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Biomedical subjects

F W Hazebroek

Publications and source records attributed to F W Hazebroek.

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↗
PubMed2001

[Differential diagnosis and treatment of girls with 46XY-karyotype and androgen insensitivity syndrome].

The importance of the secretion and action of androgens during the critical period of male sexual development is exemplified in patients with androgen insensitivity syndrome. Their karyotype is always 46XY. In 2 sisters, aged 11 and 13 years, the androgen insensitivity syndrome was diagnosed based on an androgen receptor gene mutation. Ambiguous genital development of a new-born was shown to be due to a lack of testosterone production, based on a luteinizing hormone receptor gene mutation. Finally, in a phenotypically female new-born a gene mutation of 17-beta hydroxysteroid dehydrogenase type 3 was found to be responsible for insufficient testosterone synthesis during embryonic development. The presentation of a patient, and specifically a neonate, with abnormal genital development represents a difficult diagnostic and therapeutic challenge. Referral to a centre with experience in the diagnosis and management of disorders of sexual development is advised where the emphasis should be on psychological and genetic counselling.

17-Hydroxysteroid Dehydrogenases↗
PubMed2001

The neonate with major malformations: experiences in a university children's hospital in the Netherlands.

Advancements in medical technology over the last decades have greatly benefited perioperative care of newborns undergoing major surgical interventions. Yet, a proportion of these babies will not survive, and doctors are forced to face the difficult ethical question of whether, in cases of severe congenital malformations or acquired diseases, the expected quality of life justifies the decision to continue, withhold, or withdraw treatment. In a tripartite approach, the authors present their relevant experiences with these newborns in the pediatric surgical department of the Sophia Children's Hospital (SCH). First the authors evaluated the mortality pattern and causes of death in surgical neonates over 2 periods (1986 through 1990 and 1996 through 2000). The mortality rate was the same, 10%. Pattern of mortality was classified into 3 groups: nonpreventable, permissible, and preventable death. The most striking difference between both mortality groups existed between the percentage of preventable deaths, 14.5% in the earlier period, versus 5% in the most recent period. Half of the preventable deaths in the former period occurred in relation to postoperative, infectious treatment complications, such as inadequate sepsis management. These errors were not seen anymore in our recent evaluation. Second, the authors studied the physical and psychosocial adjustment of former patients, particularly those operated on for digestive tract anomalies. The follow-up period encompasses from 8 to 12 years showing that the physical functioning of these children was relatively good. With respect to their cognitive and psychosocial functioning it seems justified to conclude that they are at risk for lower cognitive functioning, learning problems, and possibly lower educational levels. Third, the authors decided that ethical questions related to surgical treatment of necrotizing enterocolitis (NEC) actually fits within the scope of this article. They hypothesized that a particular minimum birth weight could be a decisive factor for refraining from surgery on ethical grounds in the treatment of this particular disease. A total of 116 patients with NEC were identified over a 5-year study period. Seventy-five fell into the lowest birth weight group (<1,500 g). The operative mortality rate (21 patients) increased with decreasing birth weight: group A, 29% (A1, 31%; A2, 26%); group B, 23%; and group C, 0%. The authors were particularly interested in the long-term follow-up of the A1 group (BW < 1,000 g), and 18 of the 30 survivors of this group were available for follow-up. There were 2 survivors with short bowel syndrome. Early assessment and neurodevelopmental outcome, at least 2 years after surgery, showed that in 14 of these 18 (78%) there were no major handicaps. The other 4 patients had one or more major handicaps (cerebral palsy, mental and/or visual handicap, and hearing loss). NEC-related mortality after surgery is highest for patients with birth weight less than 1,000 g (31%). However, in view of the overall good 2-year follow-up results of the survivors, we feel unable to define a minimum birth weight that, as such, could serve as a cutoff point for deciding to forbear, on ethical grounds, surgical treatment for NEC. The picture represented by our tripartite investigation of life and death in and after the intensive care unit period serves as a mirror and truthfully reflects ethical questions of our medical practices.

