Transition from paediatric surgery: how many patients do we need to plan for?

INTRODUCTION Transitional care is an NHS priority with newly published NICE guidance. Many paediatric surgical patients need quality care to continue into adulthood. We undertook an evaluation of our departmental activity to assess the magnitude of this issue. METHODS We identified all outpatients ≥ 15 years (potentially requiring imminent transition) seen over a 12 month period for all five general paediatric surgery consultants in our tertiary centre. Those patients requiring transition were highlighted and the appropriate adult team for referral recorded. RESULTS There were 2989 general paediatric surgery clinic appointments within the year; 289 (9.7%) were for young people aged 15 years or older; 62 patients (28% of those ≥ 15years) were deemed to require transition into adult care. Significantly more patients having colorectal surgery required follow-up (P = 0.0009 Chi-square test) compared with patients in other subspecialties. CONCLUSIONS More patients than expected required transition. This may be the case in other units. Current best practice includes time intensive preclinic planning, careful preparation of patient and family, followed by joint clinics. A joint clinic appointment takes 30 minutes, allowing for comprehensive handover and forging new relationships. In our department, we need at least ten transition clinics across 2 years. Coalition with adult colleagues is vital. These data enable us to plan services to provide quality care for our adolescent patients and highlights colorectal surgery as a priority.

Plasma ammonia response to incremental cycling and walking tests in COPD.

OBJECTIVE: It is well documented that plasma ammonia accumulates during exercise under conditions of metabolic stress. Metabolic stress (when skeletal muscle ATP supply fails to meet demand) occurs at low work rates during cycling in patients with COPD, but not been described during walking. Walking is an important activity for many patients with COPD and is commonly prescribed in pragmatic outpatient pulmonary rehabilitation programmes. In this study we explored whether metabolic stress occurs during incremental walking at the low work rates these patients achieve. METHODS: Twenty-nine subjects with stable COPD [mean(SD) age 68(7)years, FEV(1) 50(19)% predicted] performed maximal cardiopulmonary exercise tests on a cycle ergometer and treadmill. Plasma ammonia concentration was measured at rest, 1 and 2min of exercise, peak exercise and 2min recovery. RESULTS: Subjects achieved mean(SD) cycle work rate of 57(20)W with VO(2max) 15.5(4.6)ml/min per kg, and treadmill distance 284(175)m with VO(2peak) 16.8(4.2)ml/min per kg. Plasma ammonia concentration rose significantly (p<0.001) with walking [mean(SEM) change 24.7(3.8)micromol/l] and cycling [mean(SEM) change 35.2(4.3)micromol/l], but peak exercise ammonia was lower in walking (p<0.01). In a subgroup of subjects (n=7) plasma ammonia did not rise during either cycling or walking despite similar lactate rise and peak exercise indices. CONCLUSION: Our data indicate that failure of muscle ATP re-synthesis to meet demand and development of metabolic stress can occur during walking in COPD patients at the low work rates these patients achieve. This may therefore be a factor contributing to exercise limitation independent of ventilatory limitation.

The plasma ammonia response to cycle exercise in COPD.

The plasma ammonia response to exercise in chronic obstructive pulmonary disease (COPD) was examined and the relationship between plasma ammonia concentration and muscle adenine nucleotide metabolism was explored. In total, 25 stable COPD patients and 13 similar-aged controls underwent incremental and constant-work rate cycle exercise tests. Arterialised venous blood was sampled at rest, at 1-min intervals during exercise and

Adenine nucleotide loss in the skeletal muscles during exercise in chronic obstructive pulmonary disease.

