Mechanical bowel preparation for esophagocoloplasty in children: is it really necessary?

Esophagocoloplasty is a commonly performed procedure for esophageal replacement in children. Traditionally, mechanical bowel preparation (MBP) is performed before this operation. However, this practice has been questioned, initially in adults and now in children. The aim of this study was to evaluate the influence of MBP on esophagocoloplasty in a series of children. Data collected from 164 patients who underwent esophagocoloplasty in the Pediatric Surgery Division, University of São Paulo Medical School, from February 1978 to July 2011 were reviewed for postoperative complications. In 134 patients, at least one kind of MBP was performed before the surgery (PREP group). MBP was omitted in 30 patients (NO-PREP group). There was no statistical difference between the groups in the rates of evisceration, colocolic, or cologastric anastomotic dehiscence and death. However, in the NO-PREP group, the incidence of cervical leakage (6.6%) was significantly decreased in comparison with the classical PREP group (25.3%) (P= 0.03). The results of this study suggest that the omission of MBP has a positive impact on the incidence of postoperative complications in esophagocoloplasty.

Living related donor liver transplantation in children.

OBJECTIVE: The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors. METHODS: We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010. RESULTS: Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3). The age of the recipients ranged from 7-174 months (median, 22) and the weights ranged from 6-58 kg (median, 10). Forty-nine children (40.5%) weighed ≤10 kg. The grafts included the left lateral segment (n = 108), the left lobe (n = 12), and the right lobe (n = 1). The donors included 71 mothers, 45 fathers, 2 uncles, 1 grandmother, 1 grandfather, and 1 sister with a median age of 29 years (range, 16-53 ys) and a median weight of 68 kg (range, 47-106). Sixteen patients (12.9%) required retransplantation, most commonly due to hepatic artery thrombosis (HAT; n = 13; 10.7%). The other complications were biliary stenosis (n = 25; 20.6%), portal vein thrombosis (PVT; n = 11; 9.1%), portal vein stenosis (n = 5; 4.1%), hepatic vein stenosis (n = 6; 4.9%), and lymphoproliferative disorders (n = 8; 6.6%). The ultimate survival rate of recipients was 90.3% after 1 year and 75.8% after 3 years. Causes of early death within 1 month were HAT (n = 6), PVT (n = 2), severe graft dysfunction (n = 1), sepsis (n = 1), and intraoperative death in children with acute liver failure (n = 2). Causes of late deaths included lymphoproliferative disease (n = 3), chronic rejection (n = 2), biliary complications (n = 3), and recurrent disease (n = 3; hepatoblastoma and primary sclerosing cholangitis). CONCLUSIONS: Despite the heightened possibility of complications (mainly vascular), LRDLT represented a good alternative to transplantation from cadaveric donors in pediatric populations. It was associated with a high survival ratio.

Investigation into sleep disturbance of patients suffering from cluster headache.

The new discoveries relating to cluster headache (CH) encouraged the study of the relationship of the hypothalamus to respiratory physiology and its comorbidity with sleep apnoea. The question is whether the apnoeas are more frequent during REM sleep and the desaturations could be involved as triggers of the cluster attacks. Furthermore, could the connection with the hypothalamus, already proved, be responsible for an alteration in the structure of REM sleep and a chemoreceptor dysfunction. We set out to analyse when polysomnography investigation is necessary in patients with CH. We studied 37 patients suffering from episodic CH, 31 (83.8%) men and six (16.2%) women. For the control group, we selected 35 individuals, 31 (88.6%) men and four (11.4%) women. There was a greater percentage of obstructive sleep apnoea (OSA) in patients with CH (58.3%) compared with the control group (14.3%) and with the general population (2-4%). In cases of pain during sleep, the majority is deflagrated during the REM phase, following a desaturation episode. A stratified analysis of the apnoea/hypnoea index relating to body mass index (BMI) and age showed that patients with CH have 8.4 times more chance of exhibiting OSA than normal individuals (P < 0001). This risk increases to 24.38 in patients with a BMI > 25 kg/m(2) and increases to 13.5 in patients > 40 years old. Surprisingly, the risk decreases sharply in patients with a BMI < 25 kg/m(2) and who are < 40 years old. Due to the fact that polysomnography is a complex, costly and sometimes difficult examination, we suggest, in concordance with the results, that it should be carried out routinely in patients with CH that exhibit a BMI of > 25 kg/m(2) and/or in patients who are > 40 years of age.

Involuntary rhythmic leg movements time-locked with the respiratory cycle.

Involuntary rhythmic leg movements in childhood is an uncommon condition, the generators of which remain unknown. We report on a male 3 years of age with distinct features providing important clues concerning the location of one of these generators. At the age of 7 months, the previously healthy young male started with low frequency, rhythmic, and continuous (both during wakefulness and sleep) flexion/extension movements of the lower limbs. Movements interfered significantly with gait acquisition, and, despite normal cognitive development, he was able to walk only at age 2 years, 4 months. The neurologic examination revealed the absence of automatic stepping in the neonatal period, but was otherwise normal. A polygraphic electroencephalogram/electromyogram (EEG/EMG) recording, at the age of 2 years, 9 months, revealed rhythmic and synchronous legs with EMG activity at 0.5 Hz. A more complete polygraphic recording at the age of 3 years, 10 months, showed a lower frequency (0.35 Hz) for the movements, which were time-locked with the respiratory cycle. Magnetic resonance imaging (MRI) of the brain revealed an increased T(2) signal in the upper medulla-lower pons regions. The generator of the rhythmic legs movements is postulated to be the respiratory center, connecting with the reticulospinal projecting neurons through an aberrant pathway.