Birth Weight↗
PubMed2000

Nissen fundoplication in the management of gastroesophageal reflux occurring after repair of esophageal atresia.

BACKGROUND/PURPOSE: Gastroesophageal reflux is a major cause of complications after esophageal atresia repair. The suitability of the Nissen fundoplication in these patients is still disputed. Therefore, the authors evaluated the results of their prospective treatment protocol in those patients who underwent a Nissen fundoplication. METHODS: From 1984 to 1996, 125 patients underwent anastomosis for esophageal atresia. A Nissen fundoplication was later performed in 29 patients. The prospective protocol included x-ray after 10 days, 6 weeks, 12 weeks, 6 months, and 12 months. Forty-eight-hour pH measurements were performed between 6 and 12 weeks. Mean postfundoplication follow-up was at least 5 years (range, 2 to 13 years). RESULTS: Two of the 29 patients died after the Nissen fundoplication from unrelated causes. A third patient was excluded from the study group. Nineteen of the remaining 26 patients showed severe stricture. pH-metry succeeded in 18 patients, showing pathological reflux in 17. In 24 patients the fundoplication was performed between 1 and 24 months (median, 4 months), in the other 2 patients much later. In 4 of the 26 patients(15%) the Nissen proved to be insufficient and had to be redone. The remaining 22 patients had no short-term or long-term complications. CONCLUSION: The authors' findings in this group of patients, comparing them with the results reported in the literature, indicate that there is no reason to change their prospective treatment protocol nor their policy to perform Nissen fundoplications at an early stage.

Esophageal Atresia↗
PubMed2000

Children with congenital diaphragmatic hernia are at risk for lower levels of cognitive functioning and increased emotional and behavioral problems.

The physical, psychological, and social functioning of 11 children aged 8 to 12 years with Congenital Diaphragmatic Hernia (CDH) was assessed with several standardized assessment procedures. Physically, most children functioned well at follow-up with half of the children showing minor physical problems such as bronchial hyperreactivity. The mean IQ of the children was 15 points (1 SD) below the norm of 100. Only 6 children were at expected school level. The children showed more emotional and behavioral problems than in the general population as reported by parents and teachers. The children themselves reported more depressive problems, but not a lower self-esteem than children in the general population. These results were confirmed by the results of interviews with parents and children concerning psychosocial functioning. It is concluded that children with CDH show more cognitive and learning problems and increased rates of emotional and behavioral problems compared to children in the general population. Since no children treated with Extra Corporeal Membrane Oxygenation (ECMO) were involved in this study, the earlier reports that lower cognitive functioning is limited to children with CDH treated with ECMO can not be confirmed by this study. Considering the results of this study, there is a need for further follow-up studies concerning the long-term psychological and social functioning of children with CDH.

Child↗
PubMed2000

The impact of diagnostic delay on the course of acute appendicitis.

BACKGROUND: The diagnosis of acute appendicitis is often delayed, which may complicate the further course of the disease. AIMS: To review appendectomy cases in order to determine the incidence of diagnostic delay, the underlying factors, and impact on the course of the disease. METHODS: Records of all children who underwent appendectomy from 1994 to 1997 were reviewed. The 129 cases were divided into group A (diagnostic period within 48 hours) and group B (diagnostic period 48 hours or more). RESULTS: In the group with diagnostic delay, significantly more children had first been referred to a paediatrician rather than to a surgeon. In almost half of the cases in this group initial diagnosis was not appendicitis but gastroenteritis. The perforation rate in group A was 24%, and in group B, 71%. Children under 5 years of age all presented in the delayed group B and had a perforation rate of 82%. The delayed group showed a higher number of postoperative complications and a longer hospitalisation period. CONCLUSIONS: Appendicitis is hard to diagnose when, because of a progressing disease process, the classical clinical picture is absent. The major factor in diagnostic delay is suspected gastroenteritis. Early surgical consultation in a child with deteriorating gastroenteritis is advised. Ultrasonographs can be of major help if abdominal signs and symptoms are non-specific for appendicitis.

