Massive Retro-Pneumoperitoneum and Lower Limb Subcutaneous Emphysema After Pediatric Heart Transplantation: A Case Report.

An emphysema in a lower limb is usually a clinical sign of a severe and life-threatening infection. We report a rare case of subcutaneous emphysema of the left lower limb associated with a massive retro-pneumoperitoneum and pneumatosis intestinalis after cardiac transplantation in a 4-year-old girl. The child was nearly asymptomatic beside an abdominal distension. A benign pneumoperitoneum associated with an extensive pneumatosis intestinalis is a rare complication after organ transplantation and should be treated conservatively. The association with an emphysema in a lower limb in a child has not been previously reported to our knowledge in the literature.

Liver transplantation in defects of cholesterol biosynthesis: the case of lathosterolosis.

We report the outcome of liver transplantation (LT) in the only surviving patient with lathosterolosis, a defect of cholesterol biosynthesis characterized by high lathosterol levels associated with progressive cholestasis, multiple congenital anomalies and mental retardation. From her diagnosis at age 2 she had shown autistic behavior, was unable to walk unaided and her sight was impaired by cataracts. By age 7 she developed end-stage liver disease. After a soul-searching discussion within the transplantation team, she was treated with LT as this represented her only lifesaving option. At 1-year follow-up, her lathosterol levels had returned to normal (0.61 mg/dL from 13.04 ± 2.65) and her nutrition improved. She began exploring her environment and walking by holding onto an adult's hand and then independently. Her brain magnetic resonance imaging (MRI) had shown a normal picture at age 1, whereas a volume reduction of white matter with ex vacuo ventricular dilatation and defective myelinization were observed before transplant. At 5-year follow-up, a complete biochemical recovery, an arrest of mental deterioration and a stable MRI picture were achieved, with a return to her every day life albeit with limitations. Timely liver transplant in defects of cholesterol biosynthesis might arrest the progression of neurological damage.

[Surgical treatment of Graves' disease: results in 108 patients]. / Trattamento chirurgico del morbo di basedow: risultati in 108 pazienti.

AIM OF THE STUDY: To evaluate morbidity and functional results of surgical treatment in patients with Graves' disease. METHODS: A retrospective study was performed in 108 patients operated on during 1993-2003. Main indications for surgery were failure of treatment with antithyroid drugs (80.6%), large goiter (46.3%) and/or severe ophthalmopathy (23.1%). Surgical procedures were extensive subtotal thyroidectomy (EST; n = 33; uni- or bilateral remnant of <2 g) or total thyroidectomy (TT; n=75). Functional results were established in 89 patients (27 EST patients and 62 TT patients) after a mean follow-up of 5.9 years. RESULTS: Operative mortality was zero. There were 4 (3.7%) transient unilateral recurrent laryngeal nerve (RLN) palsies and no cases of permanent RLN palsy. Temporary hypocalcemia occurred in 15 patients (13.9%) and permanent hypoparathyroidism resulted in two (1.9%). Four patients (3.7%) developed a postoperative hematoma that required reoperation. There was no significant difference in the rate of complications between EST and TT, although temporary hypocalcemia was more common following TT than EST (17.3% vs. 6.1%) and permanent hypoparathyroidism affected only TT patients. None of the patients developed recurrent hyperthyroidism; all patients are maintained on levothyroxine. CONCLUSIONS: Surgery is an effective therapy for selected cases of Graves' disease. When performed by experienced surgeons, it can be carried out with no mortality and minimal morbidity. EST (with uni- or bilateral remnant of <2 g) and TT are both effective in order to achieve a definitive cure of hyperthyroidism.

[Surgical management of pancreatic endocrine tumors in patients with MEN 1 syndrome. Considerations on one case observed]. / Problematiche chirurgiche nel trattamento delle neoplasie pancreatiche nel paziente affetto da MEN 1. Considerazioni su un caso osservato.

INTRODUCTION: Particular problems in MEN 1 syndrome come from the morphological identification of pancreatic tumors because of their are often small [<1 cm] and multiple [89% of the cases]. However intraoperatively it could be difficult to identify with palpation the tumors described by preoperative investigations and to decide the most suitable surgical treatment. The authors describe one case recently observed to underline and update the correct management. CASE REPORT: A 34 year old woman was admitted for the surgical treatment of an insulinoma. Polimenorrea, hypercalcemia and familiarity for MEN 1 syndrome were also present. A CT scan showed the tumors in the body and tail of the pancreas [diameter 0.5-1 cm]. MRI described only a small mass in pancreatic head. A calcium angiography was positive for insulin secretion after calcium infusion in hepatic and gastroduodenal artery, and for glucagon secretion after infusion in splenic artery. An intraoperative ultrasonography discovered three nodules that were enucleated. They were one insulinoma and two glucagonomas respectively. After enucleation glycemia became immediately normal. CONCLUSION: To avoid wide surgical resections [es. left pancreatectomy] we suggest a conservative treatment [multiple enucletion with or without a pancreatic-jejunum side-to-side anastomosis] with a meticulous preoperative and intraoperative evaluation of all pancreatic nodules.