Hemisplenectomy for giant splenic cysts in children

To analyse the clinical features and outcome following surgery of giant splenic cysts in␣children, all patients presenting to a children's hospital over the years 1987–1996 were reviewed. Variables analysed included presenting symptoms, method of diagnosis, operative procedure, and complications. Six patients (aged 8–16 years) presented with abdominal pain or a large abdominal mass. Ultrasound examination demonstrated large (>5 cm) unilocular splenic cysts. Five of the six children underwent hemisplenectomy without complication or blood transfusion. The remaining patient had an infected cyst, for which total splenectomy was necessary because of major intraoperative haemorrhage. Hemisplenectomy is based on accurate dissection of the splenic hilum with clear knowledge of the intracapsular vascular anatomy of the spleen. Preoperative vaccination is recommended in the event that splenectomy is required. This retrospective review concludes that hemisplenectomy for splenic cysts is a safe procedure in children. Accepted: 3 November 1997     

Is infertility after surgery for cryptorchidism congenital or acquired?

We evaluated whether deletions of the DAZ (deleted in azoospermia) gene, abnormal karyotypes or hypogonadotropic hypogonadism (HH) were demonstrable in infertile men who had undergone surgery in childhood for cryptorchidism with a simultaneous testicular biopsy that demonstrated no or almost no germ cells. In six men with infertility after surgery for cryptorchidism, the adult karyotype and analyses for the DAZ gene, serum follicle-stimulating hormone, luteinizing hormone, and testosterone were performed from a peripheral blood sample. Testicular volume was also measured. All patients exhibited a 46,XY karyotype. There were no deletions of the DAZ-gene, no HH and no testicular atrophy. The infertility was thus not proved to be congenital, and consequently, surgical treatment is indicated before the germ cells disappear from undescended testes. This appears to be before 15 months of age.     

Neuroblastoma and insulin-like growth factor system

Neuroblastoma is, at the same time, the most common and the most puzzling extracranial solid tumour in childhood, being able to regress spontaneously despite widespread dissemination, showing a striking high incidence of the in situ form, and, finally, being resistant even to aggressive chemotherapy. The reasons of this bizarre behaviour are still largely unknown due to our little knowledge of neuroblastoma pathophysiology. There is increasing body of evidence that the insulin-like growth factor system plays a crucial role in the proliferation and differentiation of neuroblastoma cells and it is conceivable that a better knowledge of this role might potentially lead to new and more effective therapeutic strategies. Here we review the most recent insights into the biology of neuroblastoma, focusing on the close links with the insulin-like growth factor system and the potential clinical perspectives. Received: 13 May 1996 and in revised form: 15 July 1996 / Accepted: 15 July 1996     

Helicobacter pylori and hypergastrinemia in children with recurrent abdominal pain

Recurrent abdominal pain (RAP) is a significant problem in the pediatric population, and there has been much recent interest in the role that Helicobacter pylori (Hp) might play in this disorder. In this case control study, the authors aimed to determine whether Hp is an agent responsible for RAP, and to assess fasting gastrin concentrations in children with and without RAP in the Hp-positive and -negative groups. The study was conducted in 42 patients with RAP and 50 healthy children attending routine day-case surgery as a control group, aged 3 to 15 years, over a 12-month period. Of the 42 children with RAP, 30 were seropositive (71.4%) for Hp IgG, and of 50 children in the control group, 32 were seropositive (64%) for Hp IgG (P > 0.05). We found that Hp infection was as high in healthy children as in children with RAP. The mean fasting gastrin levels in 62 Hp-seropositive children (60.4 ng/l) were not different from those in 30 Hp-seronegative children (57.3 ng/l) and those in 42 children with RAP (58.2 ng/l) were also not significantly different from those in 50 healthy children (62.9 ng/l). Thus, no association between childhood Hp infection, hypergastrinemia, and RAP was found in our Turkish population. Accepted: 26 May 1998     

Herpes virus (KSHV) associated kaposi sarcoma in a 3-year-old child with non-HIV-induced immunodeficiency