Adolescent↗
PubMed1999

Long-term physical, psychological, and social functioning of children with esophageal atresia.

BACKGROUND/PURPOSE: Little is known about the long-term psychosocial functioning of children with esophageal atresia (EA). The physical, psychological, and social functioning of children with EA was studied using standardized assessment procedures, and relations between medical and social background variables and outcome were investigated. METHODS: Subjects were 36 children (20 boys, 16 girls; mean age 10.2 years) with EA. Fourteen children had minor and 11 children had major associated congenital anomalies. According to the "Montreal" classification, eight children with major congenital anomalies who also had been dependent on artificial ventilation as newborns fell into the high-risk class. The children were subjected to an intelligence test. Parents completed standardized questionnaires concerning emotional and behavioral problems, psychosocial stress, and family functioning; children completed questionnaires concerning depression and self-esteem; and teachers completed questionnaires concerning emotional and behavioral problems. Results were compared with normative data from the general population, and correlations between background and outcome variables were computed. RESULTS: According to Desjardins' classification, 16 children had excellent, nine children had good, and four children had fair outcome. The mean intelligence quotient (IQ) of the children was 90.2, which is almost 10 points lower than the standardized norm of 100 (P < .01). High-risk children (n = 7) had a significantly lower IQ (mean IQ, 79.4; P < .05). Five times as many children (n = 8; 22%) as in the general population (4%) required special education (P < .001). More than twice as many children (30% to 35%) as in the general population (15%) showed elevated rates of emotional and behavioral problems as reported by parents and teachers (P < .02). The children did not report more negative self-esteem or more depressive symptoms than children in the general population. Children with a lower IQ reported lower scholastic competence (r = .38, P < .05) and showed more emotional and behavioral problems as reported by teachers (r = -.43, P < .05). Family functioning and levels of psychosocial stress were the same as in the general population. Children in worse functioning families showed more emotional and behavioral problems as reported by parents (r = .37, P < .05) and higher depression scores as reported by themselves (r = .47, P < .01). CONCLUSIONS: In a follow-up study using standardized assessment procedures, it was shown that children with EA have more learning, emotional, and behavioral problems than children in the general population. A high-risk group of children with major associated congenital anomalies who had been ventilated as a newborn, were at special risk for cognitive problems.

Affective Symptoms↗
PubMed1999

Choledochal cysts in children: a review of 28 years of treatment in a Dutch children's hospital.

OBJECTIVE: To analyse the results of surgical treatment of choledochal cysts. DESIGN: Retrospective study. SETTING: Children's hospital, The Netherlands. PATIENTS: 14 children presenting with choledochal cysts. INTERVENTIONS: Choledochoduodenostomy, Roux-en-Y choledochojejunostomy or Roux-en-Y hepaticojejunostomy. MAIN OUTCOME MEASURES: Morbidity and mortality RESULTS: The mean age of the patients was 20 months (2 weeks to 7 years). 10 patients had a type I choledochal cyst; three a type IV, and one a type V. Mean follow-up period was 6 years (18 months to 16 years). One patient with a type I cyst died of Klebsiella pneumoniae that was resistant to treatment. One patient with a type I cyst treated by choledochojejunostomy had two episodes of cholangitis. Another patient with a type I cyst, treated by choledochoduodenostomy, had one episode of cholangitis. Both could be treated with antibiotics. The other patients had had no complications up to 1997. CONCLUSION: This rare anomaly may lead to severe complications when left untreated or after late treatment. It is easy to manage with low associated morbidity.

Child↗
PubMed1999

Brief report: parental burden and grief one year after the birth of a child with a congenital anomaly.

