Frans W.J. Hazebroek

Congenital diaphragmatic hernia: Impact of preoperative stabilization. A prospective pilot study in 13 patients

In case of congenital diaphragmatic hernia (CDH), survival generally depends not on prenatal diagnosis, planned delivery, and immediate postnatal operation, but on the gravity of pulmonary hypoplasia and persistent hypertension (PPH). Many vasoactive drugs have become available for lowering PPH, but the mortality rate for CDH still amounts to 40% to 70%. Preoperative stabilization might prevent or at least reduce the risk of PPH. This method was evaluated in a pilot study lasting 15 months and involving 13 patients. All were admitted to the pediatric surgical intensive care unit within six hours of birth, all requiring mechanical ventilation. Continuous suction of the stomach and bowel proved successful in reducing the mediastinal shift. Study parameters were alveolar-arterial oxygenation differences ((A-a)DO2), mean airway pressure (MAP), oxygenation index (OI), and ventilation index (VI), measured on admission and at set times before and after surgery. Eight patients did not survive, but in two cases death was not directly related to CDH. The following conclusions were reached: (1) satisfactory ventilation parameters on admission will remain good during the preoperative stabilization phase and will not be affected by its duration or by subsequent surgery, spelling survival; (2) unsatisfactory ventilation parameters on admission may improve with preoperative stabilization, giving these patients a better chance of survival; and (3) poor ventilation parameters on admission that fail to improve with preoperative stabilization will not improve with surgery or postoperatively, spelling death.

Ovarian germ cell tumors in children: A clinical study of 66 patients

Ovarian germ cell tumors are rare in childhood. The aim of this study is to review clinical presentation, management, and outcome in a two‐center series of girls with ovarian germ cell tumor.

Glutamine Supplementation of Parenteral Nutrition Does Not Improve Intestinal Permeability, Nitrogen Balance, or Outcome in Newborns and Infants Undergoing Digestive-Tract Surgery: Results From a Double-Blind, Randomized, Controlled Trial

Objective:To assess the effect of isocaloric isonitrogenous parenteral glutamine supplementation on intestinal permeability and nitrogen loss in newborns and infants after major digestive-tract surgery. Summary Background Data:Glutamine supplementation in critically ill and surgical adults may normalize intestinal permeability, attenuate nitrogen loss, improve survival, and lower the incidence of nosocomial infections. Previous studies in critically ill children were limited to very-low-birthweight infants and had equivocal results. Methods:Eighty newborns and infants were included in a double-blind, randomized trial comparing standard parenteral nutrition (sPN; n = 39) to glutamine-supplemented parenteral nutrition (GlnPN; glutamine target intake, 0.4 g kg−1 day−1; n = 41), starting on day 2 after major digestive-tract surgery. Primary endpoints were intestinal permeability, as assessed by the urinary excretion ratio of lactulose and rhamnose (weeks 1 through 4); nitrogen balance (days 4 through 6), and urinary 3-methylhistidine excretion (day 5). Secondary endpoints were mortality, length of stay in the ICU and the hospital, number of septic episodes, and usage of antibiotics and ICU resources. Results:Glutamine intake plateaued at 90% of the target on day 4. No differences were found between patients assigned sPN and patients assigned GlnPN regarding any of the endpoints. Glutamine supplementation was not associated with adverse effects. Conclusions:In newborns and infants after major digestive-tract surgery, we did not identify beneficial effects of isonitrogenous, isocaloric glutamine supplementation of parenteral nutrition. Glutamine supplementation in these patients therefore is not warranted until further research proves otherwise.

Postoperative morbidity in patients with esophageal atresia

A retrospective study was carried out in 110 patients with esophageal atresia seen at the Sophia Childrens Hospital, Rotterdam, from 1975 to 1984. Special attention was paid to post-operative morbidity in view of the lack of detailed information concerning this aspect in the literature. Of the 87 surviving patients, 77 had a primary end-to-end anastomosis. The experience with these 77 patients is reviewed and discussed with reference to the literature. Gastroesophageal reflux seems to be the major postoperative complication and a contributing factor to other serious conditions. Early detection and treatment of gastroesophageal reflux may prevent some of these conditions.

Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors.

AIMS Although germ cell tumors (GCT) supposedly share the same cell type of origin, their clinical course differs considerably depending on tumor site and histology. The aim of this work was to study long-term survival stratified for tumor site and tumor histology. MATERIALS AND METHODS The medical records of 193 consecutive infants and children with extracranial GCT were studied. The GCT arose in the following anatomical sites: sacrococcygeal (n = 70), ovary (n = 66), testis (n = 20), retroperitoneum (n = 12), neck (n = 8), mediastinum (n = 7), and miscellaneous (n = 10). Histological analysis revealed 152 teratomas (mature: 115, immature: 37), 27 yolk sac tumors, 8 mixed tumors, 2 dysgerminomas, 2 gonadoblastomas, 1 choriocarcinoma and 1 embryonal carcinoma. RESULTS Overall survival (OS) for the whole patient group was 0.91 +/- 0.02, and event-free survival (EFS) was 0.88 +/- 0.02 at ten years. Patients with gonadal GCT had a higher probability of OS than those with extragonadal GCT (p = 0.029). Patients with cervical and mediastinal tumors had a lower probability of EFS than those with gonadal, retroperitoneal or sacrococcygeal GCT (p = 0.018). Patients with choriocarcinoma, embryonal carcinoma, immature teratoma, yolk sac tumor and mixed GCT had a lower probability of EFS than patients with mature teratoma or gonadoblastoma (p < 0.001). CONCLUSIONS Mortality in children with extracranial germ cell tumors is not only dictated by malignant histology, but also, as in the case of mature teratomas, by occurrence at certain sites.