A 3-year-old boy of German descent suffered from two episodes of Streptococcus pneumoniae meningitis within 2 months. One month previously, the first skin lesion of Kaposi sarcoma (KS) had been observed behind his right ear. During the following 2 years KS disseminated not only mucocutaneously but also to visceral organs. Immunological evaluation revealed severe lymphocytopenia with reduced helper/suppressor T-cell ratio and impaired humoral immune response to pneumococci. Extensive laboratory tests gave no evidence for known immunocompromising infections. However, recently described DNA sequences from a Kaposi sarcoma-associated herpesvirus (KSHV) could be identified within skin tissue. As chemotherapy failed to stop tumour progression the patient was referred for bone marrow transplantation. Eighteen months later the KS is in remission and the patient in a good general condition. Conclusion The case supports the hypothesis that KSHV is involved in the aetiology of KS. Bone marrow transplantation is possibly a therapeutic option for KS in patients with immunodeficiency not related to human immunodeficiency virus infection. Received: 1 August 1996 / Accepted: 15 December 1996     

Neonatal benign sacrococcygeal teratoma with N-type rectobulbar fistula

A unique case of benign sacrococcygeal teratoma associated with an N-type rectobulbar fistula is reported. Accepted: 12 September 1997     

Ascorbic acid enhances hydroxyl radical formation in iron-fortified infant cereals and infant formulas

Infant cereals and formulas are usually fortified with iron to prevent iron deficiency. To enhance iron bioavailability, supplemental ascorbic acid is recommended. Ascorbic acid is considered to be an antioxidant in vivo, but has pro-oxidant effects when exposed to non-protein-bound iron. We measured formation of free radicals in cereals and infant formulas after addition of ascorbic acid. The production of hydroxyl radicals was assessed by hydroxylation of salicylic acid to 2,5-dihydroxybenzoic acid (2,5-DHBA). Production of 2,5-DHBA increased with increasing ascorbic acid doses added. Addition of 0.8 mM ascorbic acid to breast milk produced less radicals (0.03 ± 0.05 μM) than addition of ascorbic acid to low-iron formula (0.13 ± 0.08 μM, P = 0.019), medium-iron formula (0.34 ± 0.12 μM, P < 0.0001) or high-iron formula (0.44 ± 0.08 μM, P < 0.0001). Even when iron content in breast milk was adjusted to a level comparable with that of formulas, production of 2,5-DHBA was lower. Breast milk seems to contain substances that reduce hydroxyl radical formation. Conclusion Supplemental ascorbic acid causes hydro-xyl radical formation in iron-fortified infant nutrients in vitro. Received: 30 March 1996 / Accepted in revised form: 10 December 1996     

Germ-cell degeneration in experimental unilateral cryptorchidism: role of apoptosis

We investigated the possible involvement of apoptosis in the increased germ-cell degeneration in undescended testes (UDT). Experimental unilateral cryptorchidism was induced in 21-day-old rats, and both testes were removed for in-situ TUNEL staining of apoptotic cells at 1, 3, 7, 10, and 14 days postoperation. A gradual increase in the incidence of apoptosis was seen at 21–28 days of age in the control testes, followed by a decrease thereafter. After 10 days postoperation, the weight of the UDT was significantly lower than that of the contralateral descended testis (CDT) and the controls. However, the weight of scrotal testes in each group was similar. UDTs demonstrated a markedly increased incidence of apoptosis. By 7 days postoperation, the percentage of seminiferous tubules containing apoptotic germ cells significantly increased in UDTs compared with that in CDTs and controls (P < 0.001). Moreover, there was a significant difference in the percentage of seminiferous tubules containing apoptotic germ cells between CDTs and controls (P < 0.01). In addition, an increased incidence of seminiferous tubules containing 8–10 and >10 apoptotic germ cells from 7, 10, and 14 days postoperation in UDTs was detected. In-situ TUNEL analysis demonstrated spermatocytes to be the main type of germ cells affected in all groups. These findings suggest that spermatogenesis decreases not only in the␣UDT, but also in the CDT, and that the germ-cell degeneration in cryptorchidism took the form of apoptosis.     

