Gestational gigantomastia complicated by pseudo-angiomatous stromal hyperplasia - a multidisciplinary management approach.

SUMMARY: Gestational gigantomastia is a rare condition typified by disproportionate bilateral breast enlargement in pregnant women, resulting in skin thinning, ulceration, and bleeding. Less than sixty cases have been documented worldwide, and only one other in South Africa. Pseudo-angiomatous stromal hyperplasia (PASH) is a rare benign proliferation of stromal tissue in a tumorous or diffuse pattern. This, to the best of our knowledge, is the first published case, a 27-year-old human immunodeficiency virus (HIV) positive woman, to present with both conditions concurrently. Medical management with cabergoline was initiated and, seven months post-delivery, a novel Goldilocks mastectomy was performed with acceptable outcomes.

Review: Endometrial function in pregnancy establishment in cattle.

The endometrium is fundamentally required for successful pregnancy in ruminants and species where the posthatching conceptus undergoes a protracted elongation and peri-implantation phase of pregnancy. Moreover, there are substantial waves of pregnancy loss during this pre- and peri-implantation period of pregnancy the precise source of which has not been clearly defined i.e., the maternal uterine contribution to this loss. Understanding the molecular interactions required for successful pregnancy in cattle will allow us to intervene to support pregnancy success during this vulnerable window. The endometrium contributes to most key developmental milestones of pregnancy establishment, including (1) contributing to the regulation of the oestrus cycle, (2) nourishing the preimplantation conceptus, (3) responding to the conceptus to create a more receptive microenvironment, (4) providing essential biophysical support, and (5) signalling and producing factors which affect the mother systemically. This review will summarise what we currently know about conceptus-maternal interactions as well as identify the gaps in our knowledge that could be filled with newer in vitro model approaches. These include the use of microfluidics, organ-on-a-chip devices, and bioinformatic approaches. This will help maximise food production efficiency (both meat and dairy) and decrease the environmental burden, while enhancing our understanding of the fundamental processes required for successful implantation in cattle.

Retroperitoneoscopic adrenalectomy - introducing a new surgical technique in South Africa.

BACKGROUND: Posterior retroperitoneoscopic adrenalectomy (PRA) is purported to be superior to the laparoscopic transperitoneal approach (LA) in patients with a select spectrum of benign adrenal pathologies. Advantages attributed to the technique include minimal blood loss, shortened operative times, and decreased postoperative pain, explained anatomically by the direct access to the adrenal gland. Reduced workspace is a limitation. METHODS: A retrospective review of 22 consecutive PRAs performed in a single centre between 1 September 2016 and 30 October 2020 is presented. Two experienced laparoscopic surgeons operated on all patients after acquiring the technique at international centres. Suitable candidates were carefully pre-selected. Non-benign pathology, high vascularity, body mass index (BMI) ≥ 45 kg/m2 and anatomical concerns on prior computed tomography (CT) imaging were exclusion criteria. RESULTS: Twenty-two PRAs were performed for a spectrum of benign adrenal pathologies. Twenty-one surgeries (95%) were completed. A single case of pheochromocytoma required conversion. The procedure was safe, a minor postoperative complication (pneumaturia) occurred in one case, and the mortality rate was 0%. Median operative time (80 minutes) is comparable to other reported series in the literature, with a modest linear descending tendency noted over the study period. Underlying pathology influenced operative times. Pheochromocytomas proved to be most challenging and required careful preoperative evaluation. CONCLUSION: The PRA procedures performed at Tygerberg Academic Hospital were safe and reproducible in a select group of cases with benign adrenal pathology. Endocrine surgeons proficient with laparoscopic techniques should be encouraged to use the PRA approach when encountering adrenal neoplasms that fit the criteria.

A comparison of invasive lobular carcinoma with other invasive breast cancers at Tygerberg Academic Hospital.

BACKGROUND: The second most common histological subtype of invasive breast carcinoma is invasive lobular carcinoma (ILC) occuring with a frequency 10-15% in Western countries and approximately 5%, in Africa, the Middle East and Asia (AMA). Combined hormone replacement therapy (CHRT) is a risk factor for the development of ILC which is infrequently diagnosed at our centre.This study aimed to investigate the incidence and clinicopathological characteristics of ILC as compared to invasive breast carcinoma of no special type (IBC-NST). METHODS: Clinical and pathological data on breast carcinoma patients attending the breast and endocrine unit at Tygerberg Academic Hospital since 2017 have been recorded on a Stellenbosch University REDCap® database. RESULTS: IBC-NST was the most frequent subtype diagnosed (83.9%) and ILC the second most common subtype (5.2%). Most ILCs were of luminal B intrinsic subtype, and the median size was slightly smaller than IBC-NST. There were significantly more grade 2 ILCs than IBC-NSTs (81.5% vs 50.9%). There was no statistical difference between stage and histological subtype. CONCLUSION: ILC has clinicopathological differences when compared to IBC-NST, although these were less pronounced in this study. The prevalence of ILC was similar to numbers reported in AMA. We hypothesise that there may be a discrepancy in the prevalence of ILC between public and private healthcare systems in South Africa, and that it may be due to differing trends in prescribing CHRT.

