underwent LDLT from January 2006 to May 2021. Of these 26 (0.80%) patients had PSC as indication for liver transplantation (PSC = 24, PSC-AIH overlap = 2). Data analysis was done to look for baseline demographics, clinical details, transplant outcomes, PSC recurrence, and survival. RESULTS: Mean age of study group was 42 (± 13.8) years and 19 patients (73.1%) were males. All patients had decompensated cirrhosis at the time of transplant. Mean CTP score and MELD score were 9.5 (± 1.8) and 18.9 (± 7.1), respectively. Sixteen patients received modified right lobe graft, seven extended right lobe graft and five patients received left lateral graft. Median graft weight and mean graft to recipient weight ratio (GRWR) were 633.5 (IQR 473.5-633.5) grams and 1.23 (± 0.42), respectively. Most common biliary anastomosis was hepaticojejunostomy, done in 19 (73.1%) while duct to duct anastomosis was performed in 7 (26.9%) patients. Median follow-up was 96 (36-123) months. One patient had ulcerative colitis and none had cholangiocarcinoma. Two (7.7%) patients had bile leak during early post-transplant period. Three (11.1%) patients developed graft rejection and were managed successfully with steroid pulses. Three patients died during early post-transplant period while seven deaths occurred during long-term follow-up including one death due to COVID-19. Five (21.73%) patients had recurrence of PSC of which two patients had graft loss including one after retransplantation. The one year graft and patient survival rate was 88.5%. CONCLUSION: LDLT can be performed in PSC with good long-term outcomes with a risk of PSC recurrence in about one-fifth patients.

A randomised controlled trial of Lichtenstein repair with Desarda repair in the management of inguinal hernias.

BACKGROUND: Ever since the advent of mesh hernioplasty with low recurrence rates, surgeons have turned a blind eye towards its devastating mesh related complications. Consequently, the quest for the best hernia surgery, that is as effective as the mesh repair but lacks its complications, continues. OBJECTIVES: The present study was carried out to compare the results of the Lichtenstein repair with the Desarda repair in the treatment of inguinal hernias. METHODS: A total of 77 patients with 87 hernias were randomly allocated into two groups to undergo either the Desarda repair (Group I, 39 patients with 45 hernias) or the Lichtenstein repair (Group II, Control, 38 patients with 42 hernias). 3 patients didn't complete the follow-up and were excluded from analysis. Finally, 40 hernias were analyzed in the Lichtenstein group and 44 in the Desarda group. RESULTS: After a 6-month follow-up period it was found that neither of the two groups had any recurrence. The incidence of chronic inguinodynia was much higher in the Lichtenstein group as compared to Desarda group. The pain scores, mean operating time, mean time to return to work and analgesic requirement was much lower with the Desarda repair as compared to Lichtenstein repair. CONCLUSION: Desarda repair was found to be as effective as the Lichtenstein repair in terms of recurrence and better in terms of chronic inguinodynia, complications and post operative pain scores. Desarda repair requires a significantly shorter operating time. The economic burden of this repair is much less compared to mesh repair.

Pre-sternal thyroid swellings: a case of rare aberrant site recurrence and review of literature.

BACKGROUND: Thyroid swellings enlarge caudally into the mediastinum behind the sternum. Pre-sternal swelling of thyroid origin is very rare. We present our case of pre-sternal thyroid swelling which was albeit a surprisingly rare site of papillary thyroid carcinoma recurrence and review of pre-sternal thyroid swellings reported till date. CASE SUMMARY: A 60 year old female presented with a painless, progressive swelling on the anterior part of the chest for the past 2 years. A 15 cm × 8 cm vertically aligned, non tender, well defined swelling was present on the pre-sternal region, with consistency ranging from soft to firm. The swelling was fixed to the underlying tissues and a fixed level IV lymph node was palpable on the right side. Ultrasonography revealed a large mass of 15 × 7 cm with multiple cystic areas. Fine needle aspiration cytology was inconclusive twice. Patient had undergone a total thyroidectomy for papillary carcinoma 10 years back. Computed tomography findings revealed a large 15 × 6.6 × 7 cm lobulated, pre-sternal, soft tissue lesion with solid & cystic components. The mass was infiltrating the right sided strap muscles and sternocleidomastoid. FNAC was inconclusive and thyroid scan could not pick up any activity in the mass. Henceforth a PET scan was done that showed increased FDG uptake by the lesion and the level IV lymph node. The patient underwent wide excision of the mass with right functional neck dissection, along with removal with both sternal head of sternocleido-mastoid, the strap muscles and the surrounding fascia. Histopathology confirmed papillary thyroid carcinoma. Patient received post-operative radioactive iodine ablation and is healthy with no recurrence up to 30 months of follow up. DISCUSSION: The mechanisms for pre-sternal thyroid swelling are not understood due to paucity of cases. The mechanisms proposed are invasion of strap muscles and cervical linea alba and tumor cells spread anterior to sternum, truly ectopic thyroid tissue, de novo carcinogenesis in the embryonal remnants like the thyro-thymic residues, sequestered thyroid tissue which grows later or migration of thyroid cells, incomplete clearance at the time of primary surgery or intraoperative seeding. CONCLUSION: Pre-sternal region masses of thyroid origin are very rare. A proper work up, suspicion for thyroid mass and array of tests will be required to come to a provisional diagnosis. Since the masses reported in literature were primarily malignant, any such mass may be treated on lines of malignancy with radical surgery.

Malrotation of the Gut in Adults: An Often Forgotten Entity.

Malrotation of the gut is a common paediatric condition that usually presents in the first month of life. However, presentation in adults is rare, and as a diagnostic dilemma quite often surprises the surgeon intraoperatively. If this condition is not timely recognized, it may result in disastrous consequences, such as gangrene of the small gut. We present the case of a 21-year-old male who presented to the emergency room with recurrent episodes of colicky abdominal pain and bilious vomiting. Contrast-enhanced computerized tomography (CT) revealed malrotation of the gut. The patient was planned for Ladd's procedure. Malrotation in adults may present in an acute way due to midgut volvulus or may have a chronic indolent course with recurrent vomiting and abdominal pain. In patients with acute obstruction, this differential should be kept in mind, especially if the patient has no previous abdominal surgery or evidence of tuberculosis. Contrast-enhanced CT is the investigation of choice and reveals typical findings, like whirlpool sign, corkscrew sign, or reversed relation of superior mesenteric artery and vein. The treatment is surgical as failure to do so may result in intestinal gangrene. The procedure of choice is Ladd's procedure. Every patient, even if asymptomatic, warrants this procedure as it is impossible to predict the timing of catastrophic complications.