[A right-sided congenital diaphragmatic hernia masked by pneumonia]. / Een rechtszijdige congenitale hernia diaphragmatica gemaskeerd door een pneumonie.

A 1-day-old premature newborn (34 weeks and 6 days) presented with respiratory insufficiency due to a group B haemolytic streptococcal (GBS) pneumonia. She recovered after temporary treatment with mechanical ventilation and antibiotics. At the time of discharge there was a slight increased fogging on the right side of the chest X-ray, interpreted as residual pleural effusion. Three days later the patient was readmitted with respiratory failure and a need for respiratory support. A chest CT scan revealed a right-sided congenital diaphragmatic hernia (CDH) with a large part of the liver and intestine in the chest. The diaphragmatic defect was closed during a surgical procedure. After an uneventful recovery the patient was discharged in good clinical condition. The combination of delayed presentation of right-sided CDH and neonatal GBS infection occurs rarely but has been described. Its pathogenesis is still unclear.

Preduodenal portal vein and malrotation: what causes the obstruction?

AIM AND METHOD: Preduodenal portal vein is a rare congenital abnormality, and occurs either as a single malformation, in association with other malformations or as part of "polysplenia" syndrome. Preduodenal portal vein has seldom been reported as a cause of intestinal obstruction, however corrective surgery is nearly always performed. We conducted a 25-year retrospective study in a single centre to investigate the cause of obstruction in patients with preduodenal portal vein. Furthermore, we reviewed the literature on preduodenal portal vein. RESULTS: Over a period of 25 years, preduodenal portal vein was diagnosed in five patients. The diagnosis was made during surgery performed because of symptoms of high intestinal obstruction. All five patients had intestinal malrotation as well and, in all patients, another cause for high intestinal obstruction than preduodenal portal vein was found. CONCLUSION: Preduodenal portal vein is mainly asymptomatic. It is often associated with other intestinal congenital abnormalities more likely to cause high intestinal obstruction. Therefore, the (paediatric) surgeon should always be alert for another associated cause of intestinal obstruction. Because of the potential for technical problems from preduodenal portal vein during surgery, it nevertheless should be on the surgeon's mind during surgery when the patient has high intestinal obstruction.

Disturbed cerebral circulation during opening of the venoarterial bypass bridge in extracorporeal membrane oxygenation.

PURPOSE: To describe the effects on cerebral blood flow velocity (CBFV) of intermittent opening of the venoarterial bridge (VA bridge) during venoarterial extracorporeal membrane oxygenation (VA-ECMO). STUDY DESIGN: Prospective study in 22 newborns during VA ECMO. CBFV was measured in the pericallosal artery by Doppler ultrasound. Changes in peak systolic flow velocity (PSV), end diastolic flow velocity (EDV) and time-averaged mean flow velocity (TAM) on day 1, 2, 3, and 5 and at low ECMO flow (50- 150 ml/min) were analyzed (mean percentage+/-standard deviation (t-tests, p<0.05)). Changes >25% were considered relevant. The relationship between changes in CBFV and ECMO flow rate (Pearson correlation, p<0.01) was studied. RESULTS: Opening of the VA bridge resulted in statistically significant and relevant decreases in PSV (35 +/- 18%), EDV (93 +/- 15%) and TAM (68 +/- 13%), persisting during the consecutive days of treatment. Smaller changes in CBFV at low ECMO flow were statistically significant and mostly relevant: PSV (15 +/- 7%), EDV (76 +/- 21%) and TAM (40 +/- 12%). Changes in CBFV were positively correlated to the ECMO flow. CONCLUSION: Use of the VA bridge results in significant and relevant ECMO flow-dependent changes in CBFV, persisting during the treatment. The VA bridge should be used in such a way as to allow regular unclamping to be omitted.

Factors associated with recurrence and metastasis in sacrococcygeal teratoma.

BACKGROUND: Sacrococcygeal teratoma (SCT) is a relatively uncommon tumour, with a high risk of recurrence and metastasis. The factors associated with recurrence and metastatic disease were studied. METHODS: A retrospective review was conducted of 173 children with SCT treated between January 1970 and February 2003 at the paediatric surgical centres in the Netherlands. Risk factors were identified by univariate and multivariate analysis. RESULTS: Eight children died shortly after birth or around the time of operation. Nine children, all over 18 months old, had metastases at presentation. Four teratomas with metastasis showed mature histology of the primary tumour. Nineteen children had recurrence of SCT a median interval of 10 months (range 32 days to 35 months) after primary surgery. Risk factors for recurrence were pathologically confirmed incomplete resection (odds ratio (OR) 6.54 (95 per cent confidence interval (c.i.) 2.11 to 20.31)), immature histology (OR 5.74 (95 per cent c.i. 1.49 to 22.05)) and malignant histology (OR 12.83 (95 per cent c.i. 3.27 to 50.43)). Size, Altman classification, age and decade of diagnosis were not risk factors for recurrence. One-third of the recurrences showed a shift towards histological immaturity or malignancy, compared with the primary tumour. Seven patients died after recurrence, five with malignant disease. CONCLUSION: This national study showed that SCT recurred in 11 per cent of the children within 3 years of operation. Risk factors were immature and malignant histology, or incomplete resection. Mature teratoma has the biological capability to become malignant.

