An algorithmic approach to perineal reconstruction after cancer resection--experience from two international centers.

AIM: This paper aims to simplify the approach to reconstruction of the perineum after resection of malignancies of the anal canal, lower rectum, vulva, and vagina. MATERIALS AND METHODS: The data were collected from 2 centers, namely, Addenbrooke's Hospital, University of Cambridge, United Kingdom and Christchurch Hospital, University of Otago, New Zealand. All patients who underwent perineal reconstruction from 1997 to 2009 at Christchurch Hospital (13 years) and 2001 to 2009 at Addenbrooke's Hospital (9 years) were included. The diagnosis (indication), primary surgery, reconstructive surgery, complications, tumor outcomes (recurrence and survival), and follow-up were entered into a database (Microsoft Excel; Redmond, Wash). The incidence of previous radiotherapy, requirement for adjuvant radiotherapy, and length of inpatient stay were also recorded. RESULTS: Forty-six patients were identified for this study--13 in New Zealand and 33 in Cambridge. Indications for perineal reconstruction included resection of anal and rectal malignancies (24), vulval and vaginal malignancy (19), perineal sarcoma (1), and perineal squamous cell carcinoma arising in an enterocutaneous fistula (Table 1). The reconstructive strategies adopted included rectus abdominis myocutaneous flaps (26), gluteal fold flaps (9), gracilis V-Y or advancement flaps (7) and others (4), gluteal rotation flaps (1), local flap (2), and free latissimus dorsi flaps (1). CONCLUSIONS: Although various surgeons performed the reconstructive surgeries at 2 different centers, the essential approach remained the same. Smaller defects were best treated by local flaps, whereas the rectus abdominis flap remained the standard option for larger defects that additionally required closure of dead space. On the basis of our 2 center experience, we propose a simple algorithm to facilitate the planning of reconstructive surgery for the perineum.

Cutaneous manifestations of NAXD deficiency - A case report.

Metabolism is a tightly regulated sequence of events, supported by key reactions between enzymes and enzyme-specific substrates. These reactions have the potential to produce metabolic side products that can have deleterious effects to further key metabolic reactions. The nicotinamide repair system consists of two partner enzymes, NAD(P)HX epimerase (NAXE) and NAD(P)HX dehydratase (NAXD). These enzymes regulate the levels of metabolic side products. Here we present a case of an 11-month old child who presented to our paediatric department with pyrexia, lethargy and multiple cutaneous lesions on the background of NAXD deficiency, a lethal neurometabolic disorder of early childhood. Despite early intervention with intravenous antibiotics, the patient failed to improve and subsequently passed away. The skin lesions were thought to be a consequence of systemic disease rather than a propagator of infection. Clinicians should be aware of this incredibly rare metabolic disease, its potential to cause widespread systemic dysfunction and the developing avenues for management.