Epidermoid cyst in a wandering spleen

A wandering spleen and splenic cysts are uncommon conditions in children. A combination of both entities has only rarely been reported in the literature. Another case of this complex pathology in 12-year-old girl is presented. She was initially referred for evaluation of a large abdominal mass. Diagnostic studies raised the suspicion of a hematoma in an abnormally located spleen, but the definitive diagnosis was established at laparotomy. Successful surgical management consisted of total cystectomy and splenopexy. Accepted: 6 November 1997     

Diagnostic delay in neurofibromatosis type 1

Since 1985 a multidisciplinary team in the Sophia Children's University Hospital in Rotterdam provides diagnostic follow up and genetic counselling services for neurofibromatosis type 1 (NF1) patients and their families. Parents of 68 affected children as well as 24 affected parents were interviewed. Of the affected children, 50% and 33% of the affected adults were treated for symptoms related to NF1 before a specific diagnosis was made. Although the disease is fully penetrant by the age of 5 years, 35% of the affected children had not been diagnosed by this age. Parents stated a preference for early diagnosis of NF1. Diagnosis of NF1 did not seem to be a reason to refrain from having children. The general attitude towards prenatal diagnosis was positive; however few parents would actually terminate an affected pregnancy. Conclusion Overall delay in diagnosis of NF1 is significant. Knowledge of symptoms should make an early diagnosis possible with beneficial effects for the patient and family members. Received: 25 July 1996 / Accepted: 21 October 1996     

Comparison of the pulmonary vasculature in newborns and stillborns with congenital diaphragmatic hernia

The purpose of this study was to compare structural changes in the pulmonary vasculature in newborns with congenital diaphragmatic hernia (CDH) complicated by persistent pulmonary hypertension (PPH) and stillborns with CDH. Victorian blue van Gieson (VVG) staining and immunostaining with anti-alpha smooth-muscle actin (ASMA) was performed on lung tissue obtained at autopsy from 23 newborns with CDH complicated by PPH, 7 stillborns with CDH, and 11 age-matched controls with sudden infant death syndrome (SIDS). The degrees of adventitial and medial thickness and area were measured in pulmonary arteries with an external diameter (ED) of <75 μm, 75–100 μm, 100–150 μm, 150–250 μm, 250–500 μm, and >500 μm by image analyzer and compared statistically. The degrees of adventitial and medial thickness and area were measured in pulmonary veins with an ED of <100 μm, 100–200 μm, and >200 μm by image analyzer and compared statistically. In order to determine whether the characteristic structural changes were size-related, each was related to ED. There was a significant increase in adventitial thickness and area in arteries of all sizes in both newborns and stillborns with CDH compared to SIDS patients (P < 0.05). The degree of medial thickness in newborns and stillborns with CDH was significantly increased compared to SIDS patients (P < 0.01). The degree of medial area was significantly increased for arteries with ED less than 100 μm (P < 0.05) in newborns and stillborns with CDH compared with SIDS patients. There was a significant increase in adventitial thickness and area in veins of all sizes in newborns with CDH compared to stillborns with CDH and SIDS (P < 0.05). The degree of adventitial thickness and area of pulmonary veins were similar in stillborns with CDH and SIDS. There were no significant differences in medial thickness of veins between the three groups. The presence of abnormally thick-walled pulmonary arteries in stillborns with CDH suggests that the intrapulmonary arteries in CDH may become excessively muscularized during fetal life, becoming unable to adapt normally at birth. The absence of structural changes in pulmonary veins in stillborns with CDH suggests that the pulmonary venous changes observed in newborns with CDH complicated by PPH occur after birth as a result of increases in transvascular pressure or a response to release of peptide growth factors. Accepted: 10 March 1998     

Use of albumin in neonatal resuscitation

The use of albumin plasma has become popular during resuscitation of the term baby with very low Apgar scores (≤ 2 at 1 min). There is no evidence of benefit from this practice which may actually be damaging to babies with severe asphyxia causing myocardial damage. Received: 4 May 1996 and in revised form: 7 October 1996 / Accepted: 16 October 1996     

Functional outcome after pyeloplasty for unilateral symptomatic hydronephrosis

The functional outcome and factors influencing improvement after pyeloplasty for ureteropelvic junction (UPJ) obstruction are still debated. This retrospective study was aimed at evaluating the factors associated with functional improvement in symptomatic unilateral hydronephrosis (HDN). Patients (n = 68) who underwent successful pyeloplasty for unilateral symptomatic UPJ obstruction without any other associated urological abnormality were included. Preoperative evaluation included a diuretic renogram (DR) to confirm obstruction and assess the split renal function (SRF). A follow-up DR was obtained 3 months and 1, 2, and 5 years postoperatively. An absolute increase in the differential function of the operated kidney by over 5% was considered significant; such kidneys were classified as improved (group A) and the others as unimproved (group B). The difference between the preoperative and 3-month SRF was highly significant (P < 0.001). Significant (P < 0.01) improvement in SRF continued until 1 year after pyeloplasty. Patients who presented with a mass had significantly greater improvement (P < 0.05) than those who presented with other clinical features. In group A, a significantly higher number of patients presented with a mass. It was also evident that patients aged less than 1 year showed significantly greater (P < 0.01) improvement in SRF than older patients. Thus, in unilateral symptomatic HDN with impaired function, patients who present with a mass or those in whom pyeloplasty was performed before 1 year of age showed significantly greater improvement.     