[Dumping syndrome in a child with an incorrectly positioned gastrostomy catheter]. / Dumpingsyndroom bij een kind met een verkeerd gelegen gastrostomiekatheter.

A 13-month-old girl experienced an epileptic fit due to hypoglycaemia. She was born with a posterolateral diaphragm defect and at the age of 1.5 months had undergone a Nissen fundoplication with which a gastrostomy was also performed. The tip of the gastrostomy catheter was found to be lying in the bulbus duodeni. Once this had been pulled back, the patient made a good recovery. Dumping syndrome is usually the result of a quicker gastric emptying time or a smaller gastric capacity, and in children it is mostly due to a Nissen fundoplication. In patients fed by tube who exhibit symptoms of dumping syndrome, the position of the feeding tube must be checked before other diagnostic or therapeutic steps are undertaken.

Recirculation in double lumen catheter veno-venous extracorporeal membrane oxygenation measured by an ultrasound dilution technique.

Recirculation is a limiting factor for oxygen delivery in double lumen catheter veno-venous extracorporeal membrane oxygenation (DLVV-ECMO). This study compares three different methods for the determination of the recirculation fraction during double lumen catheter veno-venous ECMO at ECMO flow rates of 150, 125, 100, 75, and 50 ml/kg.min in nine lambs: (1) an ultrasound dilution method, in which the change in ultrasound velocity in blood after injection of a saline bolus as a marker is used for determination of recirculation; (2) an SvO2 method using real mixed venous blood oxygen saturation, the gold standard, for determination of recirculation fraction; and (3) the CVL method, in which oxygen saturation of a blood sample of the inferior vena cava is considered to represent mixed venous oxygen saturation. In all methods, the recirculation fraction increased with increasing ECMO flow rate. The correlation coefficient between the ultrasound dilution method and the SvO2 method was 0.68 (p < 0.01); mean difference was -2.4% (p = 0.6). Correlation coefficient between the ultrasound dilution method and the CVL method was 0.48 (p < 0.01); mean difference was -18.1% (p < 0.01). The correlation coefficient between the SvO2 method and the CVL method was 0.51 (p < 0.01); mean difference was -15.7% (p < 0.01). The ultrasound dilution method is a useful method for measurement of the recirculation fraction in DLVV-ECMO and is easier to use than the other methods.

Remodeling of pulmonary arteries in human congenital diaphragmatic hernia with or without extracorporeal membrane oxygenation.

PURPOSE: The aim of this study was to describe in detail the perinatal developmental profile of the pulmonary vasculature in congenital diaphragmatic hernia (CDH) and to examine the potential beneficial effects of extracorporeal membrane oxygenation (ECMO) on the vascular morphology. Additionally the authors aimed to identify the differences in pulmonary vascular morphology among CDH cases according to the primary cause of death: either extreme lung hypoplasia (LH) or persistent pulmonary hypertension (PPH). METHODS: The authors studied autopsy sections from 30 high-risk CDH cases with respect to the pulmonary arteries in relation to gestational age (GA) and ECMO treatment. They were grouped into CDH-I: 20 cases with GA greater than 34 weeks who were not subjected to ECMO and CDH-II: 10 cases with GA greater than 34 weeks, who were subjected to ECMO for an average time of 237 hours. Five age-matched neonates who died from placental insufficiency or birth asphyxia without evidence of lung hypoplasia served as controls (CON). Medial and adventitial thicknesses of pulmonary arteries were measured in lung sections stained with Elastic van Gieson by 2 investigators blinded for the clinical data. Immunohistological staining with anti-alpha-smooth muscle actin (alpha-SMA) was performed to confirm the precise location of the arterial media before morphometry. CDH cases were subgrouped and compared according to the primary cause of death. Unpaired Student t test was used for statistics, with significant P value < or =.05. RESULTS: In CDH newborns, a significant increase in medial, adventitial, and total wall thickness was found in pulmonary arteries with an external diameter of less than 200 microm as compared with age-matched controls (P<.004, .0001, and .0009, respectively). ECMO-treated CDH newborns showed a significantly thinner arterial adventitia than CDH patients who did not receive this treatment (P<.0001), approaching normal values. However, the medial thickness remained increased. Morphometrically, no significant differences in CDH cases between patients dying of PPH or severe LH could be determined. CONCLUSIONS: (1) In CDH, there is failure of the normal arterial remodeling processes occurring in the perinatal period. (2) Pulmonary vascular morphology in CDH does not differ between the groups with lung hypoplasia or persistent pulmonary hypertension as primary cause of death. (3) Adventitial thinning of these arteries might be one of the mechanisms by which ECMO alters PPH in CDH cases.

