Early structured surgical management plan for neonates with short bowel syndrome may improve outcomes.

BACKGROUND: In children with short bowel syndrome, maximal adaptation of the bowel after extensive resection is thought to occur during the first 2 years of life. The aim of the present study was to review children with short bowel syndrome from two intestinal rehabilitation centers, comparing those undergoing lengthening procedures <365 days of age (early) versus those whose lengthening procedure was carried out >365 days of age (late). METHODS: Retrospective data collection was performed from January 2004 to December 2010 in Manchester, UK, and from December 2006 to December 2010 in Brussels, Belgium. Both medical centers follow a similar intestinal rehabilitation program (IRP). Data collected included population demographics, bowel length preoperatively and postoperatively, age at operation, parenteral nutrition (PN), central access, and complications. RESULTS: Complete data were available for eight children who underwent lengthening surgery at <365 days of age, and six who underwent the procedure at >365 days of age. Diagnoses were similar. Groups were matched for gestation and birthweight, with no statistical difference in preoperative and postoperative bowel lengths. The mean duration of PN postoperatively was 378 days in the early cohort and 589 days in the late cohort. This trended toward statistical significance (p = 0.071). Full enteral autonomy was achieved at 17 months (early) and 59 months (late) (p = 0.01). Patients in the early group required fewer central lines than those operated on later (p = 0.035). CONCLUSIONS: Enrolling children into an IRP involving early (<365 days of age) lengthening surgery allows a shorter postoperative time to allow weaning to full enteral nutrition, as well as fewer central lines. Both outcomes provide benefits for the child and family, allowing an earlier return to normal life.

Is intestinal transplantation the future of children with definitive intestinal insufficiency?

UNLABELLED: Intestinal transplantation (IT) is the newest and most difficult of organ transplantations. The first ever (1987) and the longest surviving (1989) IT were performed in our institution. However, IT still has to demonstrate its benefit to children on long-term parenteral nutrition (PN). We tried to clarify this aspect by looking back at our 13 years' experience. PATIENTS: From 1994 to December 2007, 74 IT were performed in 69 children, 39 with an isolated small bowel (IT), 35 combined with a liver transplant (LITx). The indications were: short bowel syndrome (n = 25), congenital mucosal diseases (n = 22), and motility disorders (n = 22). Median age at transplantation was 5 years (1 - 17 years). Follow-up was 1 to 12 years (median 5 years). RESULTS: Thirty-one children have a functioning graft (42 %), 15/39 IT, 16/35 LITx. They are at home without PN, with a good quality of life. One child is PN-dependent 1.5 years post IT. Post IT, 16 children were detransplanted: 12 early on (1 for mechanical complications, 11 because of resistant rejection; 3 less than 3 years, one 9 years post SBT (chronic rejection). In 2 noncompliant teenagers, PN was reintroduced (one was detransplanted later on). Several years post LITx, 2 children underwent bowel detransplantation due to an acute viral infection complicated with rejection. Twenty-two children died (32 %, 8 IT, 14 LITx), 18 early on from infectious or surgical complications, 4 more than 1 year post IT, 3 after retransplantation (1 in another unit). Bad prognostic factors are multiple previous surgeries, an older age (> 7 y), and chronic intestinal pseudo-obstruction. DISCUSSION: Complications post IT are frequent and life-threatening, especially early on: rejection (IT), infections (LITx). Later on, the rate of complications decreases but remains significant, especially in noncompliant patients. However we describe here a 13-year learning curve; the recent results are encouraging with regard to control of rejection and viral infections. CONCLUSION: Intestinal transplantation is indicated only in selected patients in whom long-term PN cannot be performed safely any more. In every child with intestinal insufficiency, the therapeutic strategy must be discussed early on in order to perform IT at the right time under optimal conditions. IT should evolve from being a "rescue" procedure to becoming a true therapeutic option.

Hepatic abscess and umbilical venous catheter: one cannot be cavalier about proper position.

PURPOSE The umbilical venous catheter (UVC) is routinely used in neonatal intensive care. Incorrect placement may expose the patient to some dangerous complications (i.e. thrombosis, abscesses, etc). We report a case of an hepatic abscess due to incorrect positioning of this device. We describe how the abscess was treated and we emphasize that one cannot be cavalier about proper positioning of the UVC.

Measures to prevent renal impairment after resection of retroperitoneal neuroblastoma.

