Gastroschisis and exomphalos in Ireland 1998-2004. Does antenatal diagnosis impact on outcome?

Antenatal detection of anterior abdominal wall defects (gastroschisis and exomphalos) enables detailed prenatal planning and counselling with appropriate intrauterine transfer, delivery in a tertiary referral centre with prompt access to paediatric surgery and early surgical intervention. The authors believed that there was a relatively low rate of antenatal detection in Ireland and that an improved antenatal screening program would allow increased detection thus avoiding emergency retrievals from peripheral obstetric centres. Our hypothesis was that there was a significant difference in management and outcome in children with anterior abdominal wall defects detected antenatally and those detected at birth. All anterior abdominal wall defects in The Republic of Ireland are assessed and closed in two surgical centres, Our Lady's Hospital for Sick Children, and the Children's University Hospital, Dublin. A retrospective review of all admissions in both centres revealed 96 neonates with anterior wall defects (excluding bladder exstrophy and cloaca) over a 7 year period from 1998 to 2004 inclusive. Medical records, operative reports, neonatal databases and admission details were reviewed. The antenatal data search included anomaly detection, prenatal management plan and intrauterine transfer. Perinatal data included place of birth, weight, gestational age, mode of delivery, time to surgery and type of surgery, the time to establish full enteral feeding and the length of hospital stay were used as overall markers of outcome. Fifty-three patients had gastroschisis and 43 exomphalos with an antenatal detection rate of 53% (n = 28) and 34% (n = 15), respectively, with an overall detection rate of 44%. There was no significant difference in the median birth weight (2.83 vs. 2.85 kg), gestational age at birth (37 vs. 37 weeks), time to full feeding (12 vs.14 days) and length of stay (20 vs. 17 days) between those detected antenatally or postnatally, respectively. About 77 had a primary closure performed of which 63 infants had surgery within the first 24 h of life. There was no difference in the time to surgery, frequency of complications or the surgical outcome in either group. Intrauterine transfer did not affect any outcome measure assessed. The demographics and the presence of associated anomalies did not differ between the groups. The hypothesis that antenatal diagnosis in anterior abdominal wall defects improves outcome has been demonstrated to be false. Despite this result, the importance of antenatal screening and prenatal management of complex foetal conditions with consultation with experienced paediatric and neonatal staff is without doubt.

Minilaparotomy cholecystectomy in children.

AIM: Scarcity of paediatric cholelithiasis limits the obtainable experience in paediatric laparoscopic cholecystectomy (LPC). We report minilaparotomy cholecystectomy (MLC) in children which may be a useful alternative to LPC avoiding the added cost of disposable laparoscopic equipment. METHODS: Nineteen cases underwent a retrograde MLC using a 2-4 cm transverse right upper quadrant incision. Various parameters were assessed. RESULTS: Mean age at presentation was 10.87 years, mean length of the incision 2.8 cm, mean operative time 36.5 min, mean analgesic requirement 2 days, mean time to feed/ mobilize 1 day each, and mean duration of hospital stay was 2.7 days. All had satisfactory cosmetic result without postoperative complications. CONCLUSIONS: MLC in children involves a small incision, excellent cosmetic scar and rapid postoperative recovery at relatively low cost. It is a good alternative to LPC.

Abdominopelvic mesh compartmentalization reduces the complications of radiotherapy in children: a preliminary report.

AIM: The aim of this report was to assess the effects of radiotherapy (RT) in children with abdominopelvic tumours in whom a biodegradable (Polyglactin 910) mesh had been inserted prior to commencement of radiotherapy. METHODS: Retrospective analysis was carried out of four patients with abdominopelvic tumours who underwent radiotherapy between 2000 and 2002 as part of their management. RESULTS: All children tolerated radiotherapy well with no evidence of acute or chronic radiation enteritis. One child developed prolonged postoperative ileus and a second child developed infective diarrhoea and fever, not related to radiation. CONCLUSION: We have highlighted a good tolerance of radiotherapy in children following the insertion of a Polyglactin 910 mesh prior to starting radiation and would recommend further larger studies with longer follow-up to support this.

The SIS extracellular matrix scaffold-preliminary results of use in congenital diaphragmatic hernia (CDH) repair.

