Serum and CSF soluble CD26 and CD30 concentrations in healthy pediatric surgical outpatients.

Activated T-helper type 1 (Th1) lymphocytes induce a cellular type immune response, and Th2 lymphocytes, a humoral or antibody-mediated type immune response. Soluble CD26 (sCD26) and soluble CD30 (sCD30) are regarded as markers of Th1 and Th2 lymphocyte activation, respectively. Serum from 112 generally healthy pediatric surgical patients and cerebrospinal fluid (CSF) from 39, aged 1-17 years were measured for sCD26 and sCD30 using an enzyme-linked immunosorbent assay method. The detection limit for sCD26 was 6.8 ng/ml and for sCD30, 1.9 IU/ml. For serum sCD26 and sCD30, 2.5% and 97.5% percentiles constituted the reference limits, and the 95% credible intervals for the percentiles were calculated using regression models with a Bayesian approach. A significant between-gender difference was observed (P = 0.015) in serum sCD26 concentration, of which the lower limits ranged between 273 and 716 ng/ml for girls and 235 and 797 ng/ml for boys. The upper limits ranged between 1456 and 1898 ng/ml for girls and between 1419 and 1981 ng/ml for boys. Moreover, the concentrations of sCD26 increased in infants and children up to 10 years in girls and 12 years in boys. After this however, the values decreased. The serum sCD30 concentration was highest among the youngest infants aged 1 year (80-193 IU/ml), after which a consistent age-related decrease was found. The lowest values were found at the age of 17 years (10-89 IU/ml). A significant between-gender difference in sCD30 concentration was observed (P = 0.019). sCD26 and sCD30 concentrations were low in the CSF samples analyzed: 13.3 ng/ml (median); range 8.3-51.5 ng/ml and 7.6 IU/ml; 2.1-18.5 IU/ml, respectively. Reference limits for serum sCD26 in children aged 1-17 years were established as being 235-1800 ng/ml in toddlers and 400-1800 ng/ml in female adolescents and 700-2000 ng/ml in male adolescents. For sCD30; reference limits of 80-190 IU/ml were established in the youngest age group and 10-90 IU/ml in adolescents.

The shoelace method in congenital aplasia of the scalp and skull.

Aplasia cutis congenita (ACC) is an uncommon skin disorder characterised by the absence of some or all layers of the skin. It may involve any part of the body, but is most common in the scalp. Some scalp lesions are associated with a defect of the underlying skull or even dura. Small superficial lesions heal spontaneously. Options available for larger defects and those involving the skull include conservative management, skin grafts, composite grafts, local scalp or pericranial flaps, and free microvascular transplants. This case report presents a delayed primary closure of a scalp and skull defect with the shoelace method and a temporary skin graft. The method is simple, gives an excellent cosmetic result, and can be used in moderately sized defects, where a direct suture is not possible.

The Santulli enterostomy in necrotising enterocolitis.

An ideal operation for necrotising enterocolitis (NEC) would be quick and reliable, remove all nonviable bowel, minimise the loss of intestinal length, permit early restoration of intestinal continuity, and minimise the need for secondary operations. No operation currently meets all these needs. In this study we review our experience with the Santulli enterostomy in NEC. From 1992 to 1998, 19 patients (16 males, three females) underwent a Santulli enterostomy for Bell grade III NEC. The median gestational age at birth (GA) was 26 weeks (range 23-30), and the median birth weight 755 g (range 600-1095). The median patient age at operation was 10 days (range 3-59), and the median operative time was 55 min (range 25-90). Sixteen (84%) patients survived. Complications included leakage of the Santulli anastomosis in four cases, stomal necrosis in two cases, additional intestinal necrosis in two cases, leakage of a concurrent intra-abdominal anastomosis in two cases, and intestinal obstruction in four cases. Twelve patients underwent relaparotomy. Enteral feeding was started at a median of 5 days postoperatively (range 2-9), with full oral feeding possible at a median of 21 days (range 10-128). The median time until closure of the enterostomy was 48 days and the median hospital stay 87 days (range 19-197). After stomal closure, obstructive symptoms necessitated a new Santulli enterostomy in two patients and a revision of the anastomosis in one patient. Santulli enterostomy can be used to treat NEC even in very small premature babies. It enables rapid recovery of intestinal continuity and early stomal closure. However, the operation is technically demanding and carries significant morbidity.

Pylephlebitis after appendicitis in a child.

Septic phlebitis of the portal vein, or pylephlebitis, is a rare but potentially severe complication of abdominal sepsis. The authors present a case of pylephlebitis after perforated retrocecal appendicitis in a child and discuss the etiology, presentation, diagnosis, and treatment of this disorder in the modern era.

