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1.
Andrology ; 5(4): 838-843, 2017 07.
Article in English | MEDLINE | ID: mdl-28637089

ABSTRACT

The aim of this study was to compare sperm DNA damage between men with a history of congenital undescended testis (UDT) and men with a history of acquired UDT. A long-term follow-up study of men with previous UDT was performed. Fifty men with congenital UDT who had undergone orchiopexy at childhood age, 49 men with acquired UDT after a 'wait-and-see'-protocol (e.g. awaiting spontaneous descent until puberty and perform an orchiopexy in case of non-decent), and 22 healthy proven fertile men were included. The DNA fragmentation index (DFI) using sperm chromatin structure assay (SCSA) was used to express the level of sperm DNA damage. Decreased fertility potential was considered if DFI was above 30%. Sperm DNA damage was not statistically different between cases of congenital and acquired UDT. DFI was significantly more often >30% in the complete group of men with congenital UDT (9/50; 18%) and in the subgroup with bilateral congenital UDT (3/7; 43%) in comparison with the controls (none) (p-value 0.049 and 0.01, respectively). Age at orchiopexy in congenital UDT had no statistical effect on DNA damage. In men with acquired UDT, DFI did not statistically differ between those having undergone orchiopexy and those experiencing spontaneous descent. This study supports the hypothesis that UDT is a spectrum representing both congenital UDT and acquired UDT. Sperm DNA damage at adult age is not influenced by age at orchiopexy in congenital UDT cases and by orchiopexy or spontaneous descent in acquired UDT cases.


Subject(s)
Chromatin Assembly and Disassembly , Chromatin/chemistry , Cryptorchidism/pathology , DNA Damage , Flow Cytometry/methods , Spermatozoa/pathology , Case-Control Studies , Cryptorchidism/genetics , Cryptorchidism/surgery , Humans , Logistic Models , Male , Nucleic Acid Conformation , Odds Ratio , Orchiopexy , Protein Conformation , Sperm Count , Sperm Motility , Spermatozoa/chemistry , Structure-Activity Relationship
2.
Andrology ; 3(5): 888-94, 2015 Sep.
Article in English | MEDLINE | ID: mdl-26216342

ABSTRACT

The aim of this study was to report on different anomalies found by physical examination and scrotal ultrasound in men with previously unilateral congenital undescended testes (UDT; N = 50), acquired UDT (N = 49), their contralateral normally descended testis (CNDT) and control testes (N = 53). Acquired UDT significantly more often had a testicular volume being <15 mL than congenital UDT (88% vs. 68%). In the congenital group, significant differences were found between UDT and CNDT for soft consistency (UDT 36% vs. CNDT 14%), epididymal diameter (UDT 7.6 mm vs. CNDT 8.9 mm), testicular volume (UDT 9.8 mL vs. CNDT 13.8 mL), and inhomogeneous parenchyma (UDT 38% vs. CNDT 14%). In the acquired group, significant differences were found between UDT and CNDT for epididymal diameter (UDT 7.5 mm vs. CNDT 8 mm), testicular volume (UDT 9.3 mL vs. CNDT 14.1 mL), testicular volume <15 mL (UDT 88% vs. CNDT 59%), and inhomogeneous parenchyma (UDT 27% vs. CNDT 6%). The following parameters of congenital UDT, acquired UDT, congenital CNDT, and/or acquired CNDT significantly differed compared with controls: soft testicular consistency (congenital UDT 36%, acquired UDT 20%, congenital CNDT 14%, acquired CNDT 12% vs. controls 0%), epididymal diameter (congenital UDT 7.6 mm, acquired UDT 7.5 mm, acquired CNDT 8 mm vs. controls 9.2 mm), testicular volume (congenital UDT 9.8 mL, acquired UDT 9.3 mL, congenital CNDT 13.8 mL, acquired CNDT 14.1 mL vs. control testes 15.8 mL), testicular volume <15 mL (congenital UDT 68%, acquired UDT 88%, congenital CNDT 66% vs. controls 43%), inhomogeneous parenchyma (congenital UDT 38%, acquired UDT 27%, congenital CNDT 14% vs. controls 0%), and testicular microlithiasis (congenital CNDT 24% vs. control testes 8%). Few differences between congenital and acquired unilateral UDT and congenital and acquired CNDT support the hypothesis of a spectrum of maldescended testes containing congenital and acquired UDT instead of them being two different entities. The CNDT also has anomalies albeit less severe than the UDT, indicating that in unilateral UDT both testes are affected.