There is no ideal bio-tensile material for tissue replacement in paediatric surgical conditions. A variety of materials have been described to replace or reinforce tissue deficits in congenital diaphragmatic hernia (CDH). The problems with these materials range from extensive surgery to body wall deformity and hernia recurrence. The ideal graft would be safe, strong, have the potential to grow and not require replacement, not affect chest wall development or produce deformity, and have minimal risk of recurrence. Surgisis is a biomaterial composed of porcine intestinal submucosa that provides inherent tensile strength as a collagen lattice and is ultimately replaced by native collagen tissue. Our objective was to prospectively assess the utility of Surgisis as a replacement graft in a cohort of 10 children undergoing primary or recurrent CDH repair. The graft was well tolerated, and there was no recurrence of herniation. We conclude that Surgisis collagen mesh may be a useful alternative for tissue replacement or reinforcement in difficult cases of CDH and diaphragmatic agenesis. The biological framework of Surgisis may also prove useful in other situations of complex tissue loss in children.

Gastric stromal tumors in children.

Gastrointestinal stromal tumors (GIST) in children are rare and their behavior has been regarded as difficult to predict on pathological criteria. We report our experience with two gastric GISTs in children aged 10 and 11 years. Both remain alive and free of disease at 5 years and 2 years respectively. Comparison of the pathological features in the resected specimens with a recently proposed guidelines for predicting outcome in this group of tumors is reported.

Glutathione S-transferase: a potential new marker of intestinal ischemia.

BACKGROUND/PURPOSE: The accurate and early diagnosis of intestinal ischemia remains difficult chiefly because of a lack of a suitable marker that is noninvasive and easy to use. The glutathione S-transferases (GST) are a family of cytosolic enzymes involved in detoxification and released from a variety of cells when the cell membrane is damaged. The enzymes are distributed widely in the intestine and show isoform specificity in their distribution throughout the intestinal tract. Several previous reports have shown the utility of these enzymes in the diagnosis of liver and renal graft damage during and after organ transplantation. The object of this study was to determine if GST levels correlated with histologic changes of intestinal ischemia in a controlled animal model of mesenteric intestinal ischemia. METHODS: Control and experimental male Sprague-Dawley rats underwent laparotomy and ligation of the Superior Mesenteric Artery (SMA) and both control and experimental animals were studied at 30, 60, 90, 120, and 240 minutes. Blood taken from the Inferior Vena Cava (IVC) and Portal Vein (PV) and jejunal and ileal perfusates were assayed for alpha and mu isoforms of GST using a commercially available enzyme immunoassay. In addition, jejunal and ileal segments were sampled and reviewed by a histopathologist blinded to the group being studied. RESULTS: A reproducible pattern of intestinal ischemia was noted with worsening grades of injury observed with greater ligation times. Luminal alpha and mu GST release (as measured by the appearance in luminal perfusate) increased with increasing ischemia times. Increased ischemia times resulted in increased levels of alpha and mu GST in both portal and systemic venous samples but lagged behind the appearance of raised luminal GST values. CONCLUSIONS: The results suggest that GST may be an interesting and useful marker in the early detection of intestinal ischemia. Its detection in peripheral blood has implications for a more detailed study design to determine the sensitivity and specificity of this marker in more diverse clinical conditions such as necrotizing enterocolitis and superior mesenteric artery occlusion.

Neonatal sigmoid volvulus: a complication of anal stenosis.

Sigmoid volvulus is an exceptionally rare cause of intestinal obstruction in neonates. Only 7 cases have been reported in the English and French literature. The authors report a recent case of sigmoid volvulus in a neonate secondary to anal stenosis and review the diagnosis and management of this serious condition. The authors believe that carefully performed radiologic reduction is the preferable alternative to surgical intervention.

Complex branchial fistula: a variant arch anomaly.

A 5-year-old boy presented with an infected left-sided branchial fistula. Despite antibiotic treatment and repeated excision of the fistula, purulent discharge from the wound persisted. Three-dimensional computed tomography (3D CT) reconstruction greatly facilitated the diagnosis and management of this case by showing the course of the fistulous tract. The complexity of the tract suggests that this represents a variant arch anomaly because it contains features of first, second, third, and fourth arch remnants.

Extracutaneous Sweet syndrome involving the gastrointestinal tract in a patient with Fanconi anemia.