Single-blind randomized clinical trial of laparoscopic versus open appendicectomy in children.

BACKGROUND: Appendicectomy is the most common emergency surgical operation in children. The aim of this study was to compare recovery after appendicectomy using either a laparoscopic or an open technique in children. METHODS: Sixty-one children aged 4-15 years undergoing appendicectomy for suspected uncomplicated appendicitis were studied. The study was prospective, randomized and single-blinded, with parallel groups. Standardized anaesthetic technique and pain management were used. The study endpoints were postoperative pain, need for rescue analgesia, and length of hospital stay. RESULTS: Children had significantly less pain after laparoscopic compared with open appendicectomy 8 h after operation and on the first and second postoperative mornings (P < 0.05). Laparoscopic appendicectomy resulted in a reduced requirement for rescue analgesia with oxycodone in hospital: mean(s.d.) 3.6(2.5) versus 5.8(3.3) doses (mean difference 2.2 doses, 95 per cent confidence interval (c.i.) 0.6--3.8 doses); P = 0.01. The mean(s.d.) length of hospital stay was significantly shorter in the laparoscopic group: 1.9(0.7) versus 2.6(0.9) days (mean difference 0.7 days, 95 per cent c.i. 0.3--1.1 days); P = 0.001. Three children who had open appendicectomy developed a wound infection. CONCLUSION: Laparoscopic appendicectomy is associated with less postoperative pain and a shorter hospital stay than open appendicectomy in children undergoing surgery for uncomplicated appendicitis.

Lung development following diaphragmatic hernia in the fetal rabbit.

Diaphragmatic hernia was created in 39 rabbit fetuses on day 23 of gestation. Fifteen fetuses underwent a sham thoracotomy (SHAM). Thirty-nine non-operated littermates served as internal controls (CTR). Fetuses were harvested by Caesarean section on days 25, 27, 29 and 30 of gestation. Pulmonary response was evaluated by lung to body weight ratio (LBWR), morphometry, and density of type II pneumocytes. No difference was found between CTR and SHAM fetuses at term. CDH fetuses had smaller lungs (LBWR 0.014 +/- 0.004 versus 0.030 +/- 0.04 in CTR, P < 0.0001), a less complex acinus [mean terminal bronchial density (MTBD) 1.786 +/- 0.408 versus 0.917 +/- 0. 188, P < 0.0001], thicker alveolar septa [mean wall transection length (LMW) 0.0221 +/- 0.008 versus 0.0142 +/- 0.002, P = 0.0003], and a lower type II cell count (144.5 +/- 19.33 versus 216.2 +/- 27.85 per high power field, P < 0.0001). The differences in MTBD and LMW were significant from gestational day 25 onwards, and the differences in type II cell count from day 27 onwards. Surgical diaphragmatic hernia in rabbit fetuses in the late pseudoglandular phase reproduces many features of the pulmonary hypoplasia associated with human congenital diaphragmatic hernia, including the delayed maturation. The effects are present within 2 days following experimental diaphragmatic hernia and progress over time.

Silo reduction of giant omphalocele and gastroschisis utilizing continucous controlled pressure.

A method is described utilizing continuous controlled pressure to achieve smooth, rapid, and safe silo reduction of an anterior abdominal-wall defect. A metal tube with larger wheels at each end is suspended by runners and counterweights to slowly roll the silo and squeeze the contents into the abdominal cavity.

Indications for laparotomy in infection with verotoxigenic Escherichia coli.

Verotoxigenic types of Escherichia coli have emerged as serious and important human pathogens. The clinical disease most frequently manifests as a painful form of bloody diarrhea, which can progress to life-threatening systemic microangiopathic hemolytic anemia, known as the hemolytic-uremic syndrome (HUS). Three children with hemorrhagic enteritis due to verotoxigenic E. coli are presented to illustrate the unique diagnostic, therapeutic, and operative management dilemmas associated with this disease. When contemplating surgery, one should seek to determine the anatomic and transmural extent of the disease.

Needle design does not affect the success rate of spinal anaesthesia or the incidence of postpuncture complications in children.

