Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood.

BACKGROUND: Surgery for total colonic aganglionosis (TCA) is designed to preserve continence and achieve satisfactory quality of life. This study evaluated a comprehensive group of clinical and social outcomes. METHODS: An international multicentre study from eight Nordic hospitals involving examination of case records and a patient-reported questionnaire survey of all patients born with TCA between 1987 and 2006 was undertaken. RESULTS: Of a total of 116 patients, five (4·3 per cent) had died and 102 were traced. Over a median follow-up of 12 (range 0·3-33) years, bowel continuity was established in 75 (73·5 per cent) at a median age of 11 (0·5-156) months. Mucosectomy with a short muscular cuff and straight ileoanal anastomosis (SIAA) (29 patients) or with a J pouch (JIAA) (26) were the most common reconstructions (55 of 72, 76 per cent). Major early postoperative complications requiring surgical intervention were observed in four (6 per cent) of the 72 patients. In 57 children aged over 4 years, long-term functional bowel symptoms after reconstruction included difficulties in holding back defaecation in 22 (39 per cent), more than one faecal accident per week in nine (16 per cent), increased frequency of defaecation in 51 (89 per cent), and social restrictions due to bowel symptoms in 35 (61 per cent). Enterocolitis occurred in 35 (47 per cent) of 72 patients. Supplementary enteral and/or parenteral nutrition was required by 51 (55 per cent) of 93 patients at any time during follow-up. Of 56 responders aged 2-20 years, true low BMI for age was found in 20 (36 per cent) and 13 (23 per cent) were short for age. CONCLUSION: Reconstruction for TCA was associated with persistent bowel symptoms, and enterocolitis remained common. Multidisciplinary follow-up, including continuity of care in adulthood, might improve care standards in patients with TCA.

ANTECEDENTES: La cirugía de la aganglionosis colónica total (total colonic aganglionosis, TCA) está diseñada para preservar la continencia y lograr una calidad de vida satisfactoria. Este estudio evaluó un gran número de resultados clínicos y sociales. MÉTODOS: Se realizó un estudio internacional multicéntrico en ocho hospitales nórdicos en el que se incluyeron las historias clínicas de todos los pacientes nacidos con TCA entre 1987 y 2006. Se invitó a los pacientes y a sus cuidadores a responder una encuesta sobre la función intestinal, el crecimiento y las necesidades nutricionales, así como la repercusión social de la enfermedad a largo plazo. RESULTADOS: De un total de 116 pacientes, 5 (4,3%) habían fallecido y 102 respondieron la encuesta. Con una mediana de seguimiento de 12 años (rango 0,5-33), se había restablecido la continuidad intestinal en 75/102 (74%) a una mediana de edad de 11 meses (0,5-156). La mucosectomía con un manguito muscular corto y anastomosis ileoanal directa (short muscular cuff and straight ileo-anal anastomosis, SIAA) n = 29 o con reservorio en J (JIAA) n = 26 (55/72, 76%) fueron las reconstrucciones más habituales. Las complicaciones postoperatorias precoces que precisaron una intervención quirúrgica fueron muy poco frecuentes, pero se presentaron en 4/72 (5%) pacientes. Los síntomas a largo plazo relacionados con la función intestinal tras la reconstrucción, valorados en 57 niños mayores de cuatro años, fueron la dificultad para retener la defecación en 14 (25%), la encopresis en 21 (37%), las pérdidas fecales > 1/semana en 9 (16%) y el aumento de la frecuencia de defecación en 51 (89%). A largo plazo, se desarrolló una enterocolitis en 35/72 (47%) pacientes. Se precisó de nutrición enteral y/o parenteral suplementaria en algún momento del período de seguimiento en 51/93 (55%) pacientes. En los pacientes que respondieron a la encuesta entre 2 y 20 años (n = 56) de edad, se detectó un índice de masa corporal menor en 20 (35%) y una altura baja en 13 (23%) para su edad. En 35/57 (61%) pacientes mayores de 4 años con restauración del tránsito intestinal había restricciones sociales debido a los síntomas intestinales, de los que en 10 (17%) casos fueron moderadas o graves. CONCLUSIÓN: La reconstrucción de una TCA se asocia con síntomas intestinales persistentes y la enterocolitis sigue siendo frecuente. Un seguimiento multidisciplinario, incluso en la edad adulta, podría mejorar los resultados en la cirugía de la TCA.