Subject(s)
Cryptorchidism/diagnostic imaging , Epididymis/anatomy & histology , Scrotum/diagnostic imaging , Testis/anatomy & histology , Adolescent , Child , Child, Preschool , Cryptorchidism/diagnosis , Epididymis/physiology , Humans , Male , Retrospective Studies , Scrotum/anatomy & histology , Testis/physiology , Ultrasonography
3.
Andrology ; 3(4): 677-84, 2015 Jul.
Article in English | MEDLINE | ID: mdl-26084887

ABSTRACT

The aim of this study was to evaluate testicular function in men with previous acquired undescended testis (UDT) in whom orchiopexy was performed at diagnosis compared with a similar group of men in whom spontaneous descent was awaited until puberty. Secondly, we examined the influence of age at orchiopexy on fertility parameters in adult life. A total of 169 men of the 'orchiopexy at diagnosis' group and 207 men of the 'wait and see' protocol group were invited for participation. All participants underwent an andrological evaluation, including medical history, physical examination, scrotal ultrasound, determination of reproductive hormones, and semen analysis. Results were compared for men in whom orchiopexy was performed at diagnoses with men in whom spontaneous descent was awaited until puberty followed by orchiopexy in case of non-descent. In the 'orchiopexy at diagnosis' group, 63 men of whom 14 with bilateral UDT, and in the 'wait and see' protocol group, 65 men of whom 15 with bilateral UDT were included. For unilateral UDT Inhibin B was found to be significantly lower and median progressive motility was higher in men with orchiopexy at diagnosis. For bilateral UDT, semen concentration and progressive motility showed a trend toward a favorable outcome for orchiopexy at diagnosis. Age at orchiopexy being under or above 10 years of age had no significant influence on the fertility potential. The outcome of physical examination, scrotal ultrasound, endocrine function, and semen analysis indicates a compromised fertility potential in men with previous acquired UDT. None of the protocols proved to be superior. For bilateral UDT, a trend toward favorable outcome of orchiopexy at diagnosis was seen. Furthermore, age at orchiopexy did not have an influence on fertility parameters. Therefore, in our opinion a 'conservative policy' is warranted for unilateral UDT, especially because over 50% of acquired UDT descend spontaneously.


Subject(s)
Cryptorchidism/surgery , Fertility , Orchiopexy , Adolescent , Adult , Age Factors , Child , Child, Preschool , Cryptorchidism/diagnosis , Gonadal Hormones/blood , Humans , Male , Regression Analysis , Retrospective Studies , Semen Analysis , Watchful Waiting , Young Adult
4.
Andrology ; 1(1): 100-8, 2013 Jan.
Article in English | MEDLINE | ID: mdl-23258637

ABSTRACT

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.


Subject(s)
Cryptorchidism/complications , Fertility , Infertility, Male/etiology , Administration, Intranasal , Adolescent , Adult , Aerosols , Age Factors , Case-Control Studies , Child , Child, Preschool , Cryptorchidism/diagnosis , Cryptorchidism/physiopathology , Cryptorchidism/therapy , Female , Follow-Up Studies , Gonadotropin-Releasing Hormone/administration & dosage , Humans , Infant , Infertility, Male/diagnosis , Infertility, Male/physiopathology , Kaplan-Meier Estimate , Logistic Models , Male , Middle Aged , Multivariate Analysis , Orchiopexy , Risk Factors , Time Factors , Treatment Outcome , Young Adult
5.
Eur J Pediatr Surg ; 21(6): 351-5, 2011 Dec.
Article in English | MEDLINE | ID: mdl-21879460

