[Acquired undescended testes: treatment and consequences for future fertility]. / Verworven niet-scrotale testis.

1-3% of boys develop an acquired undescended testes (UDT), meaning that the testes cannot be returned into the scrotum after previously having been located in a stable scrotal position. Fertility issues for patients with acquired UDT are comparable to those for patients with congenital UDT. Hypothetically speaking, patients with acquired UDT are at lower risk of testicular cancer than patients with congenital UDT. The appearance of an asymmetrical scrotum, which is associated with UDT, may negatively influence quality of life. Over 50% of the acquired UDTs will spontaneously descend at the start of puberty, thereby justifying conservative management of the condition. Orchidopexy directly after diagnosis does not have any advantages over awaiting spontaneous descent until puberty when fertility in later life of patients with unilateral acquired UDT is concerned; however, it may be beneficial in bilateral acquired UDT.

INSL3 and AMH in patients with previously congenital or acquired undescended testes.

BACKGROUND: In previous reports no differences in Leydig and Sertoli cell function were found between congenital undescended testis (CUDT) and acquired UDT (AUDT) on the basis of serum levels of LH, testosterone, FSH or inhibin B. This study tried to detect differences in Leydig and Sertoli cell function between CUDT and AUDT using insulin-like peptide 3 (INSL3) and anti-Müllerian hormone (AMH). METHOD: 118 men with a history of UDT (CUDT N=55 (6/55 bilateral), AUDT N=63 (15/63 bilateral)) were investigated. Differences between CUDT and AUDT, influence of age at surgery in CUDT, and effect of spontaneous descent or orchiopexy in AUDT were evaluated. RESULTS: For INSL3, no significant differences were found. AMH levels in bilateral CUDT were significantly lower compared with bilateral AUDT (6.4 (1.7-11.4) vs 13.2 (6.1-30.1) µg/l, p=0.02). AMH levels in unilateral CUDT were significantly higher than in bilateral CUDT (12.1 (2.4-43.7) vs. 6.4 (1.7-11.4) µg/l, p=0.02). CONCLUSION: No differences in Leydig cell function on the basis of INSL3 levels between the different UDT groups were found. Sertoli cell function evaluated by AMH, was more negatively affected in bilateral CUDT in comparison with bilateral AUDT and unilateral CUDT. LEVEL OF EVIDENCE RATING: Level III Treatment Study.

Ethical aspects of care in the newborn surgical patient.

This article places focus on three main subjects that are all related to the ethical aspects of care of newborns undergoing major surgical interventions. The first concerns the communication between the surgeon, as a representative of the treatment team, and the parents. The second is the way to handle new developments in neonatal surgery. The third issue covers several aspects of the ethical decision-making process with regard to forgoing life support in surgical neonates. These issues will be discussed on the basis of two clinical case reports.

Interdisciplinary structural follow-up of surgical newborns: a prospective evaluation.

BACKGROUND: Information on physical and developmental outcomes of children with anatomical congenital anomalies (CAs) may indicate the need for early intervention and reduce impact on the child's life and parental burden. METHODS: From 1999 to 2003, 101 children with CA (76.5% of initial survivors) were seen 6-monthly in a tertiary children's hospital. Growth, neurologic outcome, mental and psychomotor development as determined with the Bayley Scales of Infant Development, and categorization of predictive sociodemographic and medical variables of the children were evaluated prospectively and longitudinally. RESULTS: Congenital diaphragmatic hernia (CDH) and esophageal atresia patients showed impaired growth, that is, both height for age (-1.5 standard deviation score [SDS]) and weight for height (-1.0 SDS). Overall neurologic outcome was normal, however, suspect or abnormal for 40% of CDH patients. Overall mental development was normal, but psychomotor scores were significantly lower than the norm (95% confidence interval, 83.8-92.2 at 6 months and 87.9-98.5 at 24 months). Sex, maternal age, socioeconomic status, CA, severity-of-disease covariables, and need of medical appliances at home could predict negative outcome significantly (P < .05). CONCLUSIONS: The CA survivors show impaired growth and psychomotor developmental delay up to age 2 years. This warrants specific follow-up programs and infrastructure for these patients.

Late presentation of gastric tube ulcer perforation after oesophageal atresia repair.

Ulcer formation in intrathoracic grafts after oesophageal replacement is considered an infrequent complication of the procedure. We present a rare case of a gastric tube ulcer with perforation, more than 30 years after gastric tube interposition for oesophageal atresia.

Cost-effectiveness of neonatal surgery: first greeted with scepticism, now increasingly accepted.