A thyroxine dosage of 8 μg/kg per day is appropriate for the initial treatment of the majority of infants with congenital hypothyroidism

 The adequate l-thyroxine dosage for the initial treatment of infants with congenital hypothyroidism is a subject of controversy. Some recommend higher dosages (> 10 μg/kg/day) to ensure adequate levels, while others advocate lower dosages to permit normalisation of thyroid status. The aim of this study was to evaluate the␣results of a treatment strategy using an initial dosage of 7.5–8.0 μg/kg per day, TSH measurements being␣taken at 15 and 30 days of treatment. Fifty one newborns infants with primary congenital hypothyroidism␣detected by neonatal screening were treated with the same therapeutic strategy. A mean l-thyroxine dosage of 7.9 μg/kg per day at the onset of treatment and 6.6 μg/kg/d at 2 months, normalised the FT₄ and FT₃ levels at 15 days in 100% and TSH levels at 2 months in 90% of cases. Many patients showed elevated levels of FT₄ and a systematic higher initial dosage could expose many infants to a dangerous hyperthyroidism. Patients with abnormal TSH levels at 2 months already had higher TSH levels in the first 8 weeks of life and, despite higher l-thyroxine dosage, also exhibited lower FT₄ and FT₃ levels. These patients who needed an early increase in dosage had already shown a more profound ante and neonatal hypothyroidism. This subgroup of patients require a higher dosage of thyroxine and early assessment of FT₄, FT₃ and TSH levels are required for optimum dosage choice. Conclusion Even though a subgroup of patients may require a higher dosage of l-thyroxine, an initial␣dosage of 7.5–8.0 μg/kg per day, with an early assessment of␣FT₄, FT₃, and TSH levels, is adequate for the treatment of the majority of infants with congenital hypothyroidism. Received: 17 August 1995 / Accepted 10 June 1996     

The impact of surfactant replacement therapy on cerebral and systemic circulation and lung function

 The influence of surfactant administration on cerebral and systemic circulation and on lung function was evaluated in 12 premature mechanically ventilated infants (mean birth weight 1560 ± 770 g, mean gestational age 30.0 ± 3.2 weeks) with respiratory distress syndrome (RDS) receiving surfactant replacement therapy. We measured mean cerebral blood flow velocity (MCBFV), heart rate (HR), mean arterial pressure (MAP), static compliance (Crs), resistance of respiratory system (Rrs), functional residual capacity (FRC) and fraction of inspired oxygen (FiO₂). In addition to a very low compliance and a moderately elevated resistance of the respiratory system a significant drop in MAP, HR, MCBFV and FiO₂ was noticed after surfactant administration. After 30 min HR, MAP and MCBFV values returned to baseline levels. We postulate that the drop in MCBFV, MAP, HR and FiO₂ with a minor, though not significant, improvement of the FRC can most likely be explained by a “relative” hypovolaemia in other organs and parts of the body due to expansion of the lung vascular bed. Compensation for the redistribution of circulatory volume occurred within several minutes. Blood pressure control and treatment of hypovolaemia is mandatory before surfactant is administered. Conclusion In RDS patients there is a significant drop of MAP, HR, MCBFV and FiO₂ after bolus surfactant administration. Received: 30 August 1995 and in revised form: 30 April 1996 / Accepted 23 July 1996     

Covered exstrophy: a rare variant

A case of covered exstrophy without sequestration of a bowel segment is reported. A 4-year-old female presented with dribbling of urine. Treatment to date has been simple excision of the covered membrane with functional closure of the bladder and bilateral posterior iliac osteotomies, with reconstruction of the bladder neck and genitalia to be performed at a later date. The embryogenesis of this rare variant, a review of the reported cases, and management options are discussed. Accepted: 6 November 1997     