Osteosarcoma: oncologic and functional results. A single institutional report covering 22 years.

BACKGROUND AND OBJECTIVES: The oncologic and functional results in patients treated because of osteosarcoma (OS) were evaluated. METHODS: Fifty-one patients with high-grade OS were treated between 1974 and 1996 at our hospital. All patient records were studied, and the surviving patients were evaluated according to the American Musculoskeletal Tumor Society functional rating system. The majority of patients received adjuvant chemotherapy (prior to 1983) or neoadjuvant chemotherapy (from 1983). Until 1987, all patients with extremity OS had ablative surgery; from 1987, the majority had limb-saving surgery. Lung metastases were resected in most cases. RESULTS: Overall 2-year and 5-year disease-free survival (DFS) rates were 27 of 51 and 16 of 42, respectively. Patients with vertebral or pelvic OS or contaminated margins after resection had a very bad outcome. In all other subgroups, including patients with various types of chemotherapy, response to chemotherapy, diameter of tumor, presence or absence of metastatic spread, and location of tumor, a 5-year DFS of about 50% was found. Recurrent disease in patients who had achieved a 2-year disease-free interval was relatively low (4/23 patients). CONCLUSIONS: Survival in our series was worse than in most other studies. A very bad outcome was found in patients with vertebral or pelvic OS or with contaminated margins after resection.

[Results of extracorporeal membrane oxygenation in 100 newborns with cardiorespiratory insufficiency]. / Resultaten van extracorporele membraanoxygenatie bij 100 pasgeborenen met cardiorespiratoire insufficiëntie.

OBJECTIVE: To describe the results of treatment with extracorporeal membrane oxygenation (ECMO) in newborns with severe cardiorespiratory insufficiency. DESIGN: Prospective, descriptive. METHODS: For all 100 newborns treated with ECMO in 1989-1997 in the Academic Hospital Nijmegen, department of Neonatology, the Netherlands, indications for treatment, complications during treatment and mortality within 6 weeks after cessation of ECMO were registered. RESULTS: The 100 children comprised 66 boys and 34 girls, with a mean age of 2 days (range: 1-15). Indications for ECMO treatment were: meconium aspiration syndrome: 39 with 37 survivors (95%), congenital diaphragmatic hernia: 31 with 23 survivors (74%), sepsis or pneumonia: 20 with 14 survivors (70%) and 'others' among which persistent pulmonary hypertension of the newborn: 10 with 8 survivors (80%). Eighteen children died (18%). Causes of death were rebound pulmonary hypertension (9 times), intracranial haemorrhage (4), multi-organ failure (3) and pulmonary problems (2). The most important complications during treatment were bleeding problems (29), clotting problems (20) and infections (11).

Surgical repair of congenital diaphragmatic hernia during extracorporeal membrane oxygenation: hemorrhagic complications and the effect of tranexamic acid.

Extracorporeal membrane oxygenation (ECMO) was incorporated in a strategy of delayed repair of congenital diaphragmatic hernia (CDH) and was used for preoperative stabilization in patients who were unresponsive to maximal conventional treatment. If ECMO was required for preoperative stabilization the diaphragmatic defect was repaired while the patient was on ECMO. In the early experience with this approach all patients suffered from bleeding complications. Therefore, we adopted the use of antifibrinolytic therapy with tranexamic acid (TEA) during and immediately after CDH repair on ECMO. The efficacy of TEA was studied in an unblinded study using historical controls by comparing the postoperative blood loss and the transfusion requirements of red blood cells (RBC) in patient groups treated without (n = 9) and with TEA (n = 10). Patients who received TEA had significantly less bleeding at the surgical site than patients not receiving TEA (57 v 390 mL, P = .005) and had significantly lower RBC transfusion requirements than patients not receiving TEA (1.13 v 2.95 mL/kg/h, P = .03). In the very first two patients of the TEA group we encountered fairly severe thrombotic complications. TEA may have contributed to those complications. Based on the authors' experience they conclude: (1) TEA is effective in reducing postoperative blood loss, hemorrhagic complications, and RBC transfusion requirements associated with CDH repair on ECMO. (2) TEA may be responsible for thrombotic complications. (3) The appropriate, empirically established, dosage and administration patterns of TEA for CDH repair during ECMO seem to be one bolus of 4 mg/kg TEA intravenously 30 minutes before the anticipated CDH repair and a continuous infusion of 1 mg/kg/h TEA during the 24 hours after CDH repair.

