Pediatric hemodialysis access.

Pediatric hemodialysis access is a demanding field. Procedures are infrequent, technically challenging, and associated with high complication and failure rates. Each procedure affects subsequent access and transplants sites. The choice is made easier and outcomes improved when access decisions are made by a multidisciplinary, pediatric, hemodialysis access team. This manuscript reviews the current literature and offers technical suggestions to improve outcomes.

Silver-impregnated hydrofiber dressing followed by delayed surgical closure for management of infants born with giant omphaloceles.

OBJECTIVE: We successfully employed silver-impregnated hydrofiber dressing for management of giant omphaloceles (GO) followed by delayed surgical closure. STUDY DESIGN: Between 2005 and 2008, eight consecutive GO infants were cared for at Driscoll Children's Hospital. Four patients had additional congenital anomalies including Beckwith-Wiedemann (n = 1), tetralogy of Fallot (n = 1), pulmonary hypoplasia (n = 1), and ruptured omphalocele (n=1). Infants underwent amnion epithelization using a silver-impregnated hydrofiber dressing over the course of several months followed by delayed surgical closure. Mean ± SD of parameters including maternal age, gestational age, infant weight, size of GO, preoperative intubation, preoperative hospitalization, time to epithelization, days to surgical closure, postoperative hospitalization, postoperative intubation and months of follow-up were studied. RESULTS: Five patients underwent successful closure, 2 were lost to follow-up and 1 was lost because of withdrawal of support. The maternal age, gestation age and weight of infant were 28 ± 5.3 years, 34 ± 4 weeks and 2.5 ± 0.62 kg, respectively. The GO size was 11 cm in length and 11 cm in width, respectively. Preoperative hospitalization days were 78 ± 74 days. Preoperative intubation was 3.5 ± 3.1 days with 2 neonates requiring tracheostomy and home ventilation owing to additional congenital abnormalities. Time to epithelization was 2.9 ± 0.9 months. Days to surgical closure and postoperative hospitalization were 331 ± 119 days and 5 ± 3.4 days, respectively. Average follow-up was 37 ± 27 months. No treatment associated morbidities are noted. CONCLUSIONS: Silver-impregnated hydrofiber mediated epithelization of GO followed by delayed surgical closure is safe for management of infants.

Management of preterm giant sacrococcygeal teratoma (GSCT) with an excellent outcome.

Infants born with a giant sacrococcygeal teratoma (GSCT; >10 cm) have high mortality. Risk factors for mortality include increased tumor vascularity, high cardiac output, rapid growth, diagnosis before 20-week gestation, delivery before 30-week gestation, hydrops, low birth weight, Apgar less than 7 at 5 min and polyhydramnios. We present the case of a 28-week infant born with a GSCT (15 × 12 × 16 cm) and all of these risk factors.

Eculizumab induces long-term remission in recurrent post-transplant HUS associated with C3 gene mutation.

A 15-year-old male patient developed atypical hemolytic uremic syndrome (aHUS) at 16 months of age leading to end-stage renal disease. The family history was suggestive of autosomal dominant aHUS, and he was more recently found to have a C3 heterozygous gene mutation (1835C>T mutation in exon 14, which determines the amino-acidic substitution R570W) with no other complement abnormalities. He had two renal transplants, the first at 2.5 years, and the second at 8 years of age, but allograft dysfunction developed in both transplants leading to graft failure due to recurrent HUS at 5 years and 18 months post-transplantation respectively. At 15 years of age he received a third transplant from a deceased donor with pre-emptive plasmapheresis. He had immediate graft function and nadir serum creatinine was 1.3-1.4 mg/dl. Severe allograft dysfunction and hypertension developed 2 months after transplantation following influenza infection. Renal allograft biopsy showed thrombotic microangiopathy. He received plasmapheresis followed by eculizumab therapy. Allograft function returned to baseline 3 weeks after starting therapy, and post-treatment allograft biopsies showed improvement in thrombotic microangiopathy. He continues to receive eculizumab every 2 weeks with stable graft function 13 months after transplantation.

Endoscopic management of recurrent congenital tracheoesophageal fistula: a review of techniques and results.

OBJECTIVE: Recurrent tracheoesophageal fistula (TEF) is a difficult problem in both diagnosis and management. Revision open repair with thoracotomy is challenging and has a significant associated morbidity. Because of the technical difficulty and the substantial morbidity, several authors have suggested and implemented endoscopic management. This paper reviews and describes the endoscopic techniques and management of recurrent TEF. METHODS: This retrospective study evaluates three patients who underwent endoscopic (bronchoscopic) management of recurrent TEF at Children's Memorial Hospital in Chicago, Illinois. Median follow-up is 48 months. A current literature review is presented with a synthesis of the data on techniques and results. RESULTS: Two of the three patients had successful bronchoscopic closure using fibrin adhesive. The mean number of procedures required was two. Including these patients, 62 patients with endoscopic closure of congenital TEF have been reported in the literature. The overall success rate is 60% with a mean of 2.1 procedures per patient. Of the obliterating agents described, tissue adhesive and fibrin adhesive have been employed most frequently with success rates of 48 and 55%, respectively. CONCLUSION: Endoscopic repair of recurrent TEF is an effective and safe alternative to second thoracotomy and open surgical repair. More than one endoscopic procedure is usually necessary for successful closure.

To drain or not to drain: a single institution experience with neonatal intestinal perforation.

AIMS: The optimal surgical treatment for extremely-low-birth-weight (ELBW) neonates with pneumoperitoneum is controversial. This study aimed to identify clinical factors associated with two known causes of pneumoperitoneum-necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP), and assesses the treatment outcome with primary peritoneal drainage (PPD) vs. laparotomy. METHODS: We reviewed and analyzed clinical characteristics and outcome from records of neonates with pneumoperitoneum treated at our institution from January 1999 to January 2003. RESULTS: Forty-six neonates (31 NEC, 15 SIP) were treated with either PPD (20 with NEC, 13 with SIP) or laparotomy (11 with NEC, 2 with SIP). In neonates who underwent PPD, those with NEC (vs. SIP) were less likely to have a patent ductus arteriosus, but were more likely to have been fed, have drains placed later in life, have a subsequent laparotomy, a longer total parental nutrition course, a higher 30-day mortality, and to take more days to begin enteral feeds. CONCLUSION: The etiology of pneumoperitoneum (NEC vs. SIP) in ELBW neonates can usually be determined preoperatively. Neonates with SIP should have a drain placed while those with NEC should undergo laparotomy.

Closing arguments for gastroschisis: management with silo reduction.

BACKGROUND: There are two approaches to close gastroschisis. Primary closure (PC) is reduction and fascial closure; silo closure (SC) places viscera in a preformed-silo and reduces the contents over time. We have shifted from PC to SC. This study compared the outcomes of these two techniques. METHODS: Records of babies with gastroschisis from 1994-2004 were reviewed. Closure type, ventilator days, days to full-feeds, hospital days, complications, and mortality were recorded. RESULTS: Twenty-eight patients underwent PC; 20 patients had SC. Differences in ventilator days, days to full-feeds, and hospital days were not statistically significant. Nine PC patients developed closure-related complications vs. none in SC (P < 0.05). Eight PC vs. two SC patients had non-closure-related complications (P < 0.05). Four PC vs. zero SC patients developed necrotizing enterocolitis (P < 0.05). Five PC vs. one SC patients had ventral hernia (P < 0.05). No patient died. CONCLUSION: PC resulted in higher incidence of reclosure, non-closure-related complications, and necrotizing enterocolitis. Consequently, we recommend SC as the preferred treatment.