Strategy for management of newborns with cervical teratoma

Abstract Background: Cervical teratomas are extremely rare tumors with high perinatal mortality and morbidity rates. Objective: To compare our experience and outcome in newborns with cervical teratoma with similar reports from the literature, in order to propose a structured approach. Methods: A retrospective review of seven patients treated between 1986 and 2000 was performed. The results of these seven patients were compared with and added to a series of 44 well-documented patients retrieved from the literature. Results: In four of the seven patients, the diagnosis was not suspected antenatally. Three of the patients survived, one died. In the other three, the diagnosis was made antenatally. Two were born using the ex-utero intrapartum treatment (EXIT) procedure, one by planned cesarean section. Only one of these three survived. Mortality in the total series of 51 patients was 33% overall, and 46% in the group in which the diagnosis had been made antenatally. Peri- and post-operative complications were reported in 27%. Although larger tumors caused polyhydramnios more frequently than smaller tumors, and were associated with more severe respiratory distress, the relationship between tumor volume at birth and final outcome could not be established. This makes difficult the identification of fetuses with a disastrous prognosis. Conclusion: Although mostly benign, cervical teratomas are still associated with high mortality rates. Timely antenatal diagnosis is indispensable in reducing morbidity and mortality caused by upper airway obstruction. A structured approach to the management of cervical teratoma is proposed.

Nonpalpable testes: is there a relationship between ultrasonographic and operative findings?

BackgroundUltrasonography (US) as a diagnostic tool in the work-up of boys with nonpalpable testes (NPT) is still controversial.ObjectiveTo evaluate the relation between US and operative findings in boys with NPT.Materials and methodsDuring a 7-year period we saw 135 boys with 152 NPT. All were examined by the referring physician or a paediatric surgeon, underwent US examination, and were then re-examined afterwards by a specialist. Finally, all boys were surgically explored for testicular position.ResultsUS located 103 NPT (68%), 16 within the abdomen and 87 in the inguinal canal. With knowledge of the US result, 37 testes were palpable on re-examination. The sensitivity of US was 97% for inguinal and 48% for abdominal viable testes. Of the 49 testes (32%) missed by US, 16 were viable in either the abdominal (n = 14) or the inguinal (n = 2) position.ConclusionAll boys with presumed NPT should be referred to a specialist. US is useful to determine localization of NPT, which facilitates planning the surgical procedure. An inguinal exploration is called for when US identifies the testis in the inguinal canal. Because the sensitivity of US for viable abdominal testes is only 48%, we now always perform laparoscopic exploration when US is negative.

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 Childrens 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.

Fertility potential in men with a history of congenital undescended testes: a long-term follow-up study

Men with a history of congenital undescended testes (UDT) have an increased risk of fertility problems. Despite no definitive proof, current guidelines recommend early surgical intervention because this may have a positive effect on future fertility potential by preventing degenerative changes of the testes in early life. Also surgical intervention facilitates observability of the testes in view of possible malignancy. We evaluated testicular function in adult men with previous UDT treated at different ages before puberty. A long‐term follow‐up study of men with previous UDT was performed. Andrological evaluation included medical history taking, physical examination, scrotal ultrasound, determination of reproductive hormones, and semen analysis. Findings were compared with those of a control group of men with normal testicular descent. The influence of age at orchiopexy on future fertility parameters was evaluated in a multivariate regression analysis. 62 men were included of whom seven had had bilateral UDT. Twenty‐four patients had had their orchiopexy before the age of 24 months of whom eight men had it before 12 months of age. Forty‐eight men had had unsuccessful luteinizing‐hormone‐releasing‐hormone (LHRH) nasal spray treatment during childhood, whereas 14 of 24 men operated before 24 months of age had not received LHRH treatment before orchiopexy. Fertility potential in men with a history of UDT is compromised in comparison with controls. We could not detect any influence of age at orchiopexy on fertility parameters. However, the number of patients operated before the age of 12 months is limited. This study does not support the assumption that early orchiopexy results in better fertility potential.

Why luteinizing-hormone-releasing-hormone nasal spray will not replace orchiopexy in the treatment of boys with undescended testes***

Two courses of LHRH nasal spray (400 micrograms three times a day for 28 days), partly administered in a double-blind placebo-controlled study, resulted in the descent of 48 of 281 testes (18%) in 237 prepuberal boys. Logistic regression analysis confirmed the clinical observation that pretreatment testicular position was the major factor influencing treatment results: the lower the pretreatment testicular position, the better the result. Of the unsuccessfully treated boys, 170 with 196 undescended testes subsequently underwent orchiopexy revealing anatomic anomalies that accounted for failure of hormonal treatment in 80% of the cases. Most frequent finding amounted to an underdeveloped processus vaginalis, extending no further than the level of the pubic bone, often associated with major epididymal deformities and a lack of obliteration of the processus vaginalis (107 of 196 operations). Obstruction of the scrotal entrance due to abnormal Scarpas fascia or abnormal gubernacular remnant was found in 35 and testicular absence in 15 cases. LHRH nasal spray might be effective when the testis can be manipulated to at least the scrotal entrance before treatment, but in view of our surgical findings, LHRH nasal spray will not replace orchiopexy.