Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant.

We will report a case of a desmoid tumour (DT), which developed at the surgical site of the pacemaker after a late childhood heart transplant. Patients with idiopathic dilated cardiomyopathy followed up in the paediatric cardiology service. It evolved with the dissociation of ventricular rhythm caused by severe heart failure, which led to the implantation of a cardiac resynchronization device prior to heart transplantation. The progression to end-stage heart disease culminated in a heart transplant at 12 years old. One year after the transplant, at the age of 13 years, he presented a progressively growing mass on the generator site of the resynchronization device. The initial decision was to remove the device. During the removal surgery, there was no haematoma or fluid collection. However, there was a progression of the lesion. The lesion was biopsied with the anatomopathological diagnosis of a DT. Resection surgery happened 4 months after the start of the mass growth. At that time, the tumour reached 20 cm in diameter. The lesion infiltrated the pectoralis major muscle and this muscle was resected partially en bloc with the lesion. The defect had primary closure. The patient evolved without postoperative complications and was discharged on the 14th postoperative day. The surgical specimen came with negative circumferential margins. However, the deep margin was microscopically positive. Due to deep involvement, the patient underwent adjuvant radiotherapy. Currently, the patient is under clinical follow-up and has no evidence of tumour recurrence. DT is a rare tumour, with unpredictable courses. Surgery can be considered in the progression of lesions. Treatment is justified by long survival after a heart transplant and in DT patients. DT is a differential diagnosis to be considered in progressive growth lesions.

Madelung disease (multiple symmetric lipomatosis).

Madelung disease or multiple symmetric lipomatosis (MSL) is a rare entity among the overgrowth syndromes. It is characterized by painless non-encapsulated and symmetric fatty deposits in the neck, torso, mammary, and abdominal areas, and in the upper and lower limbs. The etiology of the disease is still unknown. Chronic alcohol consumption may play a role in adipocyte hyperplasia in genetically susceptible individuals. Besides the overgrowth of adipose tissue, patients with MSL present features of metabolic syndrome. Patients seek medical attention usually for esthetic reasons. We present the case of a middle-aged man who sought the outpatient clinic complaining of bulging masses in the posterior upper part of the thorax, the occipital area, and the neck. The masses grew over a period of 2 years. The physical examination and imaging study revealed the presence of symmetric lipomatosis. A two-step surgical treatment was undertaken for the excision of the lipomatous tissue. The postoperative outcome was uneventful with satisfactory esthetic results.

Abdominal and pelvic actinomycosis due to longstanding intrauterine device: a slow and devastating infection.

Actinomycosis is a chronic or subacute bacterial infection characterized by large abscess formation, caused mainly by the gram-positive non-acid-fast, anaerobic, or microaerophilic/capnophilic, obligate parasites bacteria from the Actinomyces genus. Although pelvic inflammatory disease is an entity associated with the longstanding use of intrauterine devices (IUDs), actinomycosis is not one of the most frequent infections associated with IUDs. We present the case of a 43-year-old female patient who was referred to the emergency facility because of a 20-day history of abdominal pain with signs of peritoneal irritation. Imaging exams revealed collections confined to the pelvis, plus the presence of an IUD and evidence of sepsis, which was consistent with diffuse peritonitis. An exploratory laparotomy was undertaken, and a ruptured left tubal abscess was found along with peritonitis, and a huge amount of purulent secretion in the pelvis and abdominal cavity. Extensive lavage of the cavities with saline, a left salpingo-oophorectomy, and drainage of the cavities were performed. The histopathological examination of the surgical specimen revealed an acute salpingitis with abscesses containing sulfur granules. Therefore, the diagnosis of abdominal and pelvic actinomycosis was made. The postoperative outcome was troublesome and complicated with a colocutaneous fistula, which drained through the surgical wound. A second surgical approach was needed, requiring another extensive lavage and drainage of the recto-uterine pouch, plus the performance of a colostomy. Broad-spectrum antibiotics added to ampicillin were the first antimicrobial regimen followed by 4 weeks of amoxicillin during the outpatient follow-up. The patient satisfactorily recovered and is already scheduled for the intestinal transit reconstitution.

Madelung disease (multiple symmetric lipomatosis)

Madelung disease or multiple symmetric lipomatosis (MSL) is a rare entity among the overgrowth syndromes. It is characterized by painless non-encapsulated and symmetric fatty deposits in the neck, torso, mammary, and abdominal areas, and in the upper and lower limbs. The etiology of the disease is still unknown. Chronic alcohol consumption may play a role in adipocyte hyperplasia in genetically susceptible individuals. Besides the overgrowth of adipose tissue, patients with MSL present features of metabolic syndrome. Patients seek medical attention usually for esthetic reasons. We present the case of a middle-aged man who sought the outpatient clinic complaining of bulging masses in the posterior upper part of the thorax, the occipital area, and the neck. The masses grew over a period of 2 years. The physical examination and imaging study revealed the presence of symmetric lipomatosis. A two-step surgical treatment was undertaken for the excision of the lipomatous tissue. The postoperative outcome was uneventful with satisfactory esthetic results.

Abdominal and pelvic actinomycosis due to longstanding intrauterine device: a slow and devastating infection

Actinomycosis is a chronic or subacute bacterial infection characterized by large abscess formation, caused mainly by the gram-positive non-acid-fast, anaerobic, or microaerophilic/capnophilic, obligate parasites bacteria from the genus. Although pelvic inflammatory disease is an entity associated with the longstanding use of intrauterine devices (IUDs), actinomycosis is not one of the most frequent infections associated with IUDs. We present the case of a 43-year-old female patient who was referred to the emergency facility because of a 20-day history of abdominal pain with signs of peritoneal irritation. Imaging exams revealed collections confined to the pelvis, plus the presence of an IUD and evidence of sepsis, which was consistent with diffuse peritonitis. An exploratory laparotomy was undertaken, and a ruptured left tubal abscess was found along with peritonitis, and a huge amount of purulent secretion in the pelvis and abdominal cavity. Extensive lavage of the cavities with saline, a left salpingo-oophorectomy, and drainage of the cavities were performed. The histopathological examination of the surgical specimen revealed an acute salpingitis with abscesses containing sulfur granules. Therefore, the diagnosis of abdominal and pelvic actinomycosis was made. The postoperative outcome was troublesome and complicated with a colocutaneous fistula, which drained through the surgical wound. A second surgical approach was needed, requiring another extensive lavage and drainage of the recto-uterine pouch, plus the performance of a colostomy. Broad-spectrum antibiotics added to ampicillin were the first antimicrobial regimen followed by 4 weeks of amoxicillin during the outpatient follow-up. The patient satisfactorily recovered and is already scheduled for the intestinal transit reconstitution.