OBJECTIVE: To assess parental burden and grief one year after having a child with a congenital anomaly. METHOD: Twenty-five couples completed the Impact on Family Scale (IFS) and 22 couples answered the Perinatal Grief Scale (PGS). In addition, 27 mothers completed the Functional Health Status Scale (FSII-R). RESULTS: Mothers and fathers showed no significant differences in overall burden (IFS) and grief (PGS). Regarding the subscales, mothers reported significantly more personal strain. Foreknowledge from prenatal diagnosis about the anomaly, a low perceived functional health status of the child, and multiple congenital anomalies increased the burden and grief. CONCLUSIONS: A perinatal counseling team that provides clear and consistent information about the anomalies, the treatment, and the prognosis would help to reduce unnecessary stress and uncertainty, particularly for parents who received prenatal information and whose infant has multiple congenital anomalies.

Abnormalities, Multiple↗
PubMed1999

Normal ranges of 24-hour pH-metry established in corrected esophageal atresia.

BACKGROUND: This study was undertaken to determine baseline values of 24-hour pH-metry in children who had undergone correction for esophageal atresia. METHODS: A 24-hour pH-metry without medication was conducted in 13 patients with an uncomplicated postoperative course after correction of esophageal atresia. The pH-metry was performed in the hospital with a flexible glass electrode. RESULTS: The mean reflux index was 4.08%, the mean total number of reflux periods with a pH less than 4 was 21, and the mean number of periods of pH less than 4 lasting longer than 5 minutes was 2.5. CONCLUSIONS: Twenty-four-hour pH-metry values in asymptomatic esophageal atresia patients are the same as in children from the same age group with normal anatomy.

Esophageal Atresia↗
PubMed1999

Gastroesophageal reflux and Barrett's esophagus in adults born with esophageal atresia.

OBJECTIVE: Postoperative morbidity after correction of esophageal atresia is partly determined by gastroesophageal reflux disease, which has been proven to affect from one-half to two-thirds of patients during childhood. We conducted a follow-up study to test our hypothesis that, if former patients still show gastroesophageal reflux at adult age, they are at high risk for developing Barrett's esophagus, which is considered to be premalignant. METHODS: Of 69 patients born between 1971 and 1978, all having undergone a primary anastomosis, 24 had died, five of them because of aspiration. Of the 45 survivors, 39 could be traced; they all completed a questionnaire inquiring after symptoms related to the esophagus. Of these patients, 34 underwent an additional esophagogastrocopy. RESULTS: Only nine of the 39 patients had no symptoms at all; 30 had mild to severe dysphagia symptoms, and 13 had mild to severe reflux symptoms. Esophagogastrocopy in 34 patients revealed that the anastomosis was still recognizable in all cases, but stenoses were not found. Six patients showed a small hiatal hernia, and one a large one. The incidences of reflux symptoms (13/39, p < 0.01), reflux esophagitis (9/34, p < 0.01) and Barrett's esophagus (2/34, p < 0.001) were significantly higher than in the normal population. CONCLUSIONS: This group seems to be at risk for developing Barrett's esophagus. As this is the first follow-up study of a consecutive group of adult esophageal atresia patients, we think it is advisable to perform an esophagogastroscopy in all patients at adulthood until more long term follow-up data are available.

Adolescent↗
PubMed1998

Prospective medical and surgical treatment of gastroesophageal reflux in esophageal atresia.