Postoperative renal impairment has been reported after kidney-preserving resection of retroperitoneal neuroblastomas (NB). To avoid renal damage, intraoperative procedures include monitoring of central venous pressure (CVP) and diuresis, i.v. administration of electrolyte solutions, mannitol, and furosemide, and local application of lidocaine to the renal vessels. During the first 5 postoperative (p.o.) days, CVP, diuresis, body temperature (T), and lactate dehydrogenase (LDH) levels were monitored; color Doppler ultrasound (CDUS) was also performed on the 7th p.o. day. Simultaneous increases of T (>38.5 degrees ) and LDH (>1,500 IU/l) and/or reduction of urinary flow (< 1.5 ml/kg.h) were indicative of renal damage: in these cases dopamine was administered and CDUS performed. If renal blood flow was normal on CDUS, dopamine was administered for another 2 days; if a vascular thrombosis was detected, fibrinolytic agents (urokinase 4,000 IU/kg bolus and then 4,000 IU/kg.h for 24 h) were utilized. Between May 1997 and June 2000, 29 children (9 F, 20 M, aged) 6-72 months (median 39) affected by grade II inoperable, grade III, and grade IV NB underwent these preventive procedures at the time of surgical excision of the mass after chemotherapy 23 had an uneventful p.o. course. CDUS at 7 days was normal in 22; in 1 (polar resection at operation) it demonstrated a slight reduction of blood flow in the renal cortex. For all 23 patients, the CDUS at 3, 6, and 12 months was negative. Three patients showed a single altered parameter; the CDUS p.o. and during follow-up did not show any alteration. The other 3 patients had two or more altered parameters and were treated with dopamine: in 1 the initial CDUS was normal, but it later showed moderate hypotrophy; in 2 patients a vascular thrombosis was documented. Treatment with urokinase was successful in only 1 patient who had a normalized CDUS after 3 months. The 2nd child developed renal failure and needed hemodyalisis. In our experience, elevation of serum LDH and T and/or oliguria were good indicators of renal impairment. CDUS was also useful in detecting renal blood flow reduction and artery occlusion. These preliminary results show these intra- and postoperative measures to reliably avoid renal impairment in these children. Long-term follow-up could confirm the efficacy of these methods.

Lung volume reduction surgery in lieu of pneumonectomy in an infant with severe unilateral pulmonary interstitial emphysema.

A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.

Central venous access in pediatric patients.

Occlusion of traditional sites for central venous cannulation is a challenging problem in patients that require a permanent central venous line for chronic administration of nutrients or drugs. In rare cases, extensive central venous thrombosis of the superior and inferior vena cava may preclude catheterization, and uncommon routes should be used. We describe our approach for placement of chronic central venous lines in two pediatric patients with short bowel syndrome and extensive caval occlusion.

Vascular access in patients affected by short bowel syndrome.

The aim of this study was to evaluate the problems induced by the use of a central venous catheter (CVC) in a series of patients with short bowel syndrome observed at the university of Padua (Italy) between January 1981 and June 1997. During this period, 14 patients required central venous access for parenteral nutrition (PN); 55 catheters were inserted, 6 with percutaneous and 49 with surgical techniques. We divided the patients into two groups according to PN duration. The first group includes 11 children with short/medium-term PN 8 are now eating, and 3 died from respiratory failure) while the second group includes 3 patients on home long-term PN. Thirteen catheters were placed in the first group, and the mean PN duration was 173 days; the three patients on home PN required 42 catheters. It is our experience that the use of catehters in patients requiring short/medium- term PN is a safe procedure with few complications; patients on home long-term PN present an increasing number of complications, and the vascular access could become a serious problem as the number of PN-dependent increases.

[Steroid therapy of hemangioma in children]. / Considerazioni sulla terapia steroidea degli emangiomi infantili.

Hemangiomata is the more frequent pediatric tumor, absent at birth and usually growing, sometimes very quickly, during the first week of life. It potentially represents a serious problem because of his unforeseeable evolution during the time: is it more useful to use a corticosteroid therapy or to wait for the involution of the tumor? Here we have the results of the treatment on 51 babies who presented with hemangiomas of various sites: 52.6% of the lesions were on the face and head, 13.4% on chest wall, 10% on upper limbs, 4% on abdominal wall, 10% on pelvis region and 10% multiple. Therapy was compression in 10% of patients, corticosteroid injection in 66%, oral administered corticosteroid in 12%, surgery post corticosteroid therapy in 11.5%, others in 2.5%. Best results were obtained on the lesion of the head and face (good in 96%), worst in those of the abdomen (any modification in 100%).

[Phimosis: when does it require surgical intervention?]. / Fimosi: quando è patologia di interesse chirurgico?

One of the most recurrent questions in pediatric age is the phimosis; this is a frequently underestimated problem and its resolution often cause a lot of discomforts in babies and parents. In many countries it has been treated on the roots of ancient sanitary measures (circumcision) whereas in others it's routinely treated with painful and useless maneuvers; instead, until three years of age this don't represents a problem but the normal situation of the baby. Herein the authors report the results of topic corticosteroid treatment of fimosis in 83 patients aged 1-14 years, with good outcome in 86.7% of cases. Therapy and results are discussed.