Transanal endorectal vs. Duhamel pull-through for Hirschsprung's disease.

INTRODUCTION: The aim of this study was to test the hypothesis that the early functional outcome for patients with rectosigmoid Hirschsprung's disease (HD) is comparable for the Duhamel pull-through procedure and the transanal endorectal pull-through (TERPT) procedure, with less discomfort for the patient postoperatively after the TERPT technique. MATERIAL AND METHODS: Eleven patients operated on with the TERPT technique (T Group) were prospectively registered and compared retrospectively with 18 patients operated on with the Duhamel pull-through (D Group). Data recorded included patient demographics, operative treatment, complications, hospital stay and bowel functions. The follow-up time was limited to 24 months. RESULTS: The T Group started oral feeding sooner, their bowel movements started sooner and they had less need for analgesia postoperatively and a significantly shorter hospital stay. 71% of the patients in the D Group needed re-intervention compared to only 18% of the T Group. Enterocolitis was seen in two patients in both groups. At the last clinical control ten patients had constipation (59%) and three had soiling (18%) in the D Group. Three patients in the T Group had constipation (27%) and one had soiling (9%). CONCLUSION: Our results support the use of the TERPT method rather than the Duhamel pull-through for rectosigmoid HD.

Continuous double U-stitch gastrostomy in children.

BACKGROUND: In children, a gastrostomy button was placed as the initial feeding tube, using laparoscopy and a modified surgical technique. The aim of this study was to test the hypothesis that a new surgical procedure developed at our institution would result in fewer postoperative complications. PATIENTS AND METHODS: Sixty-two consecutive children with nutritional problems underwent a video-assisted gastrostomy operation (VAG). The technique requires the use of a 2 or 3 mm laparoscope optic and a 5 mm trocar placed at the exit site chosen for the gastrostomy. A continuous double U-stitch absorbable suture created a purse string suture around the gastrostoma on the stomach and fixated the stomach to the abdominal wall. For comparison, we used a control group of 68 children with nutritional problems operated on with our previously published VAG technique. After surgery, the children were followed up at one and six months and all complications were documented according to a protocol. RESULTS: The two groups of children were comparable with regard to their demographic data. There were no serious intra-operative or postoperative intra-abdominal complications requiring reoperation. There was a significantly lower incidence of the minor complication of granuloma around the gastrostoma in the study group compared with the control group. CONCLUSION: This variation of the surgical technique is simple and effective. It allows primary placement of a gastrostomy button that is functionally and cosmetically comparable to a gastrostomy tube surgically placed by other methods. In this study, the patients had fewer postoperative problems than the control group.

Paediatric robotic surgery in clinical practice: a cost analysis.