Rectal atresia: pathogenesis and operative treatment.

Rectal atresia (RA) with a normal anus is a rare anomaly mostly described as part of a series of anorectal malformations. Most authors believe it to be an acquired lesion with a vascular genesis. One of the arguments quoted is the lack of other congenital anomalies. Several operative procedures are recommended for this lesion. We describe four patients with RA who had other significant congenital anomalies; two other cases were found in the literature. A lack of other congenital anomalies in patients with RA does not seem to be a strong argument for an acquired lesion. All four patients were treated by a posterior sagittal approach with good functional results.

Ewing's sarcoma of bone: oncologic and functional results.

This paper describes 29 patients with Ewing's sarcoma of bone treated between 1975 and 1990 at the University of Nijmegen Hospital, Nijmegen, The Netherlands. Osteomyelitis was the primary diagnosis in 24%. Treatment consisted of chemotherapy in combination with surgery and/or radiotherapy. Nine patients received radiotherapy only; five of them died of disease. Five patients underwent an intralesional excision; four of them died of disease. Twelve patients underwent a wide excision; there is no evidence of disease in any of them. Three patients underwent a radical disarticulation; all died of disease. The disease-free survival at 1.5 years was 66%. This figure at 5 years was 55%. After wide excision and reconstruction in tumors of expendable, femoral or radial bones good functional results were obtained in all cases.

Results of splenectomy performed on a group of 91 children.

In the period 1971-1990 91 children underwent splenectomies in the University Hospital of Nijmegen. The most important indications are hereditary spherocytosis, Hodgkin's disease and very severe immune thrombocytopenic purpura (ITP). Splenectomy after a traumatic rupture of the spleen has become less frequent: from 20% in 1971-1980 to 4% in 1981-1990. Short-term complications included thrombocytosis (84%), fever without an obvious cause (46%), which is quite regularly seen in patients suffering from Hodgkin's disease (48%), and infections of the respiratory tract in 10% of the patients. The platelet count shows a steady increase in the first nine post-operative days. No thromboembolic complications were seen. Based upon the literature there seems to be no reason at this moment for anti-platelet aggregation therapy when platelet counts are below 1000 x 10(9)/l. More information about long-term complications was obtained through a questionnaire completed by general practitioners. The morbidity through overwhelming post splenectomy infection (OPSI) is 3.8% (3/79), the mortality of OPSI is 2.5% (2/79). Underlying diseases, especially those which involve the immunological system as auto immune haemolytic anemia (AIHA), seem to play an important role in the possible development of OPSI (morbidity 2/11, 18%).

The midline cervical cleft.

The midline cervical cleft is a rare congenital anomaly of the ventral neck. With reference to two patients with a midline cervical cleft, we discuss the clinical picture, the embryology, and the surgical treatment.

Long-gap esophageal atresia: experience with reconstruction in 25 patients.

In a series of 183 patients with esophageal atresia in a period of 20 years, 25 had a "long-gap" esophageal atresia. In most of these cases we succeeded in saving the patients' own esophagus but at the cost of one to two years of intensive treatment. The overall survival was 80%. The result compares favorably with the results of esophageal replacement reported in the literature.

Rectal prolapse in children.

Rectal prolapse in children is nowadays a rare anomaly. Potty training in young children is the common cause and the resulting prolapse can be treated conservatively. In children with refractory prolapse, sclerosing injections may be used. Operative treatment by posterior rectopexy is only indicated in the very few cases of long-standing prolapse.

Subcutaneous rheumatoid nodules.

We describe seven children with subcutaneous rheumatoid nodules who had no clinical evidence of rheumatoid arthritis. Only one girl was seropositive for antinuclear factors and had a slightly raised erythrocyte sedimentation rate. Clinical aspects, risks for developing rheumatoid arthritis, and treatment of this entity are discussed.

Management of pediatric esophageal perforation.

Esophageal perforation is a serious complication necessitating immediate therapy. In a retrospective study we have evaluated the results in 13 children treated for esophageal perforation. Eleven of 13 perforations could be managed conservatively. In one child with extrapleural effusion, tube drainage was performed. The only death in this series occurred in a child who was brought for treatment after a 60-hour delay. Thoracotomy and multiple abscess drainage eventually proved unsuccessful. On the basis of our experience with children with esophageal perforation or with complications after esophageal atresia repair, we conclude that management of esophageal perforation in children differs substantially from therapy in adults and necessitates restrictive treatment guided by clinical symptoms.