BioEnterics intragastric balloon for treatment of morbid obesity in Prader-Willi syndrome: specific risks and benefits.

BACKGROUND: Obesity in Prader-Willi Syndrome (PWS) is progressive, severe, and resistant to dietary, pharmacological, and behavioral treatment. A body weight reduction is mandatory to reduce the risk of cardio-respiratory and metabolic complications. The aim of the study was to assess risks and benefits of BioEnterics Intragastric Balloon (BIB) for treatment of morbid obesity in PWS patients. METHODS: Twenty-one BIB were positioned in 12 PWS patients (4 M, 8 F), aged from 8.1 to 30.1 years, and removed after 8 +/- 1.4 months (range: 5-10 months). Auxological, clinical, and nutritional evaluations were performed every 2 months. Variations in body composition were analysed by dual energy X-ray absorbiometry (DXA). RESULTS: One patient (28.5 years, BMI: 59.3 kg/m(2)) died 22 days after BIB positioning because of gastric perforation. In another case (26.2 years, BMI: 57.6 kg/m(2)), BIB was surgically removed after 25 days because of symptoms suggesting gastric perforation (not confirmed). The remaining ten patients showed a significant decrease of BMI (p = 0.005) and of fat tissue as measured by DXA (p = 0.012). No significant modifications in bone mineral density (BMD) occurred, but a slight loss in lean body mass (p = 0.036) was documented. In five patients, BIB treatment was repeated more than once. CONCLUSION: This study shows that when noninvasive pharmacological therapies fail, BIB may be effective to control body weight in PWS patients with morbid obesity, particularly when treatment is started in early childhood. However, careful clinical follow-up and close collaboration with parents are crucial to avoid severe complications, which can be caused by persisting unrestrained food intake.

Prenatal diagnosis and clinical outcome of ovarian cysts.

Technical refinements of ultrasound (US) have greatly affected the antenatal diagnosis and treatment of ovarian cysts. From 1985 to 1990 25 consecutive fetuses with ovarian cysts were followed-up by US both during pregnancy and postnatally. All cases were diagnosed between the 28th and 39th weeks of gestation. Deliveries were all at term; cesarean section was required only for obstetric complications. Eight fetuses (32%) showed US patterns of cyst torsion, a finding confirmed at surgery in all. In five patients US patterns suggested complications postnatally that were also confirmed at operation. In six cases cysts increased or remained unchanged in size after 15 days of life: in 50% of these surgery showed ovarian torsion. In the remaining six cases spontaneous resolution occurred within 1 to 4 months. One patient required intrauterine needle aspiration. There were two cases of intestinal obstruction. To date, more than 60% of newborns with ovarian cysts require oophorectomy; however, different treatments (cystectomy, needle aspiration, uncapping) combined with a close US follow-up are likely to reduce this percentage.

Spinal dysraphism detected by magnetic resonance imaging in patients with anorectal anomalies: incidence and clinical significance.

Though the concept of caudal regression, suggested in 1961 by Duhamel, could explain the association between anorectal anomalies (ARA) and spinal dysraphism (SD), its real incidence may be underestimated and its clinical significance is debatable. From 1988 to 1993, 111 patients with ARA were treated at the authors' institution. Associated anomalies were present in 36% of cases, with the exception of vesicoureteral reflux, which was considered functional rather than anatomical. In view of the late (1 to 2 years after surgical treatment) onset of vesical dysfunction and/or orthopaedic symptoms in some of these patients, a screening protocol was started in 1991, using magnetic resonance imaging (MRI) in all patients with ARA. Fifty patients, 29 boys and 21 girls, underwent a spinal cord MRI, with pathological findings in 25 cases (50%), 13 boys and 12 girls. The authors did not find any significant difference in incidence with respect to high, low, or cloacal malformations. A thickened filum, with or without fibrolipoma, was the most frequent finding, but even tethered cord, syringomyelia, and sac morphological alterations were present. MRI was also able to detect osteoarticular and/or muscular anomalies. Even when a urodynamic study and a neurological and orthopaedic workup were performed in 20 patients undergoing MRI, the clinical significance of these findings remained unclear. However, accurate follow-up of these patients is mandatory in order to detect early neurological symptoms, because currently it is not advisable to refer for neurosurgery all the patients with ARA presenting with anomalies of the spinal cord.