BACKGROUND: Since 2006 we have used robotic assistance when performing minimally invasive laparoscopic fundoplications in children. We compared the costs of robotic surgery with the costs for open and laparoscopic surgery to test our hypothesis that the increased costs of the new technology are acceptable. METHOD: Costs were calculated using the regional hospital prices for our first 14 fundoplications in children, performed with the aid of the da Vinci Surgical System from Intuitive Surgical. We compared these costs with those of our ten latest fundoplications performed using open and laparoscopic surgery, respectively. There were no differences in the demographic data, work-up or indications for surgery between the three groups of children. RESULTS: The mean cost of robotic surgical fundoplications (EUR 9 584) was 7% higher than the mean cost of laparoscopic surgery (EUR 8 982) and 9% lower than the mean costs for open surgical procedures (EUR 10 521). These differences can be explained by the increased cost of robotic instruments (EUR 2 081 per operation). The duration of the operation and the duration of in-hospital stay are comparable to those of laparoscopic surgical interventions. The time required for the operative intervention was considerably longer than for the open surgical procedure; the duration of the in-hospital stay was only half of that of the open surgical procedure. The patients seemed to benefit from the use of robotic instruments with less morphine (as a marker of less postoperative pain) and a shorter hospital stay. CONCLUSION: The introduction of robotic assistance into surgical practice involves increased in-hospital costs, mainly because of the cost of the new instruments. This increase in cost can be offset by the shorter hospital stay compared to open surgery. After laparoscopic surgery the hospital stay is about the same as after operations performed with robotic assistance. Cheaper instruments and shorter operating time will make robotic surgery cost efficient in the future. The benefit for the patients is less trauma due to the use of minimally invasive surgery and a shorter hospital stay. Thus, the higher initial costs may be considered worthwhile.

Morgagni hernia repair in a small child using da Vinci robotic instruments--a case report.

BACKGROUND: The recently introduced use of robotic surgery in minimally invasive surgery procedures facilitates several steps in the operative procedure. We report the first case of a robot-assisted laparoscopic repair of a Morgagni hernia using the da Vinci Surgical System from Intuitive Surgical (Sunnyvale, CA, USA) in a 7.8 kg 18-month-old child. METHODS: Four trocars were used to gain access to the abdomen. The robot-enhanced instruments were used to close the hernia defect with interrupted, absorbable sutures, using intracorporeal knot tying. RESULTS: The operation was completed laparoscopically without a patch. The total setup time for the robotic system was 35 minutes including draping. The operating time at the robotic console was 80 minutes. The child tolerated an oral intake the day after surgery and was discharged home on the third postoperative day. CONCLUSION: Robot-assisted laparoscopic Morgagni hernia repair is feasible in small children.

48-hour wireless oesophageal pH-monitoring in children: are two days better than one?

BACKGROUND: Use of a catheter-free, radio telemetric, oesophageal pH-monitoring system in paediatric clinical practice allows patients to follow a more normal physiological pattern of activities and causes less discomfort. At our institution, placement of the capsule is done under general anaesthesia, which restricts the child's activity during the first day. The aim of this study was to determine whether oesophageal pH-measurements should be performed over 48 hours or whether 24-hour measurement provides sufficient and reliable results. CHILDREN AND METHODS: The study included 24 consecutive children with symptomatic gastro-oesophageal reflux problems who had undergone upper gastrointestinal endoscopies under general anaesthesia. The radio-transmitting Bravo capsule was introduced transorally and placed above the diaphragm at a width of two vertebral bodies. Oesophageal acid exposure was monitored via a portable receiver for 48 hours. The children's symptoms during measurements were registered. Wilcoxon signed rank test for paired samples was used after power analysis. RESULTS: The capsule was successfully attached to the oesophageal mucosa in all cases with minor technical problems in only one patient. The 48-hour pH-monitoring was completed in 23 patients. The median percentage time with an oesophageal pH of less than 4 was 5.4 +/- 6.8 for the first 24 hours and 5.8 +/- 7.4 for the 48-hour measurement. The DeMeester score was 20.5 +/- 23.7 and 22.2 +/- 25.7, respectively. CONCLUSIONS: Ambulatory pH-monitoring using the wireless system is feasible and safe. It was well-tolerated by the children. There was no statistical difference between the pH-measurements or DeMeester scores during the first 24 hours compared with the 48-hour measurements. Individual variations were noted but had no clinical significance except in two patients. Our results support the use of pH-measurement for a period of 24 hours only.

Transanastomotic feeding tube after an operation for duodenal atresia.

