Hepatopulmonary fusion in a newborn. An uncommon intraoperatory finding during right congenital diaphragmatic hernia surgery: case description and review of literature.

Hepatic pulmonary fusion is a rare malformation associated with right congenital diaphragmatic hernia (CDH), often only discovered during surgical repair of the defect. Fourteen previous cases have been reported in the literature. We describe a case of a full term male newborn with prenatal ultrasound diagnosis of right CDH who underwent a thoracoscopy converted to a thoracotomy, due to this rare aforementioned intraoperative incidental finding. We reviewed the previous reported literature, especially focusing on the chosen surgical approach, concluding that an early and appropriate preoperative imaging investigation may be crucial for the best management of these kinds of patients.

Giant omphaloceles with a small abdominal defect: prenatal diagnosis and neonatal management.

A giant omphalocele is a liver-containing protrusion through an abdominal defect wider than 5 cm in diameter. The giant form with a small abdominal wall defect is a rare condition which, to our knowledge, has not been described previously. We describe three cases with the typical features of elongated vascular liver pedicle and angiomatosis of the hepatic portal system. The abnormal liver organogenesis, due to extra-abdominal development, represented a significant risk factor for hepatic thrombosis after visceral reduction and liver rotation. All the neonates underwent surgery on the first day of postnatal life. One died because of a postoperative liver infarction, and the survivors needed prolonged respiratory support. Prenatal sonographic features, timing, delivery, type of surgical repair, and postnatal outcome are reviewed. A prenatal sonographic diagnosis could be useful to evaluate the abdominal ring and serial ultrasound examinations are recommended to detect promptly ominous signs of hepatic and bowel damage. Color Doppler may be useful to assess the anatomy of the abdominal vessels and their relationships with the herniated organs, although it was not used in any of the cases reported here. This congenital malformation might be considered as a pathological entity separate from giant omphalocele with large abdominal defect, with a severe prognosis due possibly to its different embryological development.

Acute airway obstruction in an infant with Pierre Robin syndrome after palatoplasty.

This report describes a complication of post-operative oedema of the palate, tongue and pharynx after a Perko-revised cleft palate repair, which resulted in a life-threatening airway obstruction in an infant with Pierre Robin syndrome. Although infants experiencing airway problems after Wardill-Kilner, von Langenbeck and Furlow palatoplasty have been described, airway complications in a group of Perko-revised repair children have not been previously reported. We speculate that this complication, which occurred in the absence of a history of previous airway problems, is due to prolonged operating time and excessive pressure exerted on the base of the tongue by the Kilner-Doughty retractor. Acknowledgments of this risk permits to identify those patients prior to surgery so that they can be managed appropriately.

Incidence of vomiting in susceptible children under regional analgesia with two different anaesthetic techniques.

BACKGROUND: Postoperative vomiting (POV) is a frequent side-effect of paediatric surgery and a leading cause of unplanned admission. Many antiemetic drugs have been studied, but less attention has been given to the effects on POV of the anaesthetic technique adopted. The aim of this study was to compare two different anaesthetic techniques in children under regional analgesia at risk for POV. METHODS: We studied 135 children suffering from motion sickness or with a previous history of POV. The patients were randomized to receive inhalation anaesthesia (group S) with sevoflurane by LMA or intravenous (i.v.) anaesthesia with ketamine and propofol (group P). All the patients were treated with an ilio-inguinal block after induction of anaesthesia. Postoperatively, the children were followed by the nursing staff and by their parents, none of whom were aware of the anaesthesia technique used. RESULTS: A significant decrease was observed in the incidence of early (0-6 h) and delayed (6-24 h) POV in those children who received i.v. sedation. There was no difference between the two groups in the level of analgesia either at the end of surgery or 2 h postoperatively. CONCLUSIONS: Anaesthesia based on propofol and ketamine is better than inhalation anaesthesia with sevoflurane by LMA for reducing POV in children at risk under an ilio-inguinal block. Tailoring the anaesthetic to the specific needs of children susceptible to POV should be considered before resorting to the routine use of expensive antiemetic prophylaxis.

Anaesthetic management of children with type II and III Gaucher disease.

The anaesthetic management of two children with Gaucher disease is described. The cases were a girl aged 12 and a boy aged 4 who had undergone 13 and 3 anaesthetic interventions respectively for endoscopic, orthopaedic, general, otolaryngologic and dental surgical procedures over a total period of 11 years. Anaesthetic management of these patients can be achieved with various agents and techniques, but care is needed to identify preoperative end-organ dysfunction and prevent postoperative respiratory insufficiency. In particular, regional anaesthesia, when suitable, has certain advantages that have made it a worthwhile alternative to general anaesthesia.

Multiple and various anaesthetics, ketamine included, in a young patient with familial dysautonomia, Case report.

A case report is presented of a 16-year-old patient who had undergone 16 general anaesthetics by different anaesthesists and under various anaesthetic techniques for dental, endoscopic, orthopaedic and ophthalmic surgical procedures over a period of 14 years. Use of ketamine, especially in an ambulatory setting, was found more suitable in terms of cardiovascular stability, safety and patient preference.