Andreas L. Lambrianides

Acquired urachal pathology: presentation of five cases and a review of the literature

Urachal anomalies are rare and are usually diagnosed in early childhood or at birth. Literature on the subject is largely confined to case reports, dating back to 1550. 1,2 They are of concern to general surgeons and urologists alike because of the variable clinical presentations. A basic knowledge of urachal development is necessary to suspect a diagnosis of urachal disease. 3 Definitive treatment consists of total surgical excision of the urachus, including a cuff of adjacent bladder. Over the past 3 years, five adults with acquired urachal disease have attended this hospital. The cases are presented below.

Open cholecystectomy: exposure and confidence of surgical trainees and new fellows

BACKGROUND The laparoscopic approach to cholecystectomy has overtaken open procedures in terms of frequency, despite open procedures playing an important role in certain clinical situations. This study explored exposure and confidence of Australasian surgical trainees and new fellows in performing an open versus laparoscopic cholecystectomy. MATERIALS AND METHODS An online survey was disseminated via the Royal Australasian College of Surgeons to senior general surgery trainees (years 3-5 of surgical training) and new fellows (fellowship within the previous 5 years). The survey included questions regarding level of experience and confidence in performing an open cholecystectomy and converting from a laparoscopic to an open approach. RESULTS A total of 135 participants responded; 58 (43%) were surgical trainees, 58 (43%) were fellows and 19 (14%) did not specify their level of training. Respondents who were involved in more than 20 open cholecystectomy procedures as an assistant or independent operator compared with those less exposed were more likely to feel confident to independently perform an elective open cholecystectomy (87.8% vs. 57.3%, P = 0.001), independently convert from a laparoscopic to open cholecystectomy (87.8% vs. 58.7%, P = 0.001) and independently perform an open cholecystectomy as a surgical consultant based on their level of exposure as a trainee (73.2% vs. 45.3%, P = 0.004). CONCLUSION This study suggests the need to ensure surgical trainees are exposed to sufficient open cholecystectomies to enable confidence and skill with performing these procedures when indicated. Greater recognition of the need for exposure during training, including meaningful simulation, may assist.