UNLABELLED: The aim of this study was to answer the question whether or not, after an operation for duodenal atresia, a transanastomotic feeding tube reduces the time to full preanastomotic feeding. The method used was a retrospective study and a prospective observation. 18 consecutive newborns with duodenal atresia, nine from each of two different centres of paediatric surgery, were studied retrospectively. The patients in one centre received a nasogastric tube and a transanastomotic feeding tube during the operation, while in the other centre only a nasogastric tube was used. Seven control patients with duodenal atresia treated postoperatively with a nasogastric tube and a transanastomotic feeding tube were prospectively observed. The main outcome measure used to compare these two groups was the time required to achieve full preanastomotic feeding. RESULTS: The patients who were treated postoperatively with the transanastomotic feeding tube needed significantly less time to achieve full preanastomotic feeding than those with a nasogastric tube only (P < 0.001, Mann-Whitney U test). CONCLUSION: The use of a transanastomotic feeding tube, after an operation for duodenal atresia, leads to earlier full preanastomotic feeding.

Stenting of the biliary tract in children.

We report on the technique and results of percutaneous transhepatic biliary drainage (PTBD) in children with obstructive jaundice. Three patients aged 8 - 15 years were treated, two of them for a benign and one for a malignant stricture. Endoscopic treatment was not possible and all the PTBD procedures were done under general anaesthesia. One of the children was treated with external-internal drainage, and the two others by insertion of a plastic endoprosthesis. There were no immediate complications. The PTBD had a good palliative effect in two cases, and in one case surgical treatment was necessary. We conclude that PTBD is a safe modality and that it can be used in children for the relief of obstructive jaundice.

Newborn infants' cry after heel-prick: analysis with sound spectrogram.

The aim of the study was to test the hypothesis that a newborn infant's cry can be used in conjunction with an instrument to measure pain. Crying due to pain was analysed after a heel-prick stimulus. In a prospective, descriptive study, 50 healthy newborn infants were subjected to a heel-prick for phenylketonuria screening. Their cries of pain were recorded and analysed. Duration of the crying sound was analysed and, using a sound spectrogram, the fundamental frequency and the cry melody of the first five cry sounds were analysed. The analysis showed that the crying sound after the painful stimulus of the heel-prick had a significantly higher fundamental frequency and lasted longer at the first than at the fifth cry. The first cry had a more varied crying melody than the fifth. There were large differences between individual cries from a single infant, as well as in the duration of each cry, total crying time, and fundamental frequencies between infants. While the first cry was more like a cry of pain, the fifth cry more resembled crying for reasons other than pain. The results suggest that newborn infants react to pain in a recognizable way. However, other stimuli may cause a similar reaction. Crying can therefore be used to measure pain in newborn infants only when the cause of crying is known.

Ulceration in an ileocolic anastomosis treated with ranitidin.

The authors report the case of a child born with a gastroschisis and an ileal atresia. After surgery, only 100 cm small bowel and the distal one third of his colon remained. Perianastomotic ulcers developed 6 years later. These were treated successfully with ranitidin, a treatment not previously reported in the literature. The authors conclude that treatment with ranitidin was successful in a patient with an ulcer in an ileocolic anastomosis.

Routine laparoscopy for nonpalpable testes?

There are still no accepted criteria for the selection of patients with nonpalpable testes for laparoscopy versus a primary surgical exploration. We here report our experience using routine laparoscopy in such patients. The aim was to determine whether laparoscopy should be the first operative intervention or follow an inguinal exploration. Included in the study were 61 boys with 69 nonpalpable testes. Thirty-three testes were found in the abdomen, and 36 testes were extra-abdominal or nonexistent. If an exploration of the inguinal region had been the initial surgical intervention, six testes would have been found, making laparoscopy unnecessary. On the other hand, in the search for 63 missing testes, laparoscopy saved the patients from laparotomy or an extensive inguinal exploration. We conclude that an accurate knowledge of testis, vas, and vessel location gained by laparoscopy facilitates the selection of an appropriate surgical strategy, saving at least 51% of patients from laparotomy or an extensive inguinal exploration.