Impact of a multidisciplinary approach to ultrasound- guided thyroid fine-needle aspiration biopsy at Tygerberg Hospital, Cape Town, South Africa: A retrospective audit.

BACKGROUND: Thyroid nodules are common and mostly benign. Inadequate sampling generally occurs in 13 - 17% of thyroid fine-needle aspiration biopsies (FNABs), but the proportion was found to be as high as 45% on evaluating 100 ultrasound (US)-guided FNABs in a previous unpublished audit at Tygerberg Hospital, Cape Town, South Africa (SA). OBJECTIVES: Primary aim: To determine the diagnostic yield of US-guided thyroid biopsy after implementing changes to existing practices, involving the creation of a specialised clinic and applying protocols for referral and FNAB. Secondary aim: To compare the results with other centres in SA. METHODS: A retrospective audit of 178 thyroid biopsies was conducted. All US-guided thyroid biopsies performed in the specialised clinic between January 2017 and July 2018 were included. Data were analysed using descriptive statistics. RESULTS: The 178 nodules were biopsied in 159 patients. The mean age was 53.7 years, with a gender ratio of 9.6:1 (female/male). A reduction in non-diagnostic biopsies was noted compared with the historical cohort (45% v. 32.6%). Sixty-one nodules (34.3%) had previously been biopsied with inadequate cytology results. When repeat biopsies were excluded, only 16.2% (n=19) were classified as insufficient. CONCLUSIONS: These findings illustrate the importance of the multidisciplinary approach and standardisation of the US-guided biopsy procedure and the value of consistency and quality control in a health system. While nodular thyroid disease is common and FNAB is relatively simple, early referral to a central specialised unit to minimise the incidence of inadequate FNAB should be considered.

Symptomatic hepatothorax presenting 25 years after penetrating thoracoabdominal injury.

Hepatic herniation through the diaphragm is a rare finding. It generally occurs due to a congenital diaphragmatic abnormality or blunt trauma resulting in a diaphragmatic defect. Making the diagnosis is difficult, as there are few definitive clinical signs and chest radiograph (CXR) findings may be non-specific. To our knowledge, only a single case report exists of penetrating right diaphragm injury leading to hepatic herniation. A 42-year-old man presented to the emergency department of a regional hospital with hyperglycaemia and exertional dyspnoea. He was diagnosed with diabetes mellitus type 2. He gave a history of smoking for 15 pack-years, was negative for retroviral disease and had no history of pulmonary tuberculosis. He had no significant surgical history but reported being stabbed with a knife in 1995. The point of entry was below the level of the nipple in the right anterior axillary line. At the time, he was treated with an intercostal drain and discharged home. CXR showed a right-sided chest mass. We considered a differential diagnosis of pulmonary consolidation, diaphragm eventration or hepatothorax. Computerized tomography of the chest and abdomen demonstrated apparent intrathoracic extension of the right liver lobe and partial attenuation of the superior vena cava and right atrium due to a mass effect. The upper border of the liver abutted the aortic arch. Surgical treatment options were discussed. The patient declined surgery and will be followed up as an outpatient.

Assessment of the delayed repair of uncomplicated inguinal hernias in infants.