Acute febrile neutrophilic dermatosis, or Sweet syndrome, is a cutaneous eruption characterized clinically by the appearance of painful red plaques and nodules and histologically by an intense dermal neutrophilic infiltrate. Extracutaneous manifestations are rare. We report a patient in whom otherwise typical cutaneous Sweet syndrome was accompanied by an extracutaneous manifestation in the ileum.

Xanthogranulomatous pyelonephritis in childhood.

BACKGROUND: Xanthogranulomatous pyelonephritis is a severe, atypical form of chronic renal parenchymal infection accounting for 6/1000 surgically proved cases of chronic pyelonephritis. Its manifestations mimic those of neoplastic and other inflammatory renal parenchymal diseases and, consequently, it is often misdiagnosed preoperatively. AIM: To examine the relation between clinical history and the results of renal investigations performed in children with xanthogranulomatous pyelonephritis. METHOD: A retrospective review of 31 cases presenting with the histopathological diagnosis of xanthogranulomatous pyelonephritis between 1963 and 1999. RESULTS: The mean follow up was 8.2 years. The male:female ratio was 1:1.1. The left kidney was affected in 26 of the 31 patients. The positive findings on examination and investigation at presentation were: fever, 16 children; pyuria, 26 children; positive urine culture, 16 children. A haemoglobin of < 100 g/l was measured in 27 of 31 patients and 15 of 18 patients tested had a raised erythrocyte sedimentation rate of > 20 mm in the first hour. Twenty six children had renal calculi, with a large reduction in the function of the affected kidney on isotope scintigraphy in 27 of the 29 patients tested. Hypertrophy of the contralateral kidney, shown on imaging, was present in 17 of 31 patients. CONCLUSIONS: Increasing awareness of this condition should lead to the diagnosis being suspected preoperatively.

In-situ emergency paediatric surgery in the intensive care unit.

The role of surgery in the intensive care unit (ICU) remains unclear. Although previously shown not to increase morbidity for patent ductus arteriosus ligation, Broviac catheter insertion, and recently, general neonatal and paediatric surgery, there remains a reluctance to operate on sick patients in the ICU (in-situ surgery, ISS). A retrospective study of 25 critically ill children and neonates who underwent ISS was performed. Surgery was aided by operating loupes and a high-intensity headlight. ISS was not associated with any morbidity, and although a 36% mortality occurred in this small series, in no case was this due to ISS. ISS avoids the risks of transfer to the operating theatre and the potential delays in theatre access. Our results suggest that ISS in a tertiary-level paediatric surgical hospital is safe and does not impact adversely on clinical outcome.

External carotid artery ligation for life-threatening hemorrhage in exsanguinating orbital facial congenital hemangiopericytoma.

A 7-week-old infant with a locally invasive, orbital, congenital hemangiopericytoma underwent emergency external carotid artery (ECA) ligation for exsanguinating hemorrhage from an intraoral biopsy site. ECA ligation was successful in controlling the life-threatening hemorrhage and in reducing tumor size and vascularity. The location and extensive nature of the tumor prevented primary excision. Preoperative adjuvant chemotherapy was unsuccessful in controlling tumor growth. After ECA ligation, with reduction in tumor bulk and blood supply, the tumor was radically excised. This technique has important implications in the management of patients with extensive hemangiopericytomas or sino-facial tumors previously regarded as unresectable and which present with life-threatening hemorrhage.

Infrared laser Doppler flowmeter in the determination of small bowel perfusion after ischemic injury: comparison with the clearance of locally generated hydrogen and fluorescein angiography.

The determination of small bowel perfusion after an ischemic insult is difficult. Regional perfusion was determined in an animal model of neonatal intestinal ischemia using the techniques of laser Doppler flowmetry and the clearance of locally generated hydrogen. Both methods reliably measured tissue perfusion in the areas of maximal ischemic injury. However, considerable variability, perhaps owing to motion artifact, was seen in areas of patchy necrosis. The results suggest that the laser Doppler flowmeter is a suitable technique to measure tissue perfusion in areas of maximal ischemia. However, efforts to reduce motion artifact will be necessary if the laser Doppler is to be used in tissue sites where blood flow is critical for safe anastomosis. This will be a subject of future study.

An inexpensive device for perioperative positioning of pediatric patients.

