Emily J. Stenhouse

A multidisciplinary team model of caring for patients with perianal Crohn's disease incorporating a literature review, topical therapy and personal practice

Background Crohns disease (CD) is characterised by periods of relapse and remission. Over time the disease leads almost inevitably to the complications of stricturing, penetration and fistulisation. Perianal CD involves areas of chronic abscess formation, ulceration, skin tags or fistula formation. This can be a particularly challenging and complex problem to manage, and a range of potential treatment modalities exist. Methods This review covers the management of perianal CD and provides recommendations for practice for the multidisciplinary team (MDT), including the use of wound management products and relevant clinical images. Results Current practice focuses predominantly on the use of antibiotic therapy, immunosuppression, immunomodulation and surgery. These therapies are used individually or in combination. The majority of evidence suggests that a combination of medical and surgical management produces the best disease outcomes. However, this treatment regime can be debilitating for the patient and compliance can be difficult. Published work on the use of topical therapy in the management of perianal CD focuses specifically on topical drug therapy; it does not, however, address the basic guiding principles of chronic wound management—in particular, optimal moisture control and the management of bacterial burden on the wound surface. Honey and silver-containing wound management products act as topical antimicrobial agents and therefore address these principles. Conclusions Perianal CD is the archetypal condition that exemplifies the need for an MDT approach in caring for patients with inflammatory bowel disease. A combination of treatment modalities that includes topical wound management is likely to produce the best patient outcomes.

Early Postnatal Ventricular Dysfunction Is Associated with Disease Severity in Patients with Congenital Diaphragmatic Hernia

Objective To assess patterns of postnatal ventricular function and their relationship to prenatal and postnatal markers of disease severity in infants with congenital diaphragmatic hernia (CDH). Study design In this observational case‐control study of cardiac function in infants with CDH in the first 5 days of life, systolic and diastolic function in the right ventricle (RV) and left ventricle (LV) were assessed using speckle tracking echocardiography‐derived global strain and tissue Doppler imaging. Correlation between cardiac function and prenatal observed:expected total fetal lung volume (TFLV), oxygenation index (OI), duration of intubation, and hospital length of stay were assessed. Results All measures of systolic and diastolic function were significantly reduced in the CDH group (n = 25) compared with controls (n = 20) at <48 hours, and were improved by 72‐120 hours. LV global systolic longitudinal strain (GLS) correlated with prenatal TFLV (R2 = 0.32; P = .03), OI (R2 = 0.35; P < .001), duration of intubation (R2 = 0.24; P = .04), and length of stay (R2 = 0.4; P = .006). Mean (SD) LV GLS at <48 hours was significantly lower in infants with CDH who did not survive and/or required ECMO compared with those who did not: −11.5 (5.3)% vs −16.9 (5.3)% (P = .02). Conclusions RV and LV function are impaired in the transitional period in infants with CDH. Early LV systolic function correlates with prenatal and postnatal markers of clinical disease severity and may be an important determinant of disease severity and therapeutic target in CDH. These findings support regular assessment of cardiac function in CDH and investigational trials of targeted cardiovascular therapies.

Congenital and Developmental Abnormalities

Congenital hand differences (CHD) have been estimated to occur in 10% of children born with congenital abnormalities. CHD can be classified according to their pre-dominant abnormality using the Swanson classification. Failure of differentiation represents the most common group. Associated (nonlimb) abnormalities are common and it is important to identify those CHD requiring systemic evaluation. Radiology is important in the diagnosis and management of CHD with plain films providing the mainstay of imaging postnatally. The management of CHD should be within a multi-disciplinary environment.

Acute abdominal pain in a child with inflammatory bowel disease

A 10 year old girl with inflammatory bowel disease presented with a two week history of eight to 10 bloody diarrhoeal stools a day, abdominal pain, and lethargy. She had been started on oral prednisolone four days before admission. On admission, her inflammatory markers were raised, with a C reactive protein 312 mg/L (reference value <3; 1 mg/L=9.52 nmol/L), white blood cell count 30×109/L (4-10), albumin 34 g/L (35-45), and haemoglobin 104 g/L (110-160). On examination she was unwell, with a heart rate of 140 beats/min, blood pressure of 100/70 mm Hg, and temperature of 38.2°C. She was cool peripherally, with a capillary refill time of three to four seconds, and her abdomen was soft but generally tender. Her paediatric ulcerative colitis activity index (PUCAI) score was 65 (≥65 defines severe disease). She underwent plain abdominal radiography (fig 1⇓). ### 1 What features are seen in this radiograph and what is the diagnosis? #### Short answer The radiograph shows abnormal colonic dilation, particularly in the transverse colon, with loss of normal haustration and thumbprinting, indicative of mucosal oedema (fig 2⇓). Mild central dilation of the small bowel is also present, but no evidence of perforation. The diagnosis is toxic megacolon complicating a case of acute severe colitis. A colonic diameter of greater than 56 mm, together with systemic toxicity, is diagnostic in children over the age of 10.1 Fig 2 Plain abdominal radiograph showing abnormal colonic dilation (double headed white arrow), loss of normal haustration, and thumbprinting indicating mucosal oedema within the colon (two single headed white arrows) in a child with inflammatory bowel disease and a toxic megacolon #### Long answer Although it may be …