BACKGROUND: Skeletal muscle adenine nucleotide loss has been associated with fatigue during high intensity exercise in healthy subjects but has not been studied in patients with chronic obstructive pulmonary disease (COPD). Changes in adenine nucleotides and other metabolites in the skeletal muscles were measured in patients with COPD and age matched healthy volunteers by obtaining biopsy samples from the quadriceps muscle at rest and following a standardised exercise challenge. METHODS: Eighteen patients with COPD (mean (SD) forced expiratory volume in 1 second 38.1 (16.8)%) and eight age matched healthy controls were studied. Biopsy samples were taken from the vastus lateralis muscle at rest and immediately after a 5 minute constant workload cycle test performed at 80% peak work achieved during a maximal incremental cycle test performed previously. RESULTS: The absolute workload at which exercise was performed was substantially lower in the COPD group than in the controls (56.7 (15.9) W v 143.2 (26.3) W, p<0.01). Despite this, there was a significant loss of adenosine triphosphate (mean change 4.3 (95% CI -7.0 to -1.6), p<0.01) and accumulation of inosine monophosphate (2.03 (95% CI 0.64 to 3.42), p<0.01) during exercise in the COPD group that was similar to the control group (-4.8 (95% CI -9.7 to 0.08), p = 0.053 and 1.6 (95% CI 0.42 to 2.79), p<0.01, respectively). CONCLUSIONS: These findings indicate that the ATP demands of exercise were not met by resynthesis from oxidative and non-oxidative sources. This suggests that significant metabolic stress occurs in the skeletal muscles of COPD patients during whole body exercise at low absolute workloads similar to those required for activities of daily living.

Mortality due to constipation and short-segment Hirschsprung's disease.

We report a case of mortality secondary to constipation. A 12-year-old child with a history of constipation from the age of 9 years presented in moribund condition with intestinal obstruction secondary to idiopathic constipation. He underwent laparotomy and died within 24 h of operation. At postmortem there was no evidence of abnormalities of the enteric nervous system. A 2nd case of early neonatal death from Hirschsprung's disease is presented to demonstrate that short-segment aganglionosis can be detected at postmortem. Enterocolitis can rapidly progress and be fatal in short-segment Hirschsprung's disease.

Small bowel volvulus due to giant mesenteric lipoma.

We report on a 14-year-old girl with acute intestinal obstruction due to volvulus of the small bowel caused by a giant mesenteric lipoma. To the best of our knowledge, this presentation of a mesenteric lipoma is unknown in the previous paediatric literature.

Gastroschisis: determinants of neonatal outcome.

This retrospective study elicits information regarding the dependence of neonatal outcome in gastroschisis upon: (1) the mode of delivery, (2) place of birth, (3) time for birth to surgery, (4) method of closure, (5) time from operation to commencement of first enteral feeds. The neonatal intensive care database from five major tertiary centres was used to identify 181 neonates with gastroschisis from 1990 to 2000. There were 8 deaths. There were no significant differences in outcome for infants delivered vaginally (102) versus Caesarean section (79), those born near the tertiary centre (133) as compared to infants born away (48), ones operated within 7 hours (125) compared with those operated after 7 hours (56), with delayed closure (30) versus primary closure (151). Neonates fed within 10 days of operation (85) had significantly lower incidence of sepsis, duration of TPN and hospital stay when compared to those fed after 10 days (96). Early commencement of feeds decreases the incidence of sepsis, duration of total parenteral nutrition (TPN) and hospital stay. Place of delivery, mode of delivery, time to surgery and type of closure do not influence neonatal outcome.

Hirschsprung's disease: the Australian Paediatric Surveillance Unit's experience.

Hirschsprung's disease (HD) was introduced into the Australian Paediatric Surveillance Unit (APSU) system in 1996 with the objective to collect data on demographics, incidence, family history, associated anomalies, clinical features, investigation, and surgical treatment in Australia. Children under 15 years of age with HD (confirmed by biopsy) were entered into a database maintained at The Children's Hospital at Westmead in Sydney. Nationwide, 127 children with HD were reported from January 1997 to December 2000, and near-complete information was available on 126. Neonatal presentation was seen in 114 cases (90%), whereas 12 (10%) presented post-neonatally. There was a history of delayed passage of meconium (more than 24 h) in 65 of the 114 patients (57%) who presented in the neonatal period. In those presenting post-neonatally there was no history of delayed passage of meconium. Primary pull-through was performed in 42 of the 82 patients (51%) operated upon. The Soave procedure was performed in 53 of the total 82 (65%) operations and 29 of 42(69%) primary pull-through operations. Pre-operative enterocolitis was seen in 15 of 126 (12%) and postoperative enterocolitis in 17 of 82 (21%) children. The majority of children with HD are diagnosed in the neonatal period (90%). Primary pull-through is becoming more popular (51%). The Soave procedure is the most commonly performed operation (65%) in Australia. Pre- and post-operative enterocolitis is still a significant problem.

