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Biomedical subjects

C Festen

Publications and source records attributed to C Festen.

At least 19 recordsLinked to original sources
PubMed2002

Excellent long-term outcome for survivors of apple peel atresia.

BACKGROUND: Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children. METHODS: The patient charts, operative reports, and office notes of 15 children with apple peel atresia from 6 pediatric surgical centers in the Netherlands were reviewed. Long-term follow-up was assessed through review of office notes and through questionnaires. RESULTS: The median age at the time of operation was 1.5 days. Postoperatively, 53% suffered from cholestasis, and 40% were septic. Three patients died (20%). At follow-up at a median age of 24 months, 1 child showed growth retardation and 2 children suffered from short bowel syndrome. At the time of the questionnaire, all children showed normal growth and development. CONCLUSIONS: Even though children with apple peel atresia often suffer serious morbidity like short bowel syndrome and sepsis during the postoperative course, late morbidity turned out to be low. If the patients survive the operative and direct postoperative period, and survive the morbidity associated with malnutrition and the long-term use of total parenteral nutrition, they have a good chance of having normal bowel function with normal growth and development.

Female↗
PubMed2002

The influence of ductal left-to-right shunting during extracorporeal membrane oxygenation.

BACKGROUND/PURPOSE: The aim of this study was to analyze whether a ductal left-to-right (L-R) shunt will prolong extracorporeal membrane oxygenation (ECMO) in neonates with severe pulmonary hypertension. This report discusses the onset and termination of a ductal L-R shunt and its potential influences on ECMO when pulmonary hypertension decreases during venoarterial bypass. METHODS: Twenty-nine neonates were monitored during veno-arterial ECMO, using bedside echocardiography with 12-hour interval observations. RESULTS: Up to 43% of the patients showed this type of shunt already after 12 hours on bypass. In total, this type of ductal shunt was found between 12 and 72 hours on ECMO in 62% of the patients. After 72 hours, the ductal L-R shunt no longer was detected. In 38% of the patients, no ductal L-R shunt was found during ECMO. Comparisons between these 2 patient groups showed a significantly longer ECMO duration in patients with ductal L-R shunt (P <.007). The mean prolongation time was 46 hours. Also, a significant decrease of left atrium to aorta ratio (P <.01) was observed during ECMO in the ductus group after closure of the duct, illustrating the decrease in volume load for the left heart and lungs. CONCLUSIONS: Ductal L-R shunting is related with a substantial prolongation of the ECMO course (mean prolongation of almost 2 days). The authors suggest that on one side, the ductal L-R shunt will lead to pulmonary hypercirculation and on the other side, postductal stealing from the descending aortic circulation will lead to prerenal failure. Possibly because of interactions with pulmonary and renal function, a ductal L-R shunt will, among other factors, interfere with weaning from ECMO, resulting in a prolonged bypass time.

Echocardiography↗
PubMed2001

Postanal sinus: single or different etiologies?

Experience with five patients who presented with a postanal sinus (PAS), all of which appeared to have a similar etiology at first hand, is reviewed. All patients were female and presented with a perianal fistula located in the midline posterior to the anus between the internal and external sphincter. All patients had a similar history and age at presentation (the 1st decade of life), which increased our assumption of a similar etiology. Further examinations revealed no internal connection to hollow organs or other pelvic structures, proving that the fistula was a sinus in all cases. One patient had a scimitar sacrum. In four cases the sinus was excised transanally, in one through a posterior sagittal approach. All patients had normal anal function postoperatively. Histologic examination was performed in all cases and showed results ranging from various types of epithelium to dermoid and epidermoid cysts, dismissing the theory of a similar etiology. In our opinion, a PAS can be a presenting sign for a variety of retrorectal developmental pathologies and should be differentiated from fistula-in-ano.

Child↗
PubMed2001

Multidisciplinary behavioral treatment of defecation problems: a controlled study in children with anorectal malformations.

BACKGROUND/PURPOSE: The most frequent consequences of being born with an anorectal malformation (ARM) are problems with fecal continence and constipation, which can have various negative implications. In this prospective, controlled study the effect of multidisciplinary behavioral treatment dealing with these problems is evaluated. METHODS: The effect of multidisciplinary behavioral treatment was studied in 24 children (15 boys, 9 girls; mean age 5.8 years). Thirteen children were allocated to the treatment condition. The 11 children allocated to the waiting list control group also were treated after a waiting period of 6 months. Children underwent follow-up after treatment. RESULTS: Compared with a waiting list control group, the experimental treatment group scores significantly better on 2 important measures ("Templeton," "Percentage of feces in toilet"). Although young children had poorer scores than older children before treatment, no significant differences in the favorable outcome of treatment were found between both groups after treatment. No effect of type of ARM on treatment was found either. The results of multidisciplinary behavioral treatment remain stable over a mean follow-up period of 7 months. CONCLUSION: Multidisciplinary behavioral treatment is an important and valuable supplement to the standard medical treatment of children born with ARM suffering from chronic defecation problems.