Physical fitness testing in children operated on for tracheoesophageal fistula.

The maximal physical activity capacity of children operated on for tracheoesophageal fistula (TEF) has not been clearly defined. Eight patients (average age, 12 years) successfully operated on for TEF at birth underwent maximal exercise stress testing on a treadmill, according to the Bruce protocol, to test physical work capacity. Heart rate (HR), oxygen consumption (VO2), and pulmonary ventilation (VE) were measured by a portable lightweight telemetric device. Nine healthy children served as controls. Exercise duration was significantly lower for TEF subjects than for controls (11.6 +/- 1.7 minutes v 15.1 +/- 2.3 minutes; P < .01). Mean HR at rest and during exercise did not differ between the groups. All children reached the maximum HR according to their age; however, for the majority of TEF patients, this occurred at an earlier stage than in the controls. No differences were seen in mean VO2 at rest and on exertion between TEF and control children. However, maximal VO2, as measured at the end of exercise, was significantly different when normalized per kilogram of body weight (VO2/kg = 52.3 +/- 5.8 v 33.3 +/- 6.6; P < .005). The physical rehabilitation of TEF children usually takes into account only respiratory and nutritional factors. However, complete assessment of their cardiac and respiratory function, at rest and on exertion, also should be performed, because this may show that some patients have reduced motor performance; evidence is now accumulating that these children can safely participate in the same physical activities of their healthy peers.

Stenting for caustic strictures: esophageal replacement replaced.

METHODS: From 1983 to 1996, 31 children with caustic esophageal strictures were seen at Bambino Gesù Children's Hospital; they were all treated conservatively except for two cases complicated by tracheoesophageal fistula. The remaining 29 patients were divided into three groups depending on the treatment, which was modified over the years. Group A (1983 to 1987) consisted of seven patients treated by periodic dilatations; group B (1988 to 1992) consisted of 10 children treated by 40 days of esophageal stenting plus dexamethasone, 0.5 mg/kg/d plus ranitidine plus no oral feeding for 7 to 10 days; group C (1993 to 1996) consisted of 12 cases treated by 40 days of esophageal stenting plus dexamethasone, 1 mg/kg/d plus omeprazole plus early oral feeding resumption. RESULTS: No differences were observed between the three groups of patients with regard to the mean age and to the ingested substance, whereas a significant difference (P = .007) was observed in the mean length of the stricture between group A and C (3.4+/-1.3 and 5.6+/-1.6 cm, respectively). In all but one of the patients (96.5%) complete healing of the stenosis was achieved by conservative treatment, with definitive relief of dysphagia. One patient in group C did not improve after a repeated stenting procedure and was surgically treated. However, in group A, resolution of the stricture was obtained after an average of 19.9+/-14.8 dilatations in a mean period of 25.3+/-17.2 months. In group B, a mean of 12+/-11.3 dilatations were required in a mean period of treatment of 14.1+/-10.6 months. In patients in group C, a mean of 3.5+/-3.2 dilatations were necessary in a mean of 5.8+/-4.8 months. A statistically significant difference was observed both with regard to the number of dilatations and to the duration of treatment, between group A and group C (P = .002) and group B and C (P = .03). CONCLUSION: Esophageal replacement should be considered only in cases complicated by tracheoesophageal fistula or in the rare patients who do not respond to repeated esophageal stenting.

Maximal oxygen consumption and stress performance in children operated on for congenital diaphragmatic hernia.

The long-term follow-up of patients operated on for congenital diaphragmatic hernia (CDH) at birth has been extensively evaluated, both clinically and with respect to respiratory function. However, little is known about the sports practice and stress performance of these subjects. Fifteen of 107 patients operated on for CDH underwent exercise stress testing with a stepwise increase in workload. A questionnaire was provided, which requested information on sports practice and lifestyle. Maximal oxygen consumption [Vo2 max] was measured along with dynamic lung volumes. Clinical examination included a whole-body assessment (height, weight, skinfolds) and vital parameters (heart rate and blood pressure). Fifteen healthy children who practiced regular physical activity (2 to 4 hours/week) served as controls. All the CDH patients experienced a good lifestyle, but only 8 of them were participating in sports. Exercise duration and Vo2 max were significantly lower for the CDH patients, and were lowest for the sedentary patients. Therefore, the reduced Vo2 max of these otherwise healthy children most likely represents a lower degree of physical fitness rather than decreased respiratory function. Fitness is an expression of well-being; thus, there is evidence that these patients could safely participate in competitive motor activities.

