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F H vd Staak

Publications and source records attributed to F H vd Staak.

9 recordsLinked to original sources

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↗

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↗

Improving survival for patients with high-risk congenital diaphragmatic hernia by using extracorporeal membrane oxygenation.

The benefit of extracorporeal membrane oxygenation (ECMO) in cases of high-risk congenital diaphragmatic hernia (CDH) was studied by comparing pre-ECMO (1987-1990) and post-ECMO (1991-1994) 3-month survival statistics. Fifty-five CDH patients who presented in respiratory distress within 6 hours after birth were referred--18 in the pre-ECMO era and 37 in the ECMO era. During the entire study period (December 1987 through July 1994) the patients were treated by the same protocol of preoperative stabilization and delayed surgery; the only difference was the addition of ECMO beginning in January 1991. The patients were stratified based on the response to conventional treatment: 1, no response (irretrievable); 2, stable; 3, unstable. The 3-month survival rate for the unstable neonates (who could not be stabilized by conventional therapy) improved from 0% (0 of 9) in the pre-ECMO era to 61% (11 of 18) in the ECMO era (P = .004). This highly significant difference shows that ECMO is a very valuable addition to the management of high-risk CDH patients whose conditions remain unstable despite maximal conventional therapy.

Extracorporeal Membrane Oxygenation↗

Do we use the right entry criteria for extracorporeal membrane oxygenation in congenital diaphragmatic hernia?

In a retrospective review we analysed alveolar-arterial oxygen difference (AaDO2) as an entry criterion for extracorporeal membrane oxygenation (ECMO) in neonates with several forms of acute respiratory insufficiency. Although for meconium aspiration syndrome, respiratory distress syndrome, sepsis, and idiopathic pulmonary hypertension of the newborn we found values in accordance with the literature, patients with congenital diaphragmatic hernia (CDH) met 80% mortality criteria with significant lower AaDO2 values. Several patients died before ever reaching usual entry criteria for ECMO, because serious lung deterioration makes AaDO2 values unreliable. Awaiting classical ECMO entry criteria for patients with CDH may at least partially explain the lower survival rate for ECMO in CDH.

Extracorporeal Membrane Oxygenation↗

Are adults content or continent after repair for high anal atresia? A long-term follow-up study in patients 18 years of age and older.

OBJECTIVE: This study investigated the current state of fecal and urinary continence in an extensive group of adults after operative correction for high anorectal malformations and how they cope with their incontinence. SUMMARY BACKGROUND DATA: Normal fecal continence is hardly to be expected after correction for high anorectal malformation; despite this, it is commonly accepted that for most patients fecal continence improves with growing age and that most adult patients have no problems. Until now, however, few long-term follow-up studies in small groups of adults have been performed to assess continence after operative repair for high anorectal malformation. METHODS: Fifty-eight adult patients (median age, 26.0 years; range, 18.1 to 56.9 years) with an operatively corrected high anorectal malformation were evaluated by questionnaire with respect to their current state of fecal and urinary continence and mode of control of defecation. RESULTS: Seven patients have a permanent ileostoma or colostoma. Of the 51 patients with anal defecation, 61% control defecation by themselves, whereas 35% control defecation by using enemas or bowel irrigations, and 4% do not have any control at all. Besides medical therapy, 65% take dietary measures to influence defecation. According to existing scoring methods, 41% reached good and 49% fair control of defecation, whereas only 10% had poor control. Current control of defecation was reached from a median age of 15.0 years (range, 5 to 31 years). CONCLUSION: Conclusively, the authors can say that after correction for high anorectal malformation nobody reached normal fecal continence. Most patients with anal defecation reached good and fair control of defecation, however. Of all 58 patients, 84% are satisfied with their level of cleanliness.

Adolescent↗

Enterostomy complications in infants.

A retrospective review of 48 infants, less than one year of age, with enterostomies was carried out to analyse the complications. Early complications were encountered in 3 patients (5%). Stoma complications occurred in 7 patients (14.6%) and revision was required in 2 cases. Total or near total parenteral nutrition was needed in 23 patients (48%). Of the 25 who tolerated oral feeding, 16 (64%) required extra sodium in their diet. Stoma-closure in 37 patients had a leakage rate of 8% and no mortality. Careful attention to technical details, diligent postoperative stoma care and early closure of the enterostomy if possible, seems to be the best way of avoiding complications.

Enterostomy↗

Nonsurgical (conservative) treatment of giant omphalocele. A report of 10 cases.

Between January 1970 and December 1984, 65 patients with a congenital anterior abdominal wall defect were admitted to the Pediatric Surgical Center of the St. Radbound Hospital, Nijmegen, The Netherlands. There were 39 cases of omphalocele. Ten had giant omphalocele, defined as omphalocele that could not be closed primarily. In these cases, a conservative method of treatment was used. The results are reported of these 10 cases of giant omphalocele with special emphasis on mortality, complications, and length of hospital stay. Mean hospital stay was 95 days. Local infection did not present serious problems. Sepsis occurred in half the patients but was managed with antibiotics. There was one case of late volvulus of the stomach. All local applications, if used regularly, gave rise to complications. Two cases of hypothyroidism caused by the application of povidon-iodine and one case of alcohol intoxication were observed. In early years, one child was lost due to mercury poisoning. There was one early and one late death unrelated to the procedure.

Abnormalities, Multiple↗