BACKGROUND: Gastroesophageal reflux is a major cause of anastomotic complications after repair of esophageal atresia. For this reason, we evaluated a prospective, postoperative treatment protocol with the emphasis on comparing medical and operative treatment. STUDY DESIGN: From 1994 to 1995, 26 consecutive patients underwent correction of esophageal atresia in the Sophia Children's Hospital. These patients were enrolled in a decision-making protocol meant to establish the effect of medical treatment on gastroesophageal reflux and stricture formation, the relation between anastomotic tension and reflux, and the question of whether performing a Nissen fundoplication was justified. Patients who showed reflux on the first postoperative x-ray were given medical treatment. Reflux was assessed after 6-12 weeks by x-ray and 48-hour pH-metry (24 hours with and 24 hours without medication). Evaluations were repeated at 18 weeks, 6 months, and 1 year. Twenty-three patients were followed for > or = 1 year. RESULTS: Twenty-four patients had classic esophageal atresia combined with tracheoesophageal fistula. Two had isolated atresia and underwent a colonic interposition. One of the others died of severe cerebral hemorrhage early after the operation. Twenty-two of the remaining 23 showed reflux on the first postoperative x-ray and were given medical treatment. The mean 3.8% total mild reflux time (range, 0.0-11.0%) decreased to a mean of 1.47% (range, 0.0-6.8%). Medical treatment given according to protocol did not influence severe reflux. Eleven of 23 patients showed stricture formation, requiring a mean of four dilatation procedures (range, 1-9). Defining a real stricture as one needing three or more dilatations, as seen in seven patients, the following results were seen: four nonrefluxing patients (proved by x-ray and pH-metry) needed a mean of 4.2 dilatations (range, 3-7), and three refluxing patients (proved by x-ray and pH-metry) needed a mean of 7.3 dilatations (range, 5-9). Three of seven patients with anastomotic tension had proved gastroesophageal reflux; reflux was also diagnosed in 8 of 15 patients without any tension on the anastomosis. Nine of 23 patients underwent a Nissen fundoplication according to the protocol. In four of them, this was decided because of severe reflux-associated respiratory problems; in one, for resistant stenosis after a Livaditis procedure; and in one with normal pH-metry, the procedure was done on clinical grounds. The latter patient needed an aortopexy at a later stage. A late fundoplication was performed in two patients for persistent gastroesophageal reflux unresponsive to medical treatment, and in one for persistent stenosis and reflux. In all patients, the outcomes were successful, without complications. CONCLUSIONS: Medical treatment of gastroesophageal reflux after repair of esophageal atresia has a distinct effect on the duration of reflux and could have a positive effect on the occurrence and treatment of stenosis. There is no clear relation between the occurrence of reflux and tension on the anastomosis. Nissen fundoplication according to the protocol was done appropriately in eight of nine patients.

Esophageal Atresia↗
PubMed1997

Malformation and stenosis of the cricoid cartilage in association with Larsen's syndrome.

Three patients with the typical features of Larsen's syndrome are described. All three developed severe respiratory symptoms caused by a congenital subglottic stenosis. Tracheotomy and treatment of the stenosis by means of laryngotracheoplasty resulted in complete collapse of the cricoid cartilage and the proximal tracheal skeleton. Lack of rigidity of the laryngeal and tracheal cartilages in patients with Larsen's syndrome could well be responsible for this failure. Surgical treatment consisted of resection of the stenotic and collapsed areas and end-to-end anastomosis. This therapy was eventually successful in all three patients.

Abnormalities, Multiple↗
PubMed1996

Staff attitudes towards continuation of life-support in newborns with major congenital anomalies.

UNLABELLED: This study was conducted to gain insight into the attitudes of medical staff towards life-support of newborns with life-threatening problems, seen against the background of these children's expected morbidity and quality of life. The opinions about the mode of life-support were determined by questionnaires and the demographic characteristics of the respondents were noted. Each patient's risk of mortality was scored by means of the standardized Paediatric Risk of Mortality Score (PRISM). Attitudes towards support were unanimous for 39 of the 46 patients. For the other 7 patients at least one of the respondents preferred a different support mode than that given at the time. The attitudes were influenced by the patient's risk of mortality. CONCLUSION: Decisions about life-support of newborns with life-threatening problems should include all the disciplines involved in patient care and should be made at an early stage.

Attitude of Health Personnel↗
PubMed1995

Staphylococcal pneumonia in childhood: will early surgical intervention lower mortality?