ABSTRACT

AIM: Surgical findings were studied to find an explanation for the phenomenon that some acquired undescended testes (UDT) descend spontaneously whereas others need orchiopexy. METHODS: In patients with acquired UDT spontaneous descent was awaited until at least Tanner stage P2G2. Orchiopexy was performed when a stable scrotal position had not been achieved by the end of follow-up. RESULTS: Orchiopexy was needed in 57 of 132 cases (43%). In cases requiring orchiopexy, the difference in testis volume compared to the contralateral healthy testis was significantly larger than for spontaneously descended testes. 41 (72%) undescended testes were found in the superficial inguinal pouch; 16 (28%) at the external annulus. 26 of the 41 testes in the superficial inguinal pouch position (63%) could be manipulated preoperatively into a non-stable scrotal position; 15 could only reach the scrotal entrance prior to surgery. None of the 16 testes located at the external annulus could reach a scrotal position. Inguinal exploration in most cases revealed a fibrous string or a partially open processus vaginalis. CONCLUSION: The mobility of acquired UDT located within the external annulus is limited. It is mainly the fibrous string and the partially open processus vaginalis that prevent normal elongation of the spermatic cord with growth. These testes are unlikely to descend spontaneously. Acquired UDT lying in the superficial inguinal pouch can often be pushed down well below the scrotal entrance. We speculate that under normal hormonal stimulation at puberty, some of these growing testes may overcome the strength of the fibrous string in the spermatic cord and descend again spontaneously.


Subject(s)
Cryptorchidism/surgery , Orchiopexy/methods , Puberty , Testis/growth & development , Adolescent , Child , Child, Preschool , Cryptorchidism/diagnosis , Diagnosis, Differential , Follow-Up Studies , Humans , Infant , Male , Retrospective Studies , Testis/surgery
6.
Dis Esophagus ; 21(8): 742-5, 2008.
Article in English | MEDLINE | ID: mdl-18459984

ABSTRACT

Patients with neuromuscular impairment, such as cerebral palsy or myotonic dystrophy, often suffer from oropharyngeal neuromuscular incoordination and severe gastresophageal reflux (GER). In 1997, Bianchi proposed total esophagogastric dissociation (TEGD) as an alternative to fundoplication and gastrostomy to eliminate totally the risk of recurrence of GER in neurologically impaired children. Little information exists about the best management for adult patients with severe neurological impairment in whom recurrent GER develops after failed fundoplication. We present our experience in three adult patients with neurological impairment in whom TEGD with Roux-en-Y esophagojejunostomy and feeding gastrostomy was performed for permanent treatment of GER.


Subject(s)
Anastomosis, Roux-en-Y/methods , Cerebral Palsy/complications , Esophagogastric Junction/surgery , Gastroesophageal Reflux/etiology , Gastroesophageal Reflux/prevention & control , Myotonic Dystrophy/complications , Adult , Cerebral Palsy/surgery , Esophagostomy , Female , Gastrostomy , Humans , Jejunostomy , Male , Middle Aged , Myotonic Dystrophy/surgery
7.
Eur J Pediatr Surg ; 18(1): 1-6, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18302061

ABSTRACT

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.


Subject(s)
Neoplasms, Germ Cell and Embryonal/mortality , Neoplasms, Germ Cell and Embryonal/pathology , Adolescent , Child , Child, Preschool , Female , Head and Neck Neoplasms/mortality , Head and Neck Neoplasms/pathology , Humans , Infant , Male , Mediastinal Neoplasms/mortality , Mediastinal Neoplasms/pathology , Neoplasm Recurrence, Local/pathology , Neoplasms, Germ Cell and Embryonal/prevention & control , Ovarian Neoplasms/mortality , Ovarian Neoplasms/pathology , Retroperitoneal Neoplasms/mortality , Retroperitoneal Neoplasms/pathology , Retrospective Studies , Sacrococcygeal Region/pathology , Survival Analysis , Testicular Neoplasms/mortality , Testicular Neoplasms/pathology
8.
J Urol ; 178(4 Pt 2): 1726-9, 2007 Oct.
Article in English | MEDLINE | ID: mdl-17707013