The role of magnetic resonance imaging in the preoperative assessment of anorectal anomalies

The role of magnetic resonance imaging (MRI) compared to other investigations in providing information relevant to and affecting surgical management in patients with anorectal anomalies (ARA) was studied in two groups of patients. In group 1 a pilot study was done in 9 patients with colostomy for high ARA awaiting posterior sagittal anorectoplasty. These patients had MRI, plain radiographs, a distal loopogram through the mucous fistula, ultrasound scans of the spinal cord and urinary tract, and a micturating cystourethrogram. The level of bowel and spinal-cord termination was correctly determined in all patients by MRI and conventional investigations. The presence or absence of a fistula, urological abnormalities, and bony abnormalities of the pelvis and spine were better visualized by conventional investigations than with MRI. The striated-muscle complex (SMC) was well-visualized by MRI, the thickness accurately graded, and the findings confirmed at operation. The information on muscle thickness and the presence or absence of a fistula was interesting, but played no part in decisions made about surgical management, and the operative technique was not altered. In group 2 the patients were much older and had MRI for persistent problems, mainly soiling; 6 had low lesions and 6 had high lesions. Three patients with low lesions were found to have intraspinal abnormalities on MRI; 4 were found to have malpositioned bowel in the SMC, which was again accurately visualized and graded. Therefore, in 7/12 patients of this group a surgically correctable abnormality was found. We conclude that MRI has no role as a primary investigation in patients with high ARA, but it is useful if there are suspected spinal-cord problems or persistent problems after definitive surgery. Accepted:  6 May 1998     

Expression of Clara cell 10-kDa protein (CC10) in congenital diaphragmatic hernia

Clara cell 10 kDa protein (CC10) has been thought to be fairly specific to Clara cells and a major secretory protein that is both synthesized and released from Clara cells. In the present study, morphometric analyses of the immunohistochemical expression of CC10 were carried out on the bronchioles of human neonates with congenital diaphragmatic hernia (CDH) and then compared with morphometric analyses from a gestationally and postnatally age-matched control group in order to clarify the immaturity of Clara cells in CDH lungs. No difference was found in CC10 expression between the affected side and the unaffected side of the lungs in the CDH group. However, compared with the lungs of the control group, the CDH group showed a significant decrease in CC10 expression, namely, the ratio of CC10-positive cells per bronchiole, per unit perimeter of bronchiole, and per unit bronchiolar surface area. These results suggest that in the lungs of CDH cases, a possible delay in either functional maturation or the development of CC10 synthesis by the bronchioles may exist, and this retardation of functional maturation of the airway is also considered to play a role in the postnatal respiratory insufficiency observed in CDH patients. Accepted: 25 February 1998     

Pneumatic dilatation for childhood achalasia

Treatment of achalasia by pneumatic balloon dilatation (PBD) is well established in adults. Due to limited experience and the rarity of the condition in children, there are relatively few reports in the paediatric literature. Although PBD has been reported as a primary method of treatment, there are no reports of secondary PBD for childhood achalasia. Between 1995 and 1999, five patients underwent treatment for achalasia (age: 9–14 years; M:F=4:1). The presenting symptoms were dysphagia (5), vomiting episodes (2), aspiration (1), food-bolus obstruction (1), and failure to thrive (1). In all patients a barium swallow and manometry were used to confirm the diagnosis. Three underwent primary PBD. Two who had previously undergone surgical myotomy underwent secondary PBD for recurrence of symptoms. Dilatation was performed using a 35-mm balloon with the child under general anaesthesia. Technical success was defined as demonstration of a waist under screening at lower pressures followed by abolition of the waist at higher pressures. In addition to reviewing our results, a systematic review of the literature was performed (Medline, Cochrane Library, Pubmed, Embase). Three patients (primary dilatation) showed excellent improvement after a single dilatation. In two cases (secondary dilatation) three and five attempts were required. No complications were encountered. The mean follow-up period was 2 years (1–3.5 years) and four patients remained asymptomatic, an overall success rate of 80%. The literature review revealed similar good results in most of the recent reports. Thus, PBD as a primary treatment for childhood achalasia has a success rate of 70%–90% with minimal side effects, short hospital stay, and good patient acceptability over an operation. We have also established the usefulness of this method as a secondary treatment when symptoms recur after surgery. Accepted: 1 September 2000