Anal Canal↗
PubMed2000

Defecation problems in children with Hirschsprung's disease: a biopsychosocial approach.

Although most patients with operated Hirschsprung's disease (HD) have good continence in adulthood, a majority have postoperative defection problems during school age. Persistence of chronic constipation and/or incontinence may have considerable consequences for psychosocial development, parent-child interactions, quality of life, and the child's general condition. Considering these consequences, it is important to treat these problems as early as possible. From a biopsychosocial view, we developed a multidisciplinary treatment aimed at resolving defecation problems by teaching the child bowel self-control, primarily by training optimal defecation skills and subsequently toilet behavior. This treatment, carried out by a child psychologist, a pediatric physiotherapist, and a pediatric surgeon, consists of five steps: explanation; extinction of fear and avoidance behavior; learning new defecation behavior; learning an adequate straining technique; and generalization toward daily life. The effect of the treatment was investigated retrospectively in 16 boys with operated HD. The children improved significantly in all aspects during treatment, suggesting that multidisciplinary treatment can significantly reduce the postoperative chronic bowel problems of most children with operated HD. The treatment was as effective in young children (2-5 years) as in older children (5-14 years).

Adolescent↗
PubMed2000

Biopsychosocial treatment of defecation problems in children with anal atresia: a retrospective study.

In a retrospective study, we examined whether multidisciplinary treatment based on a biopsychosocial approach and carried out by a pediatric surgeon, a child psychologist, and a pediatric physiotherapist is successful in reducing defecation problems (incontinence and/or constipation) in children with operated anal atresia (AA) (mean age 6.9 +/- 4.01 years). A second question was whether this treatment is successful in young children aged 2-5 years. The multidisciplinary approach consisted of standard medical treatment and a behavioral program to teach children and their parents adequate defecation behavior including an adequate straining technique. Forty-three children aged 2-16 years were included: 27 boys and 16 girls with AA, of whom 26 had high or intermediate and 17 low AA. Besides continence and constipation, defecation behavior and straining technique were evaluated. The children improved significantly during treatment in all aspects of defecation. No differences in effect of treatment were found between young children (2-5 years) and older ones, so this treatment seems to be equally effective in both age groups. This study demonstrates that both somatic and behavioral factors contribute to the persistence of chronic defecation problems. It is concluded that treatment of these problems in patients with operated AA should include behavioral modification techniques.

Adolescent↗
PubMed2000

Neonatal pulmonary hypertension during extracorporeal membrane oxygenation.

OBJECTIVES: This prospective study was designed to monitor severe pulmonary hypertension during extra corporeal membrane oxygenation using echo Doppler variables. BACKGROUND: All neonates treated with extracorporeal membrane oxygenation also have severe pulmonary hypertension. A study which monitors the reaction of the pre-existing pulmonary hypertension during extracorporeal oxygenation by frequent sampling of those variables related to pulmonary pressure is still lacking. Such a study is necessary to analyze the complex haemodynamic changes in patients undergoing extracorporeal membrane oxygenation. METHOD: In 29 neonates, we estimated pulmonary arterial pressure using peakflow velocity of regurgitation across the tricuspid- and pulmonary valve, peakflow velocity of shunting across persistent arterial ductus, and systolic time intervals of the right ventricle. Correlation between the several estimations of pulmonary arterial pressure were analysed with the Spearman correlation coefficient. RESULTS: Systolic pulmonary arterial pressure measured by the velocity of tricuspid regurgitation illustrated severe pulmonary hypertension prior to extra corporeal membrane oxygenation (mean 63 mmHg, sd 20). Similar levels for the systolic pulmonary arterial pressure could be derived (mean 73 mmHg, sd 17) from ductal shunting. A fair correlation of 0.76 (p< 0.002) could be demonstrated. Pulmonary hypertension responded well and quickly to treatment by extra corporeal membrane oxygenation, with reductions within 24 hours to mean systolic levels of 35 mmHg, sd 23. This very early reaction has not previously been demonstrated and could be of importance in defining parameters for weaning from cardiopulmonary bypass. Diastolic pulmonary arterial pressure was investigated because of its relation to vascular resistance. It proved more difficult to measure because of the low incidence of pulmonary regurgitation. Derived diastolic pressures did not show any good correlations. CONCLUSION: Pulmonary hypertension is well documented prior to extra corporeal membrane oxygenation and response very quickly to the institution of treatment. Ultra sound techniques are indicated at the bedside, and prove useful in monitoring pulmonary blood pressure during the procedure.

