Use of airway pressure release ventilation in a child with refractory hepatopulmonary syndrome after liver transplantation

HPS is a life‐threatening condition in patients with end‐stage liver disease, in which intrapulmonary vascular dilatations result in intrapulmonary shunts and hypoxemia. The only successful treatment is liver transplantation. Hypoxemia may be severe prior to transplantation; however, it can worsen or become refractory after liver transplantation and result in increased post‐operative mortality. Here, we present the case of a 10‐month‐old female infant with progressive end‐stage liver disease and severe HPS, who developed refractory hypoxemia after a successful liver transplantation. After 19 days of unsuccessful attempts to reverse the hypoxemia using conventional mechanical ventilation and HFOV, the patient responded dramatically to APRV, with rapid improvement in her PaO₂ and sharp decline in her OI. She was able to begin weaning from APRV two days later and was extubated within seven days. APRV was successful in treating refractory hypoxemia in this patient with severe HPS after liver transplantation, possibly by modifying distribution of pulmonary blood flow. Although we cannot rule out coincidental natural resolution of the HPS, APRV could be a useful rescue therapy in patients with HPS and refractory hypoxemia.     

Multiple myeloma and chronic myelomonocytic leukaemia developing in a patient with autoimmune disease

A 64-year-old lady with a personal and family history of autoimmune disease developed chronic myelomonocytic leukaemia and multiple myeloma simultaneously. Her sister died of acute myelomonocytic leukaemia, but showed no evidence of autoimmune disease. It is possible that chronic immunological stimulation, perhaps by an autoantigen, may predispose toward malignant transformation in both plasma cell and monocyte series. However, the present observations raise the alternative possibility of a primary disorder of monocytes that predisposes toward both autoimmune disease and a clonal disorder of plasma cells.     

Benign thyroid adenoma causing recurrent laryngeal nerve palsy in a child

The standard teaching is that only malignant thyroid masses produce recurrent laryngeal nerve palsy. An unusual exception to this rule is reported.     

Therapeutic aspects of fat embolism syndrome

Signs and symptoms of clinical fat embolism syndrome (FES) usually begin within 24-48 hours after trauma. The classic triad involves pulmonary changes, cerebral dysfunction, and petechial rash. Clinical diagnosis is key because laboratory and radiographic diagnosis is not specific and can be inconsistent. The duration of FES is difficult to predict because it is often subclinical or may be overshadowed by other illnesses or injuries. Medical care is prophylactic or supportive, including early fixation and general ICU management to ensure adequate oxygenation and ventilation, hemodynamic stability, prophylaxis of deep venous thrombosis, stress-related gastrointestinal bleeding, and nutrition. Studies support early fracture fixation as a method to reduce recurrent fat embolism and FES. The main therapeutic interventions once FES has been clinically diagnosed are directed towards support of pulmonary and neurological manifestations and management of acute lung injury (ALI) and acute respiratory distress syndrome (ARDS).     

The effect of obesity on the results of Karydakis technique for the management of chronic pilonidal sinus

BACKGROUND AND AIMS: Body mass index (BMI) was assessed as an objective indicator of obesity to determine whether it has an effect on the results of the Karydakis technique for managing chronic pilonidal sinus. PATIENTS AND METHODS: The prospective study included 41 consecutive patients with chronic pilonidal sinus categorized according to their BMI as obese (BMI >/=26.5, n=32) or nonobese (BMI <26.5, n=9). Patient data included demographics, symptoms, duration of disease, previous management, postoperative course, morbidity, and recurrence. All patients were treated by the Karydakis flap and were discharged on the 2nd-4th day postoperatively. Patients were followed-up for a mean of 25.7 months (range 3-38). RESULTS: The mean operating time was significantly longer in obese patients, and there was a trend to mean hospital stay being longer in them than in nonobese patients. Minor postoperative complications were encountered in four patients: two in the obese group had a seroma that resolved in 2 weeks with conservative measures, and two others, one in each group, suffered from wound infection that resulted in partial wound dehiscence in the obese patient but healed with secondary intention after 20 days without prolonging hospital stay. In the obese group the mean BMI of patients with complications (excluding recurrence) was significantly higher than that in patients without complications. Recurrence, at 13 months postoperatively, was observed in only one patient (3.1%) who had a BMI of 32.2. CONCLUSION: These findings show that (a) the Karydakis procedure for managing chronic pilonidal sinus in obese patients is easy to perform, has a fast healing time, short hospital stay, rapid return to work, and a low recurrence rate, (b) the results are similar between nonobese and obese patients, with no recurrence seen in these patients, and (c) complications occur in patients with a BMI greater than 30; in such patients a dietary regimen before operation is advisable, and inserting a suction drain during operation is recommended to avoid seroma formation.     

Intracordal Teflon injection: a question of timing

Intracordal Teflon injection is commonly performed for recurrent laryngeal nerve palsy. It is generally agreed that injection should not be performed for a period of six months from the onset of an 'idiopathic' paralysis. A case is presented in which recovery occurred late, after intracordal injection, with unfortunate results. Successful removal of the Teflon is described. The advisability of a longer wait before surgery for 'idiopathic' recurrent laryngeal nerve palsy is considered.