Nephrotic syndrome presenting as primary peritonitis in a male adolescent

A 16‐year‐old man with no relevant past medical history was admitted with sudden‐onset severe generalized abdominal pain. On examination, he was tachycardic, tachypnoeic and febrile (39.7°C), with generalized guarding and percussion tenderness. Blood tests revealed a leucocytosis (white cell count 17.0 × 10/L), and a low serum albumin (<15 g/L). An erect chest X‐ray was unremarkable. A contrast‐enhanced computed tomography scan of his abdomen and pelvis (Fig. 1) revealed free fluid in his pelvis and hepatorenal space, but no obvious underlying cause. The patient underwent emergency laparoscopy which showed purulent fluid in his pelvis and right upper quadrant, and an oedematous retroperitoneum. The appendix, stomach and gallbladder appeared normal. Because of the abnormal laparoscopic findings, the decision was taken to proceed to laparotomy, which revealed only oedema in the retroperitoneum and lymphadenopathy within the small bowel mesentery, but ultimately no underlying cause. He was treated empirically with intravenous piperacillin/tazobactam, however, group B Streptococcus (Streptococcus agalactiae) was subsequently cultured from peritoneal fluid obtained intraoperatively. A urine sample (obtained postoperatively) revealed a protein concentration of 28 000 mg/L and a protein creatinine ratio of 1800 g protein/mol creatinine (or 15.9 g protein/g creat). Preoperatively, the patient did not have obvious evidence of pitting oedema, however, sacral, ankle and facial oedema were present postoperatively, and a diagnosis of primary peritonitis secondary to nephrotic syndrome was made. Subsequent investigation with renal biopsy showed extensive effacement of glomerular epithelial foot processes (on electron microscopy, Fig. 2) consistent with a diagnosis of minimal change nephropathy. Histology from lymph nodes excised at laparotomy and from a bone marrow trephine showed no evidence of malignancy. Once the diagnosis of minimal change nephropathy was confirmed, he was commenced on prednisolone 60 mg daily for 4 weeks with a subsequent reducing dose. He also required daily frusemide for fluid retention and had bilateral pleural effusions and ascites drained. He made an excellent recovery and was discharged from hospital on day 12. Primary peritonitis is inflammation of the peritoneal cavity without a known precipitant, and is also known as spontaneous bacterial peritonitis in the setting of a documented bacterial infection. Most cases are associated with portal hypertension and ascites. Primary peritonitis is a well‐documented complication of nephrotic syndrome in children. In a series of 25 such cases published in 2005 the mean age was 7 years old, and in a series of eight cases published in 2002, the oldest child was 9 years old. As few as 16 cases have been reported of primary peritonitis (or spontaneous bacterial peritonitis) secondary to nephrotic syndrome in adults. The mechanism by which nephrotic syndrome predisposes to peritonitis is not well understood but is thought to relate to a number of factors. The presence of ascites leads to a natural culture medium for bacterial overgrowth. Protein loss via the glomerulus leads to the loss of immunoglobulins (especially IgG) and important factors in the complement pathway (especially factors B and D). The bodys ability to synthesize new specific antibodies is also impaired in nephrotic syndrome. Most cases are in patients with a pre‐existing diagnosis of nephrotic syndrome, who are often further immunosuppressed by the administration of corticosteroid. This may also be a contributory factor. Various studies have reported common organisms implicated in primary peritonitis associated with nephrotic syndrome, with Streptococcus pneumonia, alpha‐haemolytic Streptococcus and Escherichia coli being among the commonest. We could only find two published cases (from 1978 and 1979) showing group B Streptococcus as the causative organism. This case demonstrates a rare but important cause of peritonitis in adults. Although the patient is 16 years old and would be considered by many an adolescent rather than an adult, he is beyond the expected age range for this condition, and old enough that he would present to an adult surgical unit rather than a paediatric hospital. Despite the rarity of this condition in adults, it should be suspected and investigated for in the setting of unexplained low serum albumin and proteinuria. An early diagnosis is important to avoid the need for laparotomy, and institute conservative treatment with antibiotics. Appropriate broad spectrum antibiotics based on local microbiology advice is important given the variation in causative organisms seen. Appropriate attention must be paid to investigating the underlying cause for the patients nephrotic syndrome, and these patients should be referred for specialist management by a renal physician.

On‐table technique for removing faecalomas from the rectal stump before restoration of intestinal continuity

As the contents of the rectum fail to progress following a Hartmann’s procedure, liquid is lost and a faecaloma forms. The presence of a faecaloma in the rectal stump can cause unnecessary delay and precludes the formation of a secure stapled anastomosis. We wish to describe a technique for the removal of faecaloma found in the rectal stump following Hartmann’s procedure, which to our knowledge has not been previously reported. We believe that this is useful in the unexpected event of finding such a problem at surgery and provides a simple, safe, effective and cheap means of clearing the lumen. This technique has been effectively used in more than 100 consecutive patients (A. L. L.). A lubricated 24-Fr three-way irrigation urology (Bardex Foley Biocath (Covington, CA, USA) 24Ch/Fr 8.0mm catheter) with a 30-mL balloon (with the balloon deflated) is slowly introduced per anum beyond the faecaloma (Fig. 1a). The balloon is then inflated and gentle traction applied on the catheter (Fig. 1b) and the mass is gently pulled caudally out of the rectum (Fig. 1c). This process may be repeated as required. The degree of inflation of the balloon and progress of the manoeuvre is monitored by direct palpation of the stump by the intrapelvic hand of the surgeon. Following removal of the faecal mass, a washout is carried out and the anastomosis carried out using a Premium Plus CEEA (Autosuture; Coridien, Norwalk, CT, USA) single-use stapler.

Keratoacanthoma centrifugum marginatum.