Echocardiography, Doppler↗
PubMed1999

[Results of extracorporeal membrane oxygenation in 100 newborns with cardiorespiratory insufficiency].

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).

Cause of Death↗
PubMed1999

['Acute abdomen' in children].

A child is said to have an 'acute abdomen' in case of severe abdominal pain of sudden onset. Further investigations are necessary if the pain is very bad, persists for longer than 3-4 hours or is accompanied by vomiting. Failure to make the correct diagnosis may result in severe complications. The principal cause in an older child is acute appendicitis. This diagnosis is to be based on the anamnesis, physical examination and laboratory tests. If one of these is typical, active observation is indicated; if two or three are typical, appendectomy is indicated. Differential diagnoses can only be made during laparotomy. Intussusception occurs more frequently in toddlers. In a child with possible intussusception, observation is not justified: the diagnosis should be excluded by a contrast colonic X-ray, or operation should be performed either immediately or, if the symptoms persist, after the X-ray. Rest and patience, careful and child-adapted approach, and correct evaluation of aberrant symptoms may minimize unnecessary intervention.

Abdomen, Acute↗
PubMed1999

A multistate Kalman filter for neonatal clotting time prediction and early detection of coagulation disturbances.

The multistate Kalman filter was applied to develop a heparin dose proposal system and to detect coagulation disturbances during neonatal extracorporeal membrane oxygenation (ECMO). A system containing this filter was based on the activated clotting time (ACT) values and the heparin doses administered every hour during ECMO. If the ACT value can be predicted accurately from the previous heparin dose, a heparin dose proposal can be given to achieve or maintain the required ACT level. The analysis was done on 6,356 ACT level measurements in 44 ECMO neonates. The multistate Kalman filter technique showed an unbiased prediction of ACT, with a standard deviation of 23 seconds. Two out of three cases of disseminated intravascular coagulation (DIC) were detected. ACT values were predicted sufficiently accurately by the multistate Kalman filter technique to justify a prospective study on the performance of the heparin dose proposal system and its ability to detect DIC.

Drug Therapy, Computer-Assisted↗
PubMed1998

Parenting children with anorectal malformations: implications and experiences.

Parents play a crucial role in the life of a child suffering from an anorectal malformation (ARM), since their guidance contributes to the degree to which the child learns to cope with his or her disability. We investigated whether they experience stress in parenting such a child and also attempted to identify somatic or behavioral characteristics in the child that influence the stress of parenting. The parents of 109 children (69 males, 40 females; median age 5.9 years, range 1-18 years) with an ARM (58 low, 10 intermediate, 41 high) were studied. The Nijmegen Questionnaire on Child-rearing Situations (NQCS) was used to investigate the existing parenting situation. Behavioral characteristics of the children were studied by means of the Child Behaviour Checklist (CBCL) and the Teacher Report Form (TRF). In a semi-structured interview, we investigated how parents experienced the implications of the disability in everyday life with their child. Our study showed that as far as the perception of parenting stress is concerned, parents of children with an ARM do not differ from those with healthy primary-school children. Within the group of parents with ARM-afflicted children, the parents of older, incontinent children experienced relatively more stress, especially when the child concerned was male. With regard to the children's behavior, the parents and teachers under investigation did not report a higher than normal incidence of deviant behavior. However, when individual parents observed difficult behavior in their child, they found it harder to deal with than the incontinence for feces. Regarding the implications of the disorder for their everyday lives, parents were concerned and indicated a need for specific counselling. We conclude that having a child with a somatic affliction, in this case an ARM, does not automatically imply that the parents experience child-rearing problems. However, certain groups of parents are more at risk, i.e., parents with older, incontinent sons and parents with children exhibiting behavioral problems. In addition, our study shows that parents do have difficulties in coping with the implications of the disorder and express a need for support. We feel that patient care can be improved if aid is tailored to these specific problems.

Abnormalities, Multiple↗
PubMed1998

Congenital microgastria: a case report and review of literature.

Congenital microgastria is an uncommon result of impairment of normal foregut development. To date, only 39 cases have been described in the literature. We report a boy born with microgastria and bilateral hypoplastic kidneys who had feeding problems, resulting in failure to thrive and growth retardation. After a short period of conservative management, he was operated upon at the age of 11 months. A Hunt-Lawrence pouch was created, leading to toleration of increasing amounts of oral feeding. Although his feeding problems have decreased, his height and weight are below normal (<10th percentile). The embryology, clinical presentation, diagnostic procedures, associated anomalies, and management are discussed.

Abnormalities, Multiple↗
PubMed1998

Perianal abscess and fistula-in-ano in infants.