Complications of laparoscopy-aided gastrostomies in pediatric practice.

PURPOSE: The aim of this report is to establish the frequency and type of complications of laparoscopy-aided gastrostomy in pediatric practice and to identify patients at risk for postoperative complications. METHOD: This is a follow-up study of 98 children with nutritional problems including inability to swallow, inadequate calorie intake in neurologically impaired children, patients with cystic fibrosis, malignancies, neurometabolic diseases, and cardiac malformations. Laparoscopy-aided gastrostomy was attempted in all patients. These patients have undergone follow-up at our outpatient clinic. Postoperative complications and problems with the gastrostomy device were registered. The postoperative complications were divided into minor problems and major or life-threatening complications. RESULTS: There was no perioperative mortality. No life-threatening complication developed, whereas minor problems were common, necessitating medical attention postoperatively. Patients with congenital heart disease, chronic respiratory failure, and metabolic diseases experienced the highest frequency of postoperative complications. CONCLUSIONS: The surgical placement of an enteral access device in children should be considered a major surgical procedure, demanding medical attention for 1 to 2 months postoperatively. The rate and severity of complications with the method described are tolerable considering the severity of the underlying diseases.

Gastrostomy button causing perforation of the posterior gastric wall.

A gastrostomy button complication, not previously reported, is described. The button, with an inflatable balloon, was used for nutrition. The patient had had a gastrostomy for 4.5 y, with the same type of gastrostomy button for the previous 2 y and the same device for 1 y. The tip of the button caused a perforation of the posterior stomach wall, leading to death.

Two children with xeroderma pigmentosum developing two different types of malignancies simultaneously.

Patients with xeroderma pigmentosum (XP) are highly sensitive to ultraviolet radiation and prone to develop multiple skin malignancies. We report two children under 6 years of age with XP who each developed two histologically different types of malignancies simultaneously. We conclude that it is of importance to be aware of the possibility of multiple malignancies of different types, even in young children.

Single-puncture laparoscopic gastrostomy in children.

An original one-puncture technique of performing laparoscopic gastrostomy is described. The implications are analysed. The results of five operations are presented and the advantages and limitations are discussed in the light of current available methods.

Membranous duodenal stenosis.

The experience of our 16 patients treated for membranous duodenal stenosis is reported. Their treatment and course was analysed in a retrospective study. Eight patients were operated on within the first 16 days of life and in the remaining group surgery was performed at 1 month to 4 y of age. The presenting symptom leading to diagnosis was, in all but one case, non-bile-stained vomiting. Associated malformations were found in all but four patients, mostly morbus Down. The operative procedure performed was a partial excision of the duodenal membrane and a duodenoplasty in 10 patients, a duodenojejunostomy in 5 patients, and a duodenoplasty only in 1 patient. The postoperative course was without lethal complications. One late stricture in an anastomosis occurred. We conclude that in its presentation, duodenal stenosis differs from duodenal atresia, and can often be misinterpreted, resulting in a late diagnosis, and should be reported as a separate entity.

Laparoscopy aided gastrostomy in children.

BACKGROUND AND AIMS: The aim of this report is to describe a method for laparoscopy aided button gastrostomy in children. MATERIAL AND METHODS: The method includes the use of two ports, one umbilical and one subcostal on the left side. The stomach is exteriorized using a grasping forceps in the subcostal port. Under direct vision the gastrostomy button, MIC-KEY, is inserted into the stomach at the lesser curvature and secured by purse string sutures. The stomach is attached to the anterior abdominal wall. RESULTS: The results show that this method has been successfully used in 33 children without operative complications. CONCLUSIONS: We conclude that by inserting the gastrostomy button under direct vision, damage to other abdominal organs is avoided and a correct placement at the lesser curvature obtained. The combination of laparoscopic and open procedures makes the method easy and safe.