Duodenal atresia with vascular rings and subglottic stenosis.

This case report of duodenal atresia associated with a vascular ring and subglottic stenosis raises some interesting issues of management.

Lung volume reduction surgery--a comparison of the long term outcome of unilateral vs. bilateral approaches.

OBJECTIVE: Bilateral lung volume reduction surgery (LVRS) is thought to be preferable to unilateral surgery due to greater initial benefit but the subsequent rate of decline may also be greater. We compared the long term physiological and health status outcome of LVRS performed on one or simultaneously on both lungs. METHODS: Prospective data were collected on a consecutive series of 65 patients undergoing LVRS who were all suitable for bilateral surgery. Twenty-six patients: age 59 (8) years underwent bilateral LVRS by video-assisted thoracoscopy (VAT) or sternotomy and 39 patients: age 60 (6) years underwent unilateral VAT. The perioperative effects of LVRS on spirometry were prospectively recorded at 3, 6, 12 and 24 months. RESULTS: The unilateral group had similar preoperative lung volumes to the bilateral patients: forced expiratory volume in 1s (FEV(1)) 26 vs. 30% predicted, RV 275 vs. 246% predicted and total lung capacity (TLC) 148 vs. 142% predicted. Unilateral LVRS was associated with significantly lower weight of lung resected: 80 (31) vs. 118 (46) g; hospital stay: 16 (10) days vs. 28 (22) days. Thirty-day mortality was 3% in the unilateral and 8% in the bilateral group (P=0.34). Postoperative ventilation occurred in 5% in the unilateral and in 42% in the bilateral group (P=0.0002). The decline of FEV(1) during the first postoperative year was significant in the bilateral group (-313 ml/y, P=0.04) but not significant in the unilateral group (-50 ml/y, P=0.18). SF 36 scores in all eight domains were similar in both groups preoperatively and at any postoperative interval. CONCLUSION: We have found no benefit from bilateral simultaneous LVRS and prefer unilateral LVRS because of the lower morbidity, resulting in earlier discharge, and slower decline in physiological benefit.

Excision duodenoplasty: a new technique for congenital duodenal obstruction.

Slow anastomotic function is a common problem in the management of congenital duodenal obstruction. We describe a simple technique of excision duodenoplasty (ED) that results in a fixed open anastomosis, which facilitates early commencement of feeds and discharge from hospital. A retrospective case-note review (1981-2000) was undertaken to compare the results of ED with side-to-side duodenoplasty (SSD) and diamond-shaped anastomosis (DD). The outcome measures were days to commencement of feeds, duration of total parenteral nutrition (TPN), and length of hospital stay. In ED a 1.0 to 1.5-cm elliptical segment of dilated duodenum is excised. A longitudinal incision is made in the smaller distal duodenum. A side-to-side one-layer anastomosis is performed. The wall of the proximal duodenal stump is thick, and excision of the ellipse keeps the anastomosis open by preventing apposition of the opposing walls. This facilitates good drainage across the anastomosis. The time to onset of feeding was longer after SSD (median 7 days) compared to ED (5 days) and DD (5 days). The total duration of TPN was also longer for SSD (9 days) in comparison to ED (7 days) and DD (7 days). Although the duration of hospital stay was more for SSD (18 days) than ED (13 days) and DD (16 days), this difference was not statistically significant. The ED technique thus gives an equally good result as DD and a better result than SSD. It is simple to perform and to teach.

How to test the safety of homemade antegrade colonic washout fluid?