Antenatal ultrasonographic diagnosis and management of fetal ovarian cysts.

OBJECTIVES: The aim of the present study was to evaluate the outcome of fetal ovarian cysts in relation to their ultrasonic appearance and size. METHODS: Forty-two fetal ovarian cysts were diagnosed in 41 fetuses and followed with serial ultrasonograms in utero and after birth until spontaneous or surgical resolution. RESULTS: Twelve fetal ovarian cysts that were echogenic at diagnosis and six that were anechoic at diagnosis but became echogenic at subsequent prenatal sonograms were all submitted to postnatal surgery. Of the remaining 24 cysts, all anechoic, four were successfully aspirated in utero, 11 resolved spontaneously after birth, and nine underwent postnatal surgery for complication. The outcome of cysts that were anechoic at diagnosis was significantly correlated with size (P = 0.01). CONCLUSIONS: Echogenic fetal ovarian cysts should be always surgically removed. The outcome of anechoic cysts depends on the size at diagnosis, and serial ultrasonographic assessment is recommended; although not randomized, the present series suggests that in utero aspiration of cysts > 5 cm may prevent complication and subsequent oophorectomy.

The incidence of occult spinal dysraphism and the onset of neurovesical dysfunction in children with anorectal anomalies.

The urological malformations associated with anorectal anomalies (ARA) are not only anatomical, but also functional, the latter being related to congenital neurovesical dysfunction (NVD). The true incidence of spinal dysraphism (SD) in these children is still unclear and is probably underestimated. The concept of caudal regression could explain its association with the anorectal anomalies. Because of awareness of the late onset of neurovesical dysfunction and/or orthopaedic symptoms in some of our patients, in 1991 we started to screen with magnetic resonance imaging (MRI) the spinal cord of all patients with ARA. Eighteen (44%) out of 41 patients without neurological or orthopaedic symptoms and 7 (78%) of 9 children with neurological or orthopaedics symptoms screened by MRI showed pathological findings. The overall incidence of spinal dysraphism in ARA was 50%, without any great difference with respect to the type of the anomaly (high, low, cloacal). The pathological MRI findings encountered were: fibrolipoma (with or without a thickened filum terminale), tethering of the cord and syringomyelia, and sac anomalies. In order to check the onset of NVD in these children, we performed urodynamic studies with external sphincter electromyography in 24. Grouped by age: 14 were between 5 and 18 months and ten were 4 to 13 years old. Ten patients (71%) out of the first group and 3 (30%) out of the second had a normal urodynamic pattern. A total of 11 children had pathological findings; of these, 4 infants had a hyperreflexic bladder (one with detrusor-sphincter dyssynergia) suggesting upper motor neuron (UMN) lesion. Of the 7 older patients, two had UMN and 3 lower motor neuron (LMN) lesion.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurovesical dysfunction due to spinal dysraphism in anorectal anomalies.

Spinal dysraphism (SD) has been found associated with functional abnormalities of anorectal anomalies (ARA). The incidence of SD in these children is probably underestimated and a complete neuroradiological investigation of the lower urinary tract function has not been carried out routinely. In a 2 years time frame we performed urodynamic (UDS) evaluations on 14 patients (8 males and 6 females) with ARA who showed SD at Magnetic Resonance Imaging (MRI) studies. We divided them into 2 groups by age: group A (5 to 18 months) and group B (3 to 12 years). The UDS findings were classified as upper (UMN) and lower motor neuron (LMN) lesion. Out of the 9 children included in group A, 5 showed normal urodynamic pattern, while 4 had pathological UDS findings suggesting UMN lesion. Among the older (group B) children only one had normal and 4 had pathological findings: 2 hyperreflexia suggesting UMN lesion and 2 external sphincter denervation suggesting LMN lesion. These data support the hypothesis that the neurovesical dysfunction found in children with anorectal anomalies results from a possible association with spinal dysraphism. An early morphological evaluation of the spinal cord is mandatory in all children with ARA prior to definitive surgical correction of the malformation. The low incidence of pathological UDS findings in small children if compared to older ones suggest that SD, although present, may be asymptomatic.

Ultra-short-bowel syndrome is not an absolute indication to small-bowel transplantation in childhood.