ABSTRACT

PURPOSE: It is still controversial whether acquired undescended testis can best be managed by orchiopexy or by the wait and see method. We prospectively evaluated spontaneous descent of acquired undescended testes and possible predictive factors in prepubertal boys. MATERIALS AND METHODS: From 1982 to 2004 spontaneous descent was awaited until at least Tanner stage P2G2 in 109 boys with a total of 83 unilateral and 52 bilateral acquired undescended testes. Annually we established testis position and size. After Tanner stage P2G2 orchiopexy was done for all testes in an unstable scrotal position. RESULTS: Two boys (3 acquired undescended testes) were excluded from analysis. Of 132 acquired undescended testes 75 descended spontaneously (57%, 95% CI 48-65), including 40 of 75 (57%) in early puberty or before puberty and 32 of 75 (43%) in mid puberty. Orchiopexy was performed in 57 of 132 acquired undescended testes (43%). Acquired undescended testes showed an increasing chance of descending spontaneously with increasing age (p trend = 0.002). In 63 of 82 unilateral undescended testes we were able to compare testis volume at the onset of puberty with that of the healthy contralateral side. Of 17 testes that needed orchiopexy 12 (71%) had a volume that was more than 1 ml smaller than the healthy testis. This was noted in only 18 of 46 spontaneously descended acquired undescended testes (39%, p = 0.053). Other factors, such as the most caudal testicular position at referral or the frequency of confirmed descended testicular position before referral, were not predictive of spontaneous descent. CONCLUSIONS: A conservative wait and see approach to acquired undescended testis until puberty could prevent more than half of the boys from undergoing orchiopexy and it does not seem detrimental in terms of testicular volume.


Subject(s)
Cryptorchidism/physiopathology , Testis/physiopathology , Adolescent , Child , Child, Preschool , Cryptorchidism/etiology , Humans , Infant , Male , Prospective Studies , Puberty
9.
Eur J Pediatr Surg ; 16(5): 318-22, 2006 Oct.
Article in English | MEDLINE | ID: mdl-17160775

ABSTRACT

BACKGROUND: Mediastinal germ cell tumors presenting during childhood are extremely rare. Publications on this entity are very scarce. This paper reports on the clinical presentations, method(s) of treatment, complications, results and outcomes in a series of children with mediastinal germ cell tumors. METHODS: A retrospective chart review of 7 children treated between 1971 and 2001 for mediastinal germ cell tumor was carried out. Age at diagnosis and symptoms were recorded. Each patient's surgical treatment, peri- and postoperative complications, histological staging and final outcome were analysed. RESULTS: The median age of the 4 boys and 3 girls was 3 years (range 21 months-15 years). The most frequent symptoms were respiratory distress, persistent coughing, thoracic pain and anorexia/weight loss. Four patients had histologically benign tumors (mature teratoma). Their sole treatment consisted of complete surgical excision of the tumor and (part of) the thymus using either median sternotomy or left-sided thoracotomy. Recovery was uneventful. No recurrences have been observed. All four are alive with no evidence of disease, between 2.5 and 29 years after treatment. Malignant tumors were observed in three patients (1 yolk sac tumor, 1 choriocarcinoma and 1 malignant teratoma). Treatment consisted of either biopsy or debulking followed by chemotherapy (and radiotherapy in 1 case). Two of them died from uncontrollable metastatic disease. The patient with yolk sac tumor survived; he is now in remission, 4 years after diagnosis. CONCLUSIONS: Both this study and the literature review testify to the extreme rarity of mediastinal germ cell tumors in childhood. Children with this type of tumor usually are severely symptomatic. Histologically benign tumors carry an excellent prognosis provided surgical excision is complete. Histologically malignant tumors, on the other hand, have a worse prognosis. However, the use of platinum-based combination chemotherapy has considerably increased the survival rates.


Subject(s)
Mediastinal Neoplasms/diagnosis , Mediastinal Neoplasms/surgery , Neoplasms, Germ Cell and Embryonal/diagnosis , Neoplasms, Germ Cell and Embryonal/surgery , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Prognosis , Retrospective Studies , Teratoma/diagnosis , Teratoma/surgery , Treatment Outcome
10.
J Pediatr Urol ; 2(3): 197-201, 2006 Jun.
Article in English | MEDLINE | ID: mdl-18947609

ABSTRACT

Testicular germ cell tumors occurring during childhood are extremely rare. This study reports the clinical presentation, pathological diagnosis, treatment methods and outcome in a series of 20 boys, aged between 3.5 months and 16 years (median: 1.5 years; 19 were prepubertal), who were treated between 1963 and 2003. Histologically, mature teratoma was present in seven, immature teratoma in four and yolk sac tumor in nine. Nineteen patients were stage I; only one patient was stage IV. Of the 11 teratomas, 10 were treated by orchiectomy and one by testis-sparing tumor excision only. All 11 patients have survived and show no evidence of disease between 10 and 28 years after surgery. The nine patients with yolk sac tumor were managed by orchiectomy, in two plus retroperitoneal lymphadenectomy, and in eight plus chemotherapy. One patient is in remission for 10 months, seven are alive with no evidence of disease for 5.5-23 years, and one patient died from a T-cell acute lymphoblastic leukemia, 2 years after the end of treatment of the testicular tumor. A gradual switch towards less invasive treatment has been observed over the years. This study confirms the excellent cure rates obtained in children with testicular germ cell tumor, provided diagnosis is prompt and treatment accurate.