Mortality rates in neonatal surgery have dropped markedly, illustrating the enormous progress made. Yet, new questions have arisen. To mention one, health care budgets have tightened. It follows that the effects of medical interventions should be weighted against their costs. As evidence was particularly sparse, we set out to analyse cost-effectiveness of neonatal surgery. The purpose of this article is to summarise our findings and to review recent studies. Moreover, this article explains the relevance of cost-effectiveness analysis and explores how cost-effectiveness interacts with other determinants of health care priority setting. Our research revealed that treatments for two common diagnostic categories in neonatal surgery (congenital anorectal malformations and congenital diaphragmatic hernia) produce good cost-effectiveness. Other groups also published cost-effectiveness studies in the field of neonatal surgery, although their number is still small. Contemporaneously, the economic aspects of health care have captured the interest of policy makers. Importantly, this is not to say that there are no other factors playing a role in priority setting, foremost among which are ethical questions and arguments of equity. This article concludes that, according to present evidence, neonatal surgery yields good value for money and contributes to equity in health.

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

BACKGROUND: Ultrasonography (US) as a diagnostic tool in the work-up of boys with nonpalpable testes (NPT) is still controversial. OBJECTIVE: To evaluate the relation between US and operative findings in boys with NPT. MATERIALS AND METHODS: During 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. RESULTS: US 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. CONCLUSION: All 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.

Can pyloromyotomy for infantile hypertrophic pyloric stenosis be performed in any hospital? Results from two teaching hospitals.

In order to document the incidence of perioperative complications in patients with infantile hypertrophic pyloric stenosis, a descriptive cohort study was performed in two teaching hospitals in the Netherlands. One hospital specialized in pediatric surgery and the other was a general surgery teaching hospital. All consecutive infants who underwent pyloromyotomy for the diagnosis hypertrophic pyloric stenosis in both hospitals between 1998 and 2002 were included. The children were diagnosed and treated according to a standard protocol. From all charts, complications durante- and post-operationem were recorded. A total of 256 pyloromyotomies were performed. Registered perioperative complications were duodenal mucosal perforation (n=6; 2%). Perioperatively unrecognized duodenal mucosal perforation occurred four times (1%). One re-operation was performed for an incomplete pyloromyotomy (0.3%). Persistent vomiting after the operation occurred in 18 children (7%). A large majority of postoperative complications were wound infections (n=16; 6%), 12 after right upper quadrant incision and 4 after umbilical incision; most of them were treated with antibiotics and/or incision for drainage of an abscess. An incisional hernia occurred four times. Prolonged vomiting was the only postoperative complication that differed significantly between the two teaching hospitals. The overall percentages of complications were equal to complication rates in literature, and since there were no extensive differences in major complications between the two teaching hospitals in this study, we can conclude that pyloromyotomy can be performed safely in specialized centers and in general centers provided with a multidisciplinary team.

Study of the factors associated with recurrence in children with sacrococcygeal teratoma.

PURPOSE: The aim of this study was to explore effects of (1) histological involvement of resection margins with microscopic residue, (2) incomplete removal of coccyx, and (3) tumor spillage on recurrence and on survival in children operated upon for sacrococcygeal teratoma (SCT). METHODS: Retrospective review of 70 patients treated between 1960 and 2003. RESULTS: Fifty-four girls and 16 boys presented with SCT diagnosed prenatally (12), at birth (37), or later (21). Thirty-six percent of tumors were Altman type I, 27% type II, 18% type III, and 18% type IV. Histologically, mature teratoma was observed in 48 patients, immature teratoma in 11, yolk sac tumor (YST) in 9, embryonal carcinoma in one, and mixed tumor in one. Eighty-four percent of patients solely underwent surgical extirpation. Six (8.5%) patients died. However, mortality for the group of 42 patients treated during the past 15 years was as low as 2.5%. Tumor recurrence was observed in 5 patients, 2 of whom died. Of 3 patients with initially mature teratoma, 1 showed local immature recurrence and 2 malignant recurrences. One of the latter died. Of 2 patients with initially immature teratoma grade I, one relapsed with a benign lesion and one with YST leading to death. Possible eliciting factors had been demonstrated in 3 patients. Histological analysis of resection margins showed tumoral involvement in 11 patients (and also in one patient after resection of a recurrent tumor). Only one of those with YST focus in the resection margin showed recurrence. Intraoperative tumor spillage presented in 2 patients, who both died of metastatic disease. Spillage of tumoral cyst fluid occurred in 6, none developed recurrence. One of 5 patients whose coccyx had not been removed died of metastatic disease. One with immature teratoma developed a benign recurrent tumor. The other 3 showed no recurrence. CONCLUSIONS: Microscopic involvement of the resection margins of mature or immature SCT is rarely associated with recurrence, provided there are no YST foci in the resection margins. A conservative attitude then appears to be justified. Spillage of cyst fluid was never associated with recurrence, unlike spillage of tumor and absence of removal of coccyx.