Intraoperative positioning of the pediatric patient during venous access procedures is crucial. Currently such patients are positioned with a sandbag beneath the shoulders and in a head-down position. This has significant limitations because the bag must be removed for subsequent on-table radiography. The authors have devised a simple inexpensive technique that obviates this need and facilitates the procedure. They have used the device in over 80 patients undergoing venous access. No problems relating to the device have been noted, and on-table x-rays are readily facilitated.

Emergency liver transplantation after Kasai portoenterostomy.

Three patients with stable liver function after Kasai portoenterostomy developed acute liver failure secondary to liver necrosis. Doppler ultrasound at presentation revealed reversed diastolic hepatic arterial blood flow. Two patients survived after urgent liver transplantation. Liver necrosis should be suspected in children with chronic liver disease presenting with fever and rapidly deteriorating liver function.

Orthotopic liver transplantation for acute hepatic failure in children.

Thirty children received 35 liver transplants for fulminant or late-onset liver failure between March 1988 and May 1993. Aetiology included non-A non-B hepatitis in 12, Wilson's disease in 8, drug-induced hepatic failure in 6, hepatitis B in 1, hepatitis A in 1, tyrosinaemia in 1 and congenital haemochromatosis in 1. Three patients were retransplanted, one each for hepatic artery thrombosis, non-A non-B graft reinfection, and chronic rejection. Two of these three patients received a third transplant for chronic rejection and hepatic artery thrombosis. One patient in the retransplant group survived. Overall, graft and patient survival at a mean follow-up of 17 months were 49% and 57%, respectively. Mortality was related to vascular complications in three patients (hepatic venous obstruction, portal vein thrombosis and hepatic artery thrombosis). Two patients died of primary sepsis (cerebral aspergillosis and cytomegalovirus (CMV) pneumonitis in association with graft-versus-host disease). Systemic sepsis and multiorgan failure was documented as a cause of death in four children and sepsis in association with chronic rejection in a further three patients. One child died of respiratory failure 4 weeks after transplantation. Mortality in eight children less than 2 years was 75% and this was significantly greater than for older children (P < 0.003, Mantel Cox). Earlier referral, even in the absence of a definitive diagnosis and particularly in children under 2 years is advisable and may improve survival.

Supportive care of the paediatric cancer patient.

Cytotoxic therapy for paediatric malignancy continues to be associated with significant mortality and morbidity. However, survival figures continue to improve despite increasingly toxic treatment protocols. Aggressive supportive care, more than any other modality, has produced a dramatic reduction in mortality during the past 30 years, and includes the appropriate management of infection, provision of adequate nutrition, and proper use of blood and blood products. The paediatric surgeon plays an integral role in the multidisciplinary team of cancer management and together with other team specialists is responsible for the planning of surgical intervention and also ensuring the safe passage of immunologically impaired patients through the additional stress of surgery. This paper details essential aspects of supportive care as commonly practiced in paediatric patients undergoing therapy and surgery.

Aortopexy for tracheomalacia in oesophageal anomalies.

Forty-eight patients with repaired congenital oesophageal anomaly underwent aortopexy for significant tracheomalacia between 1980 and 1990. Indications for aortopexy included recurrent apnoea/cyanosis in 31, near fatal episodes in 16, recurrent respiratory distress and infection in 20 and worsening stridor in 15. Gastro-oesophageal reflux was noted in 30 patients, recurrent fistula in 6 and oesophageal stricture in 14. Aortopexy cured near fatal episodes in all patients and resulted in improvement of airway obstruction in 95%. The procedure failed in 2 patients due to unrecognised bronchomalacia and phrenic nerve palsy respectively. Aortopexy is the primary procedure of choice for significant tracheomalacia when associated with near fatal episodes and significant airway obstruction.

The effect of two-stage orchiopexy on testicular growth.

Testicular growth was measured in 33 males who had undergone 2 orchiopexies on the developing testis. Multiple surgery on the abdominal or canalicular testis was associated with a high rate of testicular atrophy (40%) and a 46% decrease in testicular volume. A similar decrease (43%) in testicular volume was noted in those patients with an infracanalicular testis but no testis atrophied in this group. The results suggest that primary orchiectomy be considered in the high testis provided the contralateral testis is normal, or alternatively a Fowler-Stephens or microvascular transplant if it is not. Patients with an infracanalicular testis may undergo a careful second-stage orchiopexy. All patients with retarded testicular growth should perform routine self-examination.