PURPOSE: The aim of this study was to obtain insight into the short- and long-term results of treatment of perianal abscess and fistula-in-ano in infants. METHODS: This is a retrospective study of the records of patients treated over a 21-year period from January 1974 until December 1994 in a Pediatric Surgical Center. A long-term (1 to 24 year, mean 7.74 year) follow-up by questionnaire (response 81%) is also included. RESULTS: Drainage of a perianal abscess is followed in 35% of cases by a fistula. Fistulotomy or fistulectomy is followed in 13% of cases by a recurrence. There were two long-term recurrences that both healed spontaneously. The persisting scar sometimes gives problems with anal cleaning. All children aged 3 years and older were continent for feces. In two, there was soiling for some time. One had constipation and one was incontinent during the night. CONCLUSIONS: Simple drainage of a perianal abscess is followed frequently by a fistula. Fistulotomy or fistulectomy of a fistula-in-ano in infants has a reasonable chance of recurrence in the short term. Long-term recurrences are exceptional. There are no serious disabilities in the long run.

Abscess↗
PubMed1998

Cardiac tamponade: a rare but life-threatening complication of central venous catheters in children.

The widespread use of central venous catheters in the treatment of pediatric patients has caused an increased incidence of complications. A rare, but potentially fatal complication occurs when the heart is perforated by the catheter tip causing a cardiac tamponade. This perforation of the heart generally is associated with the insertion procedure, but may also occur after some time because of displacement of the catheter tip. The authors present three cases in which the placement of a central venous catheter resulted in lethal cardiac tamponade. Proper positioning of the catheter tip in the superior vena cava and a high index of suspicion are essential in preventing this serious complication. Contrast-enhanced chest x-ray after insertion of the catheter must be performed to ascertain a correct position of the tip.

Cardiac Tamponade↗
PubMed1998

Recurrent congenital diaphragmatic hernia; which factors are involved?

The medical records of 66 surviving children born with congenital posterolateral diaphragmatic hernia (CDH) were reviewed to determine which factors are involved in the development of a recurrent hernia. Fifty-seven patients had a left-sided defect and 9 patients had a right sided defect. In 54 patients the defect was closed primarily and 12 patients required a prosthetic patch. Nine of the 66 patients (14%)--5 patients (9%) with a left-sided defect and 4 patients (44%) with a right-sided defect--developed a recurrent hernia on the average of 4.0 months (range 0.2-6.9) after the initial repair. Except for one, all patients with a recurrence presented with dyspnoea (n = 5) and/or feeding problems (n = 6). Of these 9 recurrences 4 patients (7%) had primary closure of the defect and in 5 patients (42%) a prosthetic patch was required. Four of the 11 patients (36%) who needed extracorporeal membrane oxygenation (ECMO) had a recurrence versus 5 patients (9%) who were conventionally treated. A hernial sac was found in 4 patients during the repair of the recurrent hernia. In 3 of them the initial defect was closed primarily. In conclusion, large-patch repaired defects and right-sided defects are risk factors for the development of a recurrent hernia. Furthermore the lack of a biological union between the sutured patch and the diaphragm tissue remnants is likely to be the cause of a reherniation. Meticulous inspection for a hernial sac during the initial operation could reduce the incidence of a reherniation.

Collagen↗
PubMed1997

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.

Antifibrinolytic Agents↗
PubMed1996

Additional congenital defects in anorectal malformations.

UNLABELLED: From 1974 until 1995 a total of 264 (141 male, 123 female) patients born with an anorectal malformation (ARM) were referred to the University Hospital Nijmegen in the Netherlands. All additional congenital defects (ACDs) were registered. Special attention was paid to whether the ACDs take part in associations, syndromes, or sequences. One or more ACDs were observed in 67% of the patients. In decreasing order the defects concerned the uro-genital tract (43%), skeleton (38%), gastrointestinal tract (24%), circulation (21%), extremities (16%), face (16%), central nervous system (15%), respiratory tract (5%), and remaining defects (5%). Associations were observed in 49% of the patients mostly (in 44%) the Vertebral, Anorectal, Cardial, Tracheo-Esophageal, Renal and Limb association. In 5% of the patients syndromes were recognized. Sequences were seen in 2% of the patients. Remarkable is the combination of trisomy 21 and ARM without a fistula. The combination of ARM and the Zellweger syndrome has not been reported before. CONCLUSION: Almost all combinations of ARM and ACDs can be classified as an association, syndrome or sequence. ARM-causing agents affect males and females in equal numbers but lead to different expression in the sexes. The origin of the Omphalocele, Extrophia of the bladder, Imperforate anus, Sacral anomalies complex probably differs from that of other forms of ARM.

Abnormalities, Multiple↗