BACKGROUND: Potential strangulation of infant inguinal hernias is the main indication for their urgent repair. Lack of theatre time delays repair and prolongs hospitalisation. We report a series of patients with uncomplicated hernias who were discharged home to have their elective surgery at a later stage and assessed the outcomes of this approach. METHODS: A retrospective audit was performed of all infants with an inguinal hernia from January 2010 to June 2015. Incomplete records and infants operated after their first birthday were excluded. Two groups were identified; immediate surgery for infants with uncomplicated hernias, and delayed surgery for infants with uncomplicated hernias. Incarceration/strangulation rates in the interim period were documented for the delayed group, and comparison made between the groups regarding perioperative and anaesthetic complications and length of postoperative hospital stay. RESULTS: The mean time delay between diagnosis and repair was 8.78 weeks. None of the hernias in the delay group strangulated while awaiting repair. There was no significant difference in the perioperative complications between the two groups. Out of the 70 cases in the immediate repair group, there was 7 (10%) surgical and 4 (5.7%) anaesthetic complications. The delayed group (169 infants) had 8 (4.7%) surgical and 6 (3.6%) anaesthetic complications. The incarceration rate after being discharged home was 4.1%. This group of infants had no anaesthetic or surgical complications. Length of hospital stay postoperatively was 1.43 days in the immediate group and 1.3 in the delayed group (p = .485). CONCLUSION: Delayed repair, up to 2 months later, for uncomplicated infant hernia carries a small risk of incarceration but does not increase the rate of strangulation or other complications.

Hypercholesterolaemia screening in Type 1 diabetes: a difference of opinion.

AIM: To assess cholesterol screening of children with Type 1 diabetes by diabetes professionals using a survey of current practice, given that National Institute of Health and Care Excellence guidelines on childhood Type 1 diabetes do not recommend cholesterol screening, yet the National Paediatric Diabetes Audit has an annual cholesterol measure (> 12 years) as a key outcome indicator. METHODS: An online survey was sent to 280 members of the Association of Children's Diabetes Clinicians to assess cholesterol screening practice in children. RESULTS: A total of 87 diabetes professionals (31%) responded. The results showed that 94% of respondents measured cholesterol, 33% did this annually on all children, and 7% measured fasting cholesterol. A total of 63% used no guidelines to decide treatment or further investigation. The definition of 'high' cholesterol varied from > 4.5 to > 8 mmol/l, with 40% giving no response or specific level. Only 14% of clinicians had started statin therapy in their diabetes clinic in the previous 5 years. CONCLUSION: Whilst the majority of diabetes professionals measured cholesterol in children with Type 1 diabetes, there was marked variability in sampling, in children screened and in action taken if levels were considered abnormal. It is debatable whether cholesterol measures should be undertaken, certainly more than once, and whether cholesterol level should feature as a key outcome in the national audit in future.

The challenges of managing breast cancer in the developing world - a perspective from sub-Saharan Africa.

Communicable diseases are the major cause of mortality in lower-income countries. Consequently, local and international resources are channelled mainly into addressing the impact of these conditions. HIV, however, is being successfully treated, people are living longer,and disease patterns are changing. As populations age, the incidence of cancer inevitably increases. The World Health Organization has predicted a dramatic increase in global cancer cases during the next 15 years, the majority of which will occur in low- and middle-income countries. Cancer treatment is expensive and complex and in the developing world 5% of global cancer funds are spent on 70% of cancer cases. This paper reviews the challenges of managing breast cancer in the developing world, using sub-Saharan Africa as a model.

Care of children with diabetes as inpatients: frequency of admissions, clinical care and patient experience.

AIM: Hospital inpatient care for children with diabetes is frequently mentioned by parents as unsatisfactory. The aim of this study was to examine the reasons for inpatient admission of children with diabetes and to understand patient and carer experience in order to improve services. METHODS: Questionnaires were given to medical teams, parents and children during admissions of children with diabetes under 16 years of age in three regions of England. RESULTS: There were 401 admissions over 6 months from 3247 patients: 334 (83%) emergency admissions and 59 (15%) elective; the reason is unknown in eight (2%). One hundred and forty-three (36%) were emergency admissions with diabetic ketoacidosis/hyperglycaemia. Clinical teams reported adverse events around insulin administration in 25, hypoglycaemia (sometimes recurrent) in 120 and food provision in 14 admissions. Others included seven incidents around elective surgery. Diabetes clinical teams were not always informed about admissions and only 33% were informed within 2 h. Parents and children reported fewer problems: 62% were involved in care most of the time and 87% were able to give insulin. Most negative comments were about poor staff management of out-of-range blood glucose levels, knowledge of insulin pumps and care of children waiting in the emergency department. CONCLUSIONS: There were a large number of admissions and the majority were emergencies. Parents generally felt that they receive good care, although with some lack of knowledge amongst the ward staff. There were an unacceptable number of adverse incidents reported. We recommend that education of ward staff in diabetes is carried out regularly with reference to the standards of care.

Longitudinal screening of serum lipids in children and adolescents with Type 1 diabetes in a UK clinic population.