11.
Arch Dis Child ; 90(10): 1071-2, 2005 Oct.
Article in English | MEDLINE | ID: mdl-15941773

ABSTRACT

BACKGROUND: It is widely believed that hydrostatic reduction of intussusception is less successful in children with prolonged symptoms prior to presentation. AIM: To prospectively evaluate success in relation to duration of symptoms. METHODS: Prospective study in which children, regardless of symptom duration, underwent an attempt at hydrostatic reduction. RESULTS: Of 113 children presenting with intussusception, 16 had peritonitis and required immediate laparotomy. A hydrostatic reduction was attempted in 97 and was successful in 77 (79%). There were 26 successful reductions with symptoms <12 hours (81%), 30 with symptoms for 12-24 hours (81%), and 21 with symptoms >24 hours (75%). CONCLUSION: The success rate with hydrostatic reduction was not significantly influenced by symptom duration.


Subject(s)
Intussusception/therapy , Acute Disease , Catheterization , Child , Child, Preschool , Colon/diagnostic imaging , Colon/physiopathology , Endosonography , Female , Humans , Hydrostatic Pressure , Infant , Intussusception/diagnostic imaging , Intussusception/physiopathology , Male , Prospective Studies , Time Factors , Treatment Outcome , Water
12.
J Pediatr Surg ; 38(6): 971-4, 2003 Jun.
Article in English | MEDLINE | ID: mdl-12778405

ABSTRACT

BACKGROUND: The authors noticed a relatively large number of patients with congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) who had a chylothorax (CT). The data are reviewed. METHODS: The charts of patients from 1990 until 2000 with CDH, treated with or without ECMO, together with the charts of patients treated with ECMO for other reasons and patients with esophageal atresia (EA) repair were reviewed. The diagnosis of CT was made if aspirated fluid appeared chylous and contained more than 90% lymphocytes or if the triglyceride level was more than 1.50 mmol/L. RESULTS: Eighty-nine patients with CDH were analyzed. Postoperatively, 10% had a CT-21% in CDH patients with ECMO treatment and 6% in CDH patients without ECMO treatment. This difference appeared to be significant (P <.05). The presence of a patch as independent variable for the development of CT also showed significance (P <.05). CONCLUSIONS: Chylothorax presented in almost all cases as a left-sided fluid accumulation, and a patch was present in the majority of patients with CDH. Therefore, CT should be considered the result of the severity of the defect rather than the consequence of ECMO as a therapeutic modality.


Subject(s)
Chylothorax/epidemiology , Extracorporeal Membrane Oxygenation/adverse effects , Extracorporeal Membrane Oxygenation/methods , Hernia, Diaphragmatic/surgery , Hernias, Diaphragmatic, Congenital , Artificial Organs/adverse effects , Artificial Organs/statistics & numerical data , Birth Weight , Chylothorax/diagnosis , Esophageal Atresia/complications , Esophageal Atresia/surgery , Extracorporeal Membrane Oxygenation/statistics & numerical data , Female , Gestational Age , Humans , Infant, Newborn , Likelihood Functions , Male , Postoperative Complications/diagnosis , Postoperative Complications/etiology , Umbilical Arteries/metabolism , Umbilical Veins/metabolism
13.
Ned Tijdschr Geneeskd ; 146(33): 1551-6, 2002 Aug 17.
Article in Dutch | MEDLINE | ID: mdl-12212504

ABSTRACT

The Working Group 'Surgrey in Children and Newborns', founded in 1974, was the precursor of the first subsection of the Association of Surgeons in the Netherlands, founded in 1981: the Netherlands Association for Paediatric Surgrey. Around 1900, paediatric surgery acquired an identity on the basis of what took place in children's hospitals. All the admissions were then on social indications with a surgeon being called in as a consultant if necessary. Following the Second World War, the development in anaesthesia and analgesia and an increasing understanding of metabolic processes made ever larger operations possible. The required specific expertise and the need to bring it together were decisive arguments for the foundation of the subsection. Since then, the developmental biological and genetic aspects of severe congenital malformations have, inter alia, become new topics for investigation; the consequences for medical ethics continue to be a point for attention.