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

BACKGROUND: 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. PROCEDURE: The records of 66 patients (median age 9 years) with histologically proven ovarian germ cell tumor (either benign or malignant), treated over a 44-year-span, were reviewed. RESULTS: Pain and an abdominal mass were the most frequent symptoms. The tumors were right-sided in 35, left-sided in 28, and bilateral in 3. Most patients (52) were stage I, 4 were stage II, 6 stage III, and 1, with liver metastases, stage IV. Sixteen patients had an emergency operation for tumor torsion. Unilateral salpingo-oophorectomy was the most frequently performed procedure (n = 46), and ovarian-sparing tumorectomy was performed in 9 patients (one bilaterally). Histologically, teratomas were found most frequently (mature: 45, immature: 9), followed by mixed tumors (n = 7), yolk sac tumors (n = 3), dysgerminoma (n = 2), gonadoblastoma (n = 2), and embryonal carcinoma (n = 1). Surgical removal of the tumor with or without the ovary and/or adnex was the sole treatment in 55 patients, chemotherapy was administered in 10 and radiotherapy + chemotherapy in one. Intra-operative spillage of tumoral fluid occurred in six; this did not influence outcome in five. Recurrence was observed in three patients. Two patients, with malignant disease, died. The 64 survivors are now between 8 months and 44 years after treatment. CONCLUSIONS: With a recurrence rate of 4.5% and a mortality rate of 3%, this series confirms the excellent prognosis for girls with ovarian germ cell tumor (GCT).

Retroperitoneal germ cell tumors: a clinical study of 12 patients.

PURPOSE: The aim of the study was to examine the clinical presentation, method(s) of treatment, complications, and results in newborns and infants with retroperitoneal germ cell tumors (GCTs). METHODS: A retrospective chart review of all patients treated between 1974 and 2002 for GCT located in the retroperitoneum in 2 institutions identified 12 patients with histologically proven retroperitoneal GCT. Vital data concerning pregnancy and delivery were analyzed. Age at diagnosis and symptoms were recorded, as well as possibly associated anomalies. Data concerning surgical treatment, perioperative and postoperative complications, histological staging, and final outcome were all analyzed. RESULTS: In 3 patients, the diagnosis had been made antenatally between 31 and 35 weeks of gestation. In 1 patient, the diagnosis was made at birth, and in 8 later in life (ages 3, 5, 7, 8, 8, 11, 18, and 24 months). Symptoms in these 8 boys and 4 girls were abdominal distension and a palpable upper abdominal mass, right-sided in 5, left-sided in 5, and central in 2; the tumor was usually big. Associated anomalies were noted in 4 patients and were chromosomal in 3 (Down syndrome in 2 and Klinefelter syndrome in 1). One baby died of uncontrollable bleeding during an emergency operation immediately after traumatic birth. The other 11 infants survived. Four other patients showed serious perioperative complications (1 caval vein tear, 1 choledochal tear, 1 cyst rupture, and 1 esophagogastric tear) which were managed without further consequences. Histologically, 4 tumors were mature teratomas, 6 were immature teratomas (grade I in 4, grade II in 1, and grade II-III in 1), and 2 were malignant yolk sac tumors (YSTs). The patients with YSTs underwent surgical biopsy, followed by chemotherapy and excision of the remaining tumor and of the metastases. No adjuvant treatment was administered in the patients with benign disease. Nine survivors with benign tumor are disease-free between 1 and 30 years after surgery. Two patients with YST have now been in remission for 6 and 5 years, respectively. CONCLUSIONS: Both this study and the literature review performed testify to the extreme rarity of GCTs in the retroperitoneum. Surgical removal of the tumors appeared to be hazardous because of the extent of the tumor, the displacement and elongation of adjacent structures and organs, and/or the adhesion of the tumor to surrounding tissues; this resulted in several perioperative complications. The long-term results are good, however, with 9 of 10 patients with benign tumors in good health after a mean follow-up of 12 years, and with the 2 patients with YST in remission for 6 and 5 years, respectively.

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 day; 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.

Strategy for management of newborns with cervical teratoma.

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.