AIMS: To determine the prevalence of abnormal lipid levels in a large group of children and adolescents with Type 1 diabetes and to examine the changes longitudinally. In addition, to study the relationships of any lipid abnormalities to glycaemic control, age and duration of diabetes. METHODS: Non-fasting blood samples were taken annually from all the patients in the Oxford Children's diabetes clinic and total cholesterol (TC), high-density lipoprotein (HDL) cholesterol, triglycerides (TG) and glycated haemoglobin (HbA(1c)) measured over a period of 8 years. Low-density lipoprotein (LDL) cholesterol and non-HDL were calculated from these values and compared. Tests performed less than 4 months after diagnosis were excluded. RESULTS: A total of 229 children had complete data from more than 1 year and 798 sets of data were examined. TC was lower in males and increased with duration of diabetes and with increasing HbA(1c). HDL cholesterol fell with increasing age, but independently increased with duration, and was not related to HbA(1c). LDL cholesterol and non-HDL cholesterol were highly correlated (r = 0.9). Both were lower in males and increased with duration of diabetes. Non-HDL cholesterol increased with HbA(1c). A total of 23.7% had HDL cholesterol < 1.1 mmol/l and 22.5% had TC > 5.2 mmol/l. Thirty-eight per cent had LDL cholesterol > 2.6 mmol/l and 10.8% > 3.4 mmol/l, the thresholds for lifestyle and drug intervention respectively. CONCLUSIONS: Abnormalities in plasma lipid levels are common in this age group and the prevalence increases with poor glycaemic control and with duration of diabetes. Around 10% of adolescents would fit criteria for lipid-lowering medication in adults, but further study is needed to examine the risks and benefits in this age group.

Diabetes services in the UK: fourth national survey; are we meeting NSF standards and NICE guidelines?

BACKGROUND: Previous surveys of children's diabetes service provision in the UK have shown gradual improvements but continuing deficiencies. AIM: To determine whether further improvements in services have occurred. METHODS: A questionnaire was mailed to all paediatricians in the UK identified as providing care for children and adolescents with diabetes. Responses were compared with results of three previous surveys, and with recommendations in the Diabetes NSF and the NICE type 1 diabetes guidelines. RESULTS: Replies were received from 187 consultant paediatricians in 169 centres looking after children; 89% expressed a special interest in diabetes, 98% saw children in a designated diabetic clinic, and 95% clinics now have more than 40 patients. In 98% of the clinics there was a specialist nurse (82% now children's trained), but 61% clinics had a nurse:patient ratio <1:100; 39% of clinics did not have a paediatric dietician and in 78% there was no access to psychology/psychiatry services in clinics. Glycated haemoglobin was measured routinely at clinics in 86%, annual screening for retinopathy performed in 80%, and microalbuminuria in 83%. All centres now have local protocols for ketoacidosis, but not for children undergoing surgery (90%) or severe hypoglycaemia (74%). Mean clinic HbA1c levels were significantly lower in the clinics run by specialists (8.9%) than generalists (9.4%). There have been incremental improvements over the last 14 years since the surveys began, but only two clinics met all the 10 previously published recommendations on standards of care. CONCLUSIONS: The survey shows continuing improvements in organisational structure of services for children with diabetes but serious deficiencies remain. Publication and dissemination of the results of the previous surveys may have been associated with these improvements and similar recurrent service review may be applicable to services for other chronic childhood conditions.

Clinicopathological features of Meckel's diverticula in 32 patients, with emphasis on the presence of Helicobacter pylori.

Meckel's diverticula (MD) with areas of gastric heterotopia (metaplasia) are more likely to become symptomatic than those that contain mucosa of purely an intestinal type. Helicobacter pylori has been associated with the pathogenesis of various diseases. The aim of the study was to evaluate whether H. pylori could be identified within metaplastic gastric mucosa in MD. A retrospective evaluation of the histopathological features and the clinical presentation of patients with MD was undertaken. The study group included 32 cases (20 paediatric and 12 adults). The commonest modes of presentation were acute pain (25%), obstruction (19%), bleeding (12.5%) and hernias (9%). On histological examination, heterotopic gastric mucosa was noted to be present in 12 cases. Of these, 3 specimens were noted to have H. pylori organisms present using a specific immunostaining technique. Two patients had concurrent gastric biopsies; one stained positively for H. pylori but the second (1 mm biopsy) was rapid urease-test positive only. In conclusion, the study provides strong evidence that H. pylori may colonise heterotopic gastric mucosa in a minority of MD. H. pylori is apparently not involved in the pathogenesis of most cases of complicated MD. Nonetheless, all 3 patients who had evidence of H. pylori were symptomatic.