Normal saline is commonly used for antegrade colonic washout enemas. The saline solution is usually made at home by adding measured quantities of common salt to tap water. The best method for the carers to check the concentration of salt in these homemade solutions has not been reported. Various concentrations of saline solutions were tested for specific gravity (SG) with an aquarium hygrometer and urine analysis sticks by two blinded independent observers. An aquarium hygrometer can read SG with an accuracy of 0.00046 for saline solutions made in tap water and 0.00051 for those made with sterile water. Urine analysis dipsticks sticks failed to achieve this accuracy. An aquarium hygrometer is a simple and reliable way to measure the SG of homemade saline solutions. Its use may avoid the problems associated with over- or under-concentration of salt in the antegrade enema solution.

Familial presacral masses: Screening pitfalls.

BACKGROUND/PURPOSE: Anterior sacral meningocele (ASM) and presacral teratoma (PT) are related malformations. Both can have familial occurrence. Surgical treatment of occult cases is essential. Hence, it is important to screen the asymptomatic relatives. Plain radiograph of the sacrum to look for any bony abnormalities is the usual accepted screening modality. The authors report ASM and PT in siblings (with positive family history) with no sacrococcygeal bony abnormality on imaging. Both the siblings had presacral mass on imaging. METHODS: The medical records of 2 siblings with ASM and PT were reviewed for the clinical presentation, diagnostic workup, and family history. RESULTS: One sibling was symptomatic, whereas the other was completely asymptomatic. A family history of sacral bony abnormalities and anterior meningoceles was present in father, 2 paternal cousins, paternal uncle, paternal aunt, paternal grandmother, and paternal grand uncle. CONCLUSIONS: These case reports suggest that imaging for screening for ASM and PT should be directed at identifying the presacral mass rather than sacral bony defect. Hence, computed tomography or magnetic resonance imaging looking for presacral mass rather than plain radiograph should be the screening modality. J Pediatr Surg 36:1841-1844.

Gastroschisis reduction under analgesia in the neonatal unit.

PURPOSE: The authors present their experience of reduction of gastroschisis in infants in the ward with analgesia and without anesthesia. METHODS: Prospective data were collected on 35 infants born with gastroschisis from 1999 to 2001. Ward reduction (WR) was not attempted in infants with obvious gut atresia, perforated or ischemic bowel, or systemic instability. RESULTS: Ward reduction was attempted in 29 of the 35 infants and was successful in 25. There were no deaths or major morbidity in those that underwent ward reduction. Ventilation was required in 2, and line sepsis occurred in 4. Umbilical hernia was seen in 7; all but 1 were treated conservatively. CONCLUSIONS: In gastroschisis, ward reduction under analgesia is safe if strict selection criteria are adhered to. Postreduction ventilation is avoided in the majority.

Effectiveness of primary endoscopic incision of ureteroceles.

Endoscopic incision of a ureterocele (EIU) is simple when compared to an open procedure such as ureterocele excision with or without an upper-pole nephrectomy. It has, however, the potential to induce vesicoureteric reflux (VUR), which traditionally requires further surgical intervention. The natural history of the VUR that develops following EIU is not known. We have treated asymptomatic VUR that developed following EIU conservatively and have surgically intervened only in cases with recurrent urinary tract infections (UTI). This is a review from a single surgeon's practice involving 29 consecutive cases of ureterocele spread over a period of 4 years. The range of follow up was 4-54 months (median 32). Of the 24 children who underwent primary EIU, 6 required a second procedure, 3 a reincision and 3 an open procedure. Of the 3 who had a reincision, 2 required a further open procedure. The indication for reincision was failure of decompression of the upper tract and the indication for an open procedure was recurrent UTI following EIU. Thus, overall success was achieved in 19 of 24 cases of primary EIU (79.2%). VUR following EIU appeared in 10 cases (41%); UTI developed in only 5 (50%) of these 10 cases. Overall, UTI developed in 6 of the 24 (25%) cases of primary EIU. Eight children had an open procedure (3 as a primary procedure and 5 after EIU); 2 (25%) from this group had UTI following the procedure, and interestingly, neither had VUR. Ureterocele incision is thus a good alternative to upper-pole nephrectomy or excision of the ureterocele, especially in infancy. There is an inherent risk of producing VUR in the postincision period, however, the majority of cases can be managed conservatively. All patients need to be monitored for hypertension and UTI following EIU.