Keratoacanthoma centrifugum marginatum (KCM) is a giant and a rarer variant of keratoacanthoma (KA), with only 30 documented cases. Our patient presented with a macroscopically roughened, somewhat depressed and pinkish-cream lesion, measuring 65mm · 60mm (Fig. 1). No regional lymphadenopathy was noted, and punch biopsies showed an ulcerated, hyperkeratotic, well-differentiated squamous proliferative lesion with no evidence of malignancy. Excision was under local anaesthesia and sedation, and the defect was closed by means of a split-skin graft with complete healing (Fig. 2). Histology confirmed KCM with typical squamo proliferative features at the periphery, and complete healing at the centre, with no evidence of malignancy (Figs 3, 4). The patient remains well 2 years after surgery, with no evidence of recurrence. Keratoacanthomas have been described under various titles.1 They arise from the hair follicles and form a raised nodule with a central keratinous plug, on exposed areas of skin in people past their fifth decade of life.1,2 They can occur following trauma, viral infections, drug eruptions and in association with certain skin conditions.1,2 Environmental hazards such as sunlight and chemical carcinogens are important initiators and promoters of the disease.1,2 Growth is characterized by three stages, comprising initial rapid growth, maturation, and resolution, each lasting 2–8weeks;2 most lesions resolve spontaneously, with only a few following an aggressive course. Keratoacanthoma centrifugum marginatum is a rare variant, showing rapidly progressive peripheral growth with coincident central healing and atrophy.3 It is this central atrophic healing despite a widening and sprawling periphery that is a characteristic of KCM.4 First reported in 1962 by Miedzinsky and Kosakiewicz,3 only 30 cases of KCM have been documented. The exact incidence is unknown. In reported cases, the diameter ranges from 5 to 30 cm and although spontaneous resolution can occur, most cases show progressive growth in a centrifugal fashion. Benest et al. note an age range of 24 to 70 years, with a median age of 60 years.5 The location is variable, with 21% resolving over 1 year.4 This contrasts with KA where resolution and healing occurs within 6months. Several cases have been noted to have been progressive over the years.4 No metastases have been described in the published reports.3 Keratoacanthoma centrifugum marginatum is known for what is termed ‘pseudocarcinomatous’ infiltration by pathologists.2,3 This feature is of great interest and importance because the cells produce a limited infiltration of the stroma, very much mimicking an early well-differentiated carcinoma2,3 (Fig. 5). It is this feature that divides pathologists into thinking that KA may be a well-differentiated variant of squamous cell carcinoma.3 Whereas several recurrences, but no metastases, have been reported, squamous cell carcinoma may occasionally arise from within KA, giving rise to the usual consequences.3 Whether this is an initial misdiagnosis, purely coincidental or a progression of KA is debated; most pathologists hold the view that KA is a distinct entity with a characteristic biological behaviour of its own.

Metastatic lobular carcinoma of the breast: an unusual case: Images for surgeons

An 83-year-old female presented to the Emergency Department with a 12-month history of left-sided abdominal pain, nausea but no vomiting and poor oral intake. She had lost 6 kg of body weight over this time. Colonoscopy 3 years prior was normal. She had previously undergone bilateral modified radical mastectomy 15 years earlier for synchronous bilateral lobular carcinoma of the breast, with adjuvant radiotherapy and hormone therapy. On clinical examination there were no positive findings aside from mild tenderness to palpation in the left quadrants. Basic hematology and biochemistry was unremarkable. Tumour markers revealed an elevated carcinoembryonic antigen level of 613 μg/L (<5), cancer antigen (CA) 19.9 of 58 kU/L (<35) and CA 15.3 of 322 kU/L (<30). A computed tomography scan of the abdomen and pelvis showed a long, thickened segment of descending colon with associated mesenteric fat-stranding (Fig. 1). Flexible sigmoidoscopy showed pale, thickened, nodular tissue almost obstructing the lumen of the descending colon (Fig. 2). Endoscopy revealed a similar appearance of the gastric antrum and proximal duodenum. Biopsies were consistent with metastatic lobular carcinoma of the breast (Fig. 3). She declined additional treatment and passed away several weeks later. Lobular carcinoma accounts for 4–15% of breast cancer. It is increasingly recognized as an entity quite different to the more predominant ductal carcinoma. Lobular carcinoma has a tendency to present with bilateral or multifocal disease. It carries a more favourable prognosis in the initial 5 years, however, by 10 years it is generally thought to have poorer outcomes in both disease-free and overall survival compared to ductal carcinoma. Indeed, it is not uncommon for lobular carcinoma to undergo late relapse with locally advanced distant metastases, as happened in this case. Gastrointestinal metastases account for less than 1% of breast cancer metastases, with lobular carcinoma responsible for the majority of these cases (70–80%). Cases of a variety of gastrointestinal metastases have been reported, including those with an infiltrative linitis-plastica type appearance. There is little evidence to guide treatment, which currently remains primarily the domain of the medical oncologists. Treatment involves palliative chemotherapy or hormone therapy, with limited success. Surgical intervention may be indicated where disease produces local complications such as obstruction or perforation. Gastrointestinal metastases are rarely isolated, and are often associated with peritoneal or retroperitoneal disease and malignant ascites.