Subject(s)
General Surgery/history , Pediatrics/history , Societies, Medical/history , Ethics, Medical/history , General Surgery/ethics , History, 20th Century , Netherlands , Pediatrics/ethics , Societies, Medical/ethics
14.
Eur J Pediatr Surg ; 12(2): 141-3, 2002 Apr.
Article in English | MEDLINE | ID: mdl-12015663

ABSTRACT

We report a case of spinal epidural abscess presenting as abdominal pain. An 7-year-old boy presented with abdominal pain. He was operated on under suspicion of appendicitis. During operation, no abnormalities were found. Postoperatively, the abdominal pain did not subside. Subsequently, the boy developed neurological abnormalities. MRI showed a spinal epidural abscess. A laminectomy was performed and the boy was treated with antibiotics; he recovered well. This case showed that it is important to consider a spinal epidural abscess as a cause of abdominal pain with fever in children.


Subject(s)
Abdominal Pain/etiology , Epidural Abscess/surgery , Spinal Diseases/surgery , Child , Epidural Abscess/complications , Epidural Abscess/diagnosis , Humans , Male , Spinal Diseases/complications , Spinal Diseases/diagnosis
15.
J Pediatr Surg ; 37(1): 61-5, 2002 Jan.
Article in English | MEDLINE | ID: mdl-11781988

ABSTRACT

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.


Subject(s)
Ileum/abnormalities , Intestinal Atresia/mortality , Jejunum/abnormalities , Female , Follow-Up Studies , Humans , Ileum/surgery , Infant, Newborn , Intestinal Atresia/complications , Intestinal Atresia/surgery , Jejunum/surgery , Male , Postoperative Complications , Prognosis , Survival Rate , Survivors
16.
Semin Pediatr Surg ; 10(4): 222-9, 2001 Nov.
Article in English | MEDLINE | ID: mdl-11689996

ABSTRACT

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.


Subject(s)
Congenital Abnormalities/surgery , Enterocolitis, Necrotizing/surgery , Ethics, Medical , Birth Weight , Congenital Abnormalities/mortality , Enterocolitis, Necrotizing/mortality , Follow-Up Studies , Hospitals, Pediatric , Humans , Infant, Newborn , Netherlands , Postoperative Complications/epidemiology , Quality of Life , Time Factors
17.
Ned Tijdschr Geneeskd ; 145(14): 665-9, 2001 Apr 07.
Article in Dutch | MEDLINE | ID: mdl-11530701

ABSTRACT

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.


Subject(s)
Androgen-Insensitivity Syndrome/diagnosis , Androgen-Insensitivity Syndrome/genetics , Genitalia, Female/abnormalities , Genitalia, Male/abnormalities , Mutation , Testosterone/genetics , 17-Hydroxysteroid Dehydrogenases/genetics , Adolescent , Androgen-Insensitivity Syndrome/enzymology , Androgen-Insensitivity Syndrome/therapy , Child , Diagnosis, Differential , Female , Genetic Counseling , Humans , Infant, Newborn , Karyotyping , Male , Phenotype , Receptors, Androgen/genetics , Receptors, LH/genetics
19.
Arch Dis Child ; 83(1): 64-6, 2000 Jul.
Article in English | MEDLINE | ID: mdl-10869003

ABSTRACT

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.


Subject(s)
Appendicitis/diagnosis , Adolescent , Age Factors , Appendicitis/complications , Appendicitis/surgery , Child , Child, Preschool , Diagnosis, Differential , Diarrhea/etiology , Female , Gastroenteritis/diagnosis , Humans , Infant , Intestinal Perforation/etiology , Length of Stay , Male , Postoperative Complications , Prognosis , Referral and Consultation , Retrospective Studies , Rupture, Spontaneous/etiology , Time Factors
20.
Eur J Pediatr Surg ; 10(1): 3-7, 2000 Feb.
Article in English | MEDLINE | ID: mdl-10770239

ABSTRACT

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.


Subject(s)
Child Behavior Disorders/etiology , Cognition Disorders/etiology , Hernia, Diaphragmatic/psychology , Child , Depression/etiology , Female , Hernias, Diaphragmatic, Congenital , Humans , Male , Socialization
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