Staphylococcus aureus pneumonia (SAP) continues to be a serious bacterial infection which is associated with a high incidence of complications. We retrospectively reviewed the case records of 36 infants and children admitted with SAP to the Sophia Children's Hospital between 1970 and 1992 to analyse changes over time in the clinical presentation, diagnostic work-up, management and complications. Fifteen of these 36 children (42%) were less than 1 year old. Fever (97%) and respiratory distress (83%) were the most common symptoms at the initial presentation. Chest X-ray findings on admission or during hospitalisation included pleural effusion (75%), pneumothorax (47%), and abscess and/or pneumatocele (39%). Diagnostic and/or therapeutic thoracentesis of pleural fluid was performed in 17 of the 36 patients (47%). Twenty-one patients (58%) needed chest tube drainage. Twelve had a thoracotomy (33%). Artificial ventilation was needed in 13 of the patients (36%). Extrapulmonary complications included convulsions in 6 patients (17%) and osteomyelitis in 2 children (6%). The mean duration of hospitalization was 36 days. Two of the 36 children died (6%). The low mortality rate in this study may be the result of the relatively high rate of thoracotomy and of improvements in supportive treatment.

Child↗
PubMed1995

Hepatic artery ligation for hepatic vascular tumors with arteriovenous and arterioportal venous shunts in the newborn: successful management of two cases and review of the literature.

The authors report their experience with hepatic artery ligation in two newborns, as well as relevant findings from a literature review. A 2-day-old boy had progressive cardiac and respiratory difficulty. A firm liver was palpable, with an overlying thrill. Sonography and arteriography showed diffuse arteriovenous shunting in both liver lobes. Hepatic artery ligation provided remarkable hemodynamic and clinical improvement. Another boy was admitted 3 weeks after birth because of bilious vomiting with abdominal distension and bloody stools. Abdominal examination showed a large liver with a systolic bruit and thrill. X-rays showed cardiac enlargement and dilated bowel loops with air-fluid levels. Arteriography and sonography showed arteriovenous and arterioportal venous shunting. Laparotomy was performed, and a large vascular malformation was palpated in both liver lobes. The entire bowel was congested and cyanotic, but there were no signs of obstruction. This patient had acute portal hypertension imitating intestinal obstruction. Ligation of the hepatic artery improved the color of the bowel, and the thrill disappeared. Five and nearly 4 years after the operation, both boys are growing normally without medication or diet. Sonography showed almost complete resolution of the hemangiomas.

Arteriovenous Malformations↗
PubMed1995

End-of-life decisions for surgical neonates: experience in The Netherlands and United States.

PURPOSE: To characterize end-of-life decisions for surgical neonates and compare similarities and differences in practice between pediatric surgeons in The Netherlands and the United States. METHODS: The authors evaluated the deaths of all neonates admitted to the surgical intensive care unit (SICU) of two major children's hospitals: Sophia Children's Hospital (SCH) in The Netherlands and Columbus Children's Hospital (CCH) in the United States. Between January 1990 and July 1993, neonatal SICU admissions totaled 362 (SCH) and 125 (CCH). Neonates who died were classified as follows: group 1 = poor prognosis, expected death, and group 2 = good prognosis, expected survival. RESULTS: The mortality rates were comparable for each SICU: 12% (SCH) and 14% (CCH). The average survival period was shorter in group 1 (1.5 days) than in group 2 (26.5 days). Criteria for assignment to group 1 differed, with "expected poor quality of life" used at SCH, and "futility" at CCH. Criteria for group 2 were similar and included significant postoperative complications. Although the percentages with a do-not-resuscitate (DNR) status were comparable (SCH, 51%; CCH, 55%), the application of the DNR order differed in each SICU. The majority of neonates at SCH had either withholding or withdrawal of life support, whereas no further escalation in treatment was offered for infants with a DNR order at CCH. The average survival period after the DNR order was 4 days at SCH and 7 days at CCH. CONCLUSION: DNR orders were used for more than half the surgical neonates with critical illness. Criteria for DNR status and implementation of patient care after the DNR order differed between the SICUs.

Euthanasia, Passive↗