Cutaneous angiosarcoma: a rare complication post‐male breast cancer radiotherapy

and to achieve a single pancreatic anastomosis, a complete resection of the split segment of the pancreatic body with both anteportal and retroportal parts was carried out in the presented patient. The right pancreatic resection should be extended about 2–3 cm to ensure a single pancreatic tail for anastomosis (Fig. 2) and can also be used for infrasplenic and complete circumportal pancreata as described by Hamanaka et al. and Hashimoto et al. The operative reconstruction can be performed either as pancreaticojejunostomy or pancreaticogastrostomy. Although the extended right resection requires the sacrifice of minimally more pancreatic parenchyma, we recommend this technique to achieve a single pancreatointestinal anastomosis to potentially lower the risk of post-operative pancreatic fistula in patients with CP.

Interesting case of gastric emphysema

A 56-year-old woman was presented to the emergency department with colicky epigastric pain on a background of diarrhoea and vomiting. An endoscopy 18 months earlier for symptomatic reflux had shown mild gastritis but was negative for Helicobacter pylori. Past medical history included systemic lupus erythematosus for which she was on regular prednisolone and Hashimoto’s thyroiditis. A computed tomography (CT) organized by her general practitioner showed a thin layer of gas tracking along the gastric wall at the fundus and proximal body of the stomach (Fig. 1). There was no associated free intraperitoneal gas. On examination, she was apyrexial and haemodynamically stable, with moderate tenderness to palpation in the epigastrium. A provisional diagnosis of gastric emphysema was made and the patient was treated conservatively with nasogastric aspiration, parenteral alimentation, broad spectrum antibiotics and proton pump inhibitor therapy. A repeat CT scan on day 11 of admission showed resolution of the emphysematous changes. An endoscopy performed during admission showed fundic gland gastric polyps but no ulceration. Biopsies revealed mild non-specific changes only. Enteric feeding was reintroduced and she was discharged after a 14-day admission. The presence of air in the wall of the stomach is a rare and concerning clinical finding. It is most often associated with bacterial infection, distention secondary to gastric outlet obstruction, mucosal trauma following instrumentation, gastric ischaemia or dissection from the mediastinum. Rarely, it can present secondary to extensive cardiopulmonary resuscitation, vomiting, ulcers and steroids. There are four theories of how air enters the gastric wall. (1) Bacterial – gas forming organisms, for example Clostridial sp, Escherichia coli, Staphylococcus aureus, generate the gas as part of an infective process. The prognosis is often poor in these cases. (2) Mechanical – air enters the wall due to increased mural pressure caused by insufflation or distal obstruction. (3) Mucosal – air enters the wall via damaged mucosa as in ulceration. (4) Pulmonary – alveolar air dissects down the mediastinum into the gastric wall in cases of severe asthma or emphysema. The condition could be further classified as gastric emphysema (intramural, interstitial or non-bacterial), emphysematous gastritis (infective or bacterial) and cystic pneumatosis (pneumatosis cystoides intestinalis). Gastric emphysema is typically less severe and sometimes asymptomatic and often resolves spontaneously. Patients often present systemically well with mild to moderate symptoms including abdominal pain. Emphysematous gastritis, however, is a severe form of gastritis often due to a more sinister underlying cause resulting in significant morbidity and mortality described at 60–80%. This is likely due to invasion through the gastric wall by gas-producing bacteria and patients present more unwell. This includes fever, nausea, vomiting/ haematemesis and abdominal pain. Differentiating gastric emphysema and emphysematous gastritis is made clinically and radiologically. Both will present with abdominal pain but the more severe pain, intractable vomiting, haemodynamic instability and rapid deterioration is hallmark of emphysematous gastritis. Early recognition of emphysematous gastritis is critical in reducing mortality. The most sensitive imaging modality to distinguish the two in conjunction with clinical findings is a CT scan of the abdomen. This will show air in the stomach wall; however, in emphysematous gastritis, the air will usually have a cystic/mottled appearance and may be associated with portal venous gas. The stomach wall may be thickened and there may be intramural penetration of contrast. In gastric emphysema, the air will appear classically more linear and there may not be any concomitant gastric wall changes. CT is also useful to exclude associated pneumoperitoneum. Endoscopic findings can include submucosal bubbles of gas, necrosis, ulceration and erosions; however, our case showed only mild non-specific changes supporting the likely diagnosis of gastric emphysema. Fig. 1. Admission computed tomography scan. IMAGES FOR SURGEONS