BACKGROUND: In adults, pencil-point spinal needles are believed to be less traumatic and therefore to be superior compared to cutting-point needles with respect to success rate and postpuncture complications. The aim of this randomised, parallel groups and prospective study was to record the success rate and to evaluate the incidence of complications following spinal anaesthesia with the two types of needles in children. METHODS: We studied 215 children aged 1 to 18 years. A 25-gauge needle was used in children up to 7 years (n=96) and a 27-gauge needle in older children (n=119). During lumbar puncture with either a cutting-point (n=109) or a pencil-point (n=106) spinal needle, we recorded puncture characteristics and the success of cerebrospinal fluid (CSF) aspiration. Hyperbaric bupivacaine 5 mg ml(-1) at a dose of 0.3-0.4 mg kg(-1) was used for the spinal anaesthesia. The incidence of postdural puncture complications was recorded from diaries completed by the children and parents one week after the lumbar puncture. RESULTS: The success rate of the spinal anaesthesia was 97% without difference between the needles. The success rate was higher when the aspiration of CSF was easy compared to if it was difficult (98% vs. 88%, P=0.02). Two hundred and seven diaries were returned (97%). Twenty-four children developed a headache, 8 of which were classified as a postdural puncture headache (PDPH), 6 with the cutting-point needle and 2 with the pencil-point needle (n.s.). Nine children developed signs of transient radicular irritation with no difference between the needles. CONCLUSION: Both types of spinal needles can be used in children, and a free aspiration of CSF results in a high success rate of the spinal block. Postpuncture complications are as common in children as in adults.

Congenital diaphragmatic hernia--does the side of the defect influence the incidence of associated malformations?

BACKGROUND/PURPOSE: Patients with congenital diaphragmatic hernia (CDH) frequently have associated anomalies. Experiments in the nitrofen CDH model have shown differential embryonic cell death patterns in rodents suggesting unique mechanisms in the formation of right-sided (RCDH) or left-sided (LCDH) diaphragmatic hernia. These findings provide insight into the pathogenesis of CDH and may aid our understanding on the spectrum of associated anomalies commonly observed in humans. This study therefore set out to test the hypothesis that the side of the diaphragmatic defect in humans is related to the incidence and severity of coexistent organ malformations. METHODS: The medical and autopsy records of 301 CDH patients presenting to two institutions over a 23-year period were examined to analyze these factors. RESULTS: One hundred patients (33%) were found to have one or more associated anomalies. The incidence of multiple-RCDH (10%) versus LCDH (7.3%) and cardiac anomalies-RCDH (10%) versus LCDH (8.5%) was similar in both groups of patients. However, the hypoplastic heart syndrome was a unique feature in 5 of 22 patients (23%) with LCDH who had cardiac abnormalities. This cardiac anomaly may be related developmentally to LCDH. CONCLUSION: The cellular mechanisms underlying the genesis of this spectrum of abnormalities in humans and the nitrofen CDH model warrant further study to elucidate factors governing embryonic cell fate and phenotype expression.

Maternal serum alpha-fetoprotein and epithelial tumour marker concentrations are not increased by fetal sacrococcygeal teratoma.

Maternal serum alpha-fetoprotein (AFP) and total beta-human chorionic gonadotrophin (hCG) concentrations were measured at the 15th gestational week in ten pregnancies complicated by fetal sacrococcygeal teratoma. The findings corresponded to those in normal pregnancies. Similarly, third-trimester concentrations of cancer antigen 12-5, tumour-associated trypsin inhibitor, and the amino-terminal propeptide of type III procollagen in the maternal serum were not significantly elevated. Histologically mature and immature/malignant cases did not differ from each other as regards the above-mentioned parameters. This abnormality cannot be detected by maternal serum trisomy screening in the second trimester.

A 45-year perspective of congenital diaphragmatic hernia.

The records of 174 consecutive patients with congenital diaphragmatic hernia were reviewed to analyse the changes in the presentation, treatment and outcome during the 45-year interval from 1948 to 1992. For comparison the period was divided into years 1948-1962, 1963-1972, 1973-1982 and years 1983-1992. The proportion of high-risk cases (symptomatic within 6 h of birth) increased from 50 per cent during the first period to 77 per cent during the second, to 86 per cent during the third and to 94 per cent during the fourth. The primary mortality rate (death within 30 days of diaphragmatic repair) increased from 25 per cent during the first period to 35 per cent during the second, 46 per cent during the third and 49 per cent during the fourth. No change occurred in the proportion of infants with diaphragmatic hernia who were stillborn or who died in the maternity unit. Post-mortem lung weight was available in 56 children. Severe pulmonary hypoplasia correlated with early postoperative death and clinical severity. The mean(s.d.) lung weight ratio (combined lung weight divided by the lung weight expected for the body-weight) decreased from 0.70(0.49) during the second period to 0.56(0.36) during the third and 0.40(0.18) during the fourth. The increased proportion of more severe cases with very hypoplastic lungs explains the rise in the mortality rate of patients operated on for congenital diaphragmatic hernia. This may reflect a real change in the disease spectrum rather than improved referral.

Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects.

Between 1948 and 1980, 107 of 164 patients survived after repair of a congenital diaphragmatic defect. Sixty of the survivors (mean age, 29.6 years; SD, 9.0 years) underwent clinical examination, chest radiography, spirometry, and diffusing capacity measurement 11 to 41 years after the diaphragmatic repair. Twenty-seven of the 60 had body plethysmography, xenon 133 radiospirometry, and a test of bronchial hyperreactivity. Subjective physical performance was below average for eight patients (13%), seven patients (12%) had asthma, and four patients (7%) reported increased susceptibility to respiratory infections. Thirty-one patients (52%) had ventilatory impairment, which was obstructive in nine (15%), restrictive in seven (12%), and obstructive and restrictive in 15 patients (25%). Nine (35%) of the 26 patients tested had bronchial hyperreactivity. The presence of ventilatory impairment and bronchial hyperreactivity correlated with the initial clinical severity of the affliction. Chest asymmetry (29 patients; 48%) and scoliosis (16 patients; 27%) were more common among patients with ventilatory impairment than among those with normal spirometric findings. Ventilatory impairment and thoracic deformities are common in adults with repaired diaphragmatic defects. Surveillance should begin in infancy and continue into adulthood.

Chest wall and spinal deformities in adults with congenital diaphragmatic defects.

Between 1948 and 1980, 107 of 164 patients survived after repair of congenital diaphragmatic defects. To study chest wall and spinal deformities among these patients, 60 survivors (mean age, 29.6 +/- 9.0 years) underwent clinical examination, chest and spinal radiography, spirometry, and diffusing capacity measurements. Twenty-seven of these patients also had body pletysmography, xenon 133 radiospirometry, and a test for bronchial hyperreactivity. Chest asymmetry was present in 29 patients (48%). Eleven patients (18%) had pectus excavatum, and one had a pectus carinatum deformity. Anterior asymmetry, pectus deformities, and a flat chest were more common among the patients who initially had a large diaphragmatic defect. Two patients underwent anterior thoracoplasty because of their deformities. Sixteen patients (27%) had significant scoliosis (Cobb angle > or = 10 degrees). Scoliosis was more common in patients with ventilatory impairment than in those with normal lung function. One patient required spinal stabilization, one with severe kyphoscoliosis remains under observation, and a patient with moderate scoliosis was treated with a spinal brace. The results of this study indicate that chest wall deformities and scoliosis are common among adults with repaired congenital diaphragmatic defects. In most patients the deformity is mild, but some will require surgery. Surveillance until adulthood is appropriate.

Long-term gastrointestinal morbidity in patients with congenital diaphragmatic defects.

Recent reports indicate a significant incidence of gastroesophageal reflux (GER) and other nonpulmonary problems after the repair of congenital diaphragmatic defects. Reports of follow-up through adulthood are few and based on a small number of patients. From 1948 to 1982, 107 of 164 patients (65%) treated at the authors' institution survived after repair of congenital diaphragmatic hernia or eventration. Sixty of the 107 survivors (56%) (mean age, 29.6 years; SD, 9.0 years) were interviewed and examined clinically. Forty-one of the sixty (68%) underwent upper gastrointestinal endoscopy. Early postoperative GER was recorded for 11 of the 60 patients (18%). Two of them underwent fundoplication because of an esophageal stricture. At the time of the follow-up study, 38 of the 60 (63%) reported symptoms suggestive of GER. Endoscopic or histological GER (esophagitis, Barrett's esophagus) was present in 22 of 41 patients (54%). No significant correlation between the initial severity of the diaphragmatic defect or neonatal postoperative problems and the late GER could be verified. Intestinal obstruction requiring hospital admission occurred in 12 of the 60 patients (20%), eight of whom had surgical treatment between 1 month and 20 years after repair of the diaphragmatic defect. GER and intestinal obstruction are common among patients who have undergone repair of a congenital diaphragmatic defect. Investigations for GER should be performed routinely during the follow-up of these patients.

Congenital eventration of the diaphragm - 45 years' perspective.

Fifty-five children with congenital eventration of the diaphragm were operated upon in a single tertiary-care children's hospital during the 45-year period from 1948 to 1992. The study was divided into three periods: 1948-1962, 1963-1977, 1978-1992. There were 14 patients in the first group, 26 in the second, and 15 in the third. Children later in the series presented earlier and with more acute symptoms. Mortality was related to severe pulmonary hypoplasia and associated anomalies, especially chromosomal defects: 14% during the first period, 27% during the second, and 7% during the third.