Antenatal prediction of hypertrophic pyloric stenosis.

This is the first reported case where the diagnosis of hypertrophic pyloric stenosis (HPS) was entertained in the antenatal period and the neonate was followed up in the postnatal period on a prospective basis until the HPS became manifest.

A new technique of anastomosis to avoid stricture formation in oesophageal atresia.

Anastomotic stricture is a common problem following repair of oesophageal atresia (OA). We describe a technique of oesophageal anastomosis that may prevent this problem. A horizontal incision is placed on the anterior hemicircumference of the upper pouch approximately 0.5 cm proximal to its blind ending to raise a flap. A corresponding vertical incision is made at the open end of the lower pouch to spatulate it. The flap from the upper pouch is laid into the open V of the lower pouch. This creates a wide anastomosis, and the suture line is not restricted to one plane. In 11 cases of OA, oesophageal continuity was established with this technique over a period of 10 years. Only 1 child developed an anastomotic stricture, which responded to a single dilatation. Two patients required Nissen's fundoplication for a distal oesophageal stricture. In neither of the patients did the anastomosis become stenotic. The technique described here is simple and effective. A suture line is created that is long and not in a single plane. This minimises the risk of stricture formation.

Insertion of an implantable venous access device in the groin using inferior epigastric vein.

BACKGROUND: Patients with cystic fibrosis (CF) need reliable venous access that can be provided by implantable venous access devices (IVAD). Such IVADs usually are placed in the upper part of the body, but placing them in this area has 3 disadvantages: a suitable vein may not be available, the portal may be conspicuous, and there may be interference with chest physiotherapy. Positioning the IVAD in the groin by using the inferior epigastric vein (IEV) is an answer to these problems. METHODS: This is a review of 29 patients from a single surgeon's practice from 1984 to 1999. A groin incision was used to implant the IVAD and to introduce the catheter via the IEV to the inferior vena cava. RESULTS: A total of 33 IVAD were inserted in 29 patients (27 with CF). The average age at first operation was 12.4 years. Infection was seen in 5. Venous thrombosis was not seen in any patient. The average longevity of IVAD is 18.5 months. Total experience is 87 IVAD years. CONCLUSION: This is a useful route of first choice for CF patients, with an overall complication rate comparable with techniques in which IVADs are placed in the upper half of body. J Pediatr Surg 36:579-581.

Adverse outcome of congenital diaphragmatic hernia is determined by diaphragmatic agenesis, not by antenatal diagnosis.

BACKGROUND/PURPOSE: The authors studied their congenital diaphragmatic hernia (CDH) cases retrospectively to ascertain if classical CDH and diaphragmatic agenesis (DA) have separate clinical manifestations, whether antenatally diagnosed cases behave differently from those not diagnosed antenatally, and if antenatal diagnosis before 25 weeks carries a worse prognosis. METHODS: The authors performed a retrospective review of 23 infants with CDH treated at their institution between January 1996 and March 1999. The patients were divided into 3 groups that were analyzed: DA and classical CDH, antenatally diagnosed and nonantenatally diagnosed, and antenatally diagnosed before 25 weeks and after 25 weeks. RESULTS: There were 8 cases of DA and 11 cases of classical CDH. Four infants died without operation and could not be classified. Neonates with DA had significantly longer mean duration of preoperative stabilization compared with classical CDH (5.25+/-2.76 days v 1.36+/-1.0 days) and postoperative mechanical ventilatory support (15.7+/-3.0 days v 4.9+/-3.0 days). Fifty percent of DA patients died; all classical CDH patients survived. Twelve cases were diagnosed antenatally, 6 before 25 weeks' gestation. Antenatally diagnosed cases had no statistically significant difference in mortality rates from those not diagnosed antenatally; 50% of those diagnosed before 25 weeks survived. CONCLUSIONS: DA cases require more preoperative preparation and postoperative ventilation and have a bad prognosis compared with classical CDH. Antenatal diagnosis of CDH does not convey a different prognosis. Fifty percent of CDH patients with antenatal diagnosis before 25 weeks survive.