Metastatic lobular carcinoma of the breast: an unusual case

An 83-year-old female presented to the Emergency Department with a 12-month history of left-sided abdominal pain, nausea but no vomiting and poor oral intake. She had lost 6 kg of body weight over this time. Colonoscopy 3 years prior was normal. She had previously undergone bilateral modified radical mastectomy 15 years earlier for synchronous bilateral lobular carcinoma of the breast, with adjuvant radiotherapy and hormone therapy. On clinical examination there were no positive findings aside from mild tenderness to palpation in the left quadrants. Basic hematology and biochemistry was unremarkable. Tumour markers revealed an elevated carcinoembryonic antigen level of 613 μg/L (<5), cancer antigen (CA) 19.9 of 58 kU/L (<35) and CA 15.3 of 322 kU/L (<30). A computed tomography scan of the abdomen and pelvis showed a long, thickened segment of descending colon with associated mesenteric fat-stranding (Fig. 1). Flexible sigmoidoscopy showed pale, thickened, nodular tissue almost obstructing the lumen of the descending colon (Fig. 2). Endoscopy revealed a similar appearance of the gastric antrum and proximal duodenum. Biopsies were consistent with metastatic lobular carcinoma of the breast (Fig. 3). She declined additional treatment and passed away several weeks later. Lobular carcinoma accounts for 4–15% of breast cancer. It is increasingly recognized as an entity quite different to the more predominant ductal carcinoma. Lobular carcinoma has a tendency to present with bilateral or multifocal disease. It carries a more favourable prognosis in the initial 5 years, however, by 10 years it is generally thought to have poorer outcomes in both disease-free and overall survival compared to ductal carcinoma. Indeed, it is not uncommon for lobular carcinoma to undergo late relapse with locally advanced distant metastases, as happened in this case. Gastrointestinal metastases account for less than 1% of breast cancer metastases, with lobular carcinoma responsible for the majority of these cases (70–80%). Cases of a variety of gastrointestinal metastases have been reported, including those with an infiltrative linitis-plastica type appearance. There is little evidence to guide treatment, which currently remains primarily the domain of the medical oncologists. Treatment involves palliative chemotherapy or hormone therapy, with limited success. Surgical intervention may be indicated where disease produces local complications such as obstruction or perforation. Gastrointestinal metastases are rarely isolated, and are often associated with peritoneal or retroperitoneal disease and malignant ascites.