Short-bowel syndrome (SBS) either in adults or in children is considered as an indication to small-bowel transplantation (SBTx), particularly in its most severe form with a residual bowel length below 20 cm. Among factors likely to worsen the prognosis, more recent reports also indicate the number of surgical interventions, early onset sepsis and early development of liver disease. We report six cases of ultra-short-bowel syndrome followed from birth to verify the importance of various prognostic factors. In our case series, the male sex is predominating (5:1). Intestinal resection was indicated in 3 patients for multiple intestinal atresias, in 2 for volvulus and in 1 for necrotizing enterocolitis. The length of intestine remaining was invariably less than 20 cm and 2 patients had a preserved ileocecal valve. In most cases, more than 50% of the colon remained. The number of abdominal operations ranged from 1 to 4. In almost all cases (5 of 6), sepsis and hepatopathy developed early. Our experience suggests that rather than depending on the length of intestine remaining or the presence of the ileocecal valve, the prognosis of patients with the extreme-short-bowel syndrome depends on recurrent neonatal onset sepsis and early onset liver impairment. In addition, our case review shows that the extreme-short-bowel syndrome is not necessarily an indication for bowel transplantation.

Ulcerative colitis in children under 10 years of age: medical and surgical treatment.

Ulcerative colitis is seen with increasing frequency in paediatric age and its diagnosis is made more difficult by atypical cases. Sixty-five patients with UC were seen at our institute and all of them underwent medical treatment. In all patients the disease extended to the whole colon (pancolitis). Eleven patients (average age 9 yrs) underwent surgical correction by Endorectal Pull Through (EPT) 8 straight and 3 with ileal reservoir. One straight EPT had to be converted to Brooke ileostomy because of unacceptable stool frequency. In the rest of the patients the disease is well controlled with medical treatment. After 2 years of follow up surgical complications, continence, stool frequency and quality of life were evaluated: results indicate that surgical complications rate is the same as in other reported series; furthermore, continence and stool frequency are good with all surgical techniques eve though straight pull-through may require a period of adaptation the length of which varies considerably. Our results confirm that children with pancolitis and severe symptoms should be offered prolonged medical treatment prior to undertake surgical correction.

[Tumors of Meckel's diverticulum]. / Tumori del diverticolo di Meckel.

The presentation of a rare case of leiomyoma of Meckel's diverticulum in a boy of 5 yr forms the starting point for a discussion of such cancers. In adult age, the complication is not so rare as the sporadic publications would suggest, incidence of tumoral forms being around 1 : 50 M.D. with a high malignancy frequency. In paediatric age, on the other hand, the tumoral degeneration of a M.D. is an exceptional occurrence: a review of more than 1,200 reported cases and personal experience (48 cases of M.D. in 15 yrs) confirm the rarity of the observation. The need to seek M.D. on each occasion and remove it to prevent the risk of tumoral degeneration in adult age is stressed. M.D. surgical removal tecniques are commented on and emphasis laid on the desirability of ample intestinal resection in cases of cancer of the diverticulum.

[Hepatic resection in the fetal rabbit. Histologic comparison of tissue regeneration in the fetus versus the adult]. / Resezione epatica fetale nel coniglio. Confronto istologico della rigenerazione tissutale del feto e dell'adulto.

Fetal tissues present peculiar features of repair after injury. Although the development of fetal hepatocytes have already been studied in vitro and in transplant models, an in vivo study of fetal liver regeneration is still missed in the literature, to the best of our knowledge. Eight time-dated pregnant California rabbits (23, 24, 25, 30 days of gestational age) and 2 adult male California rabbits were anesthetized following a standardized i.v. protocol (ketamine 50 mg/kg; xilazine 5 mg/kg; propiopromazine 0.75 mg/kg; spontaneous breathing; no anesthetic gas). All the pregnant does underwent a midline laparotomy and a minimal hysterotomy to approach a fetus per each animal. In 2 cases, 1 fetus was delivered and prior to sacrifice the fetal liver was sampled in toto (30 days of gestational age). These pregnancies were allowed to continue to term and were uneventful with a full-term spontaneous delivery of the remaining fetuses. In the other 6 pregnancies, after the hysterotomy, the fetal abdomen was entered through a right-sided longitudinal incision and the liver was partially resected by thermocauterization. Fetal abdomen was closed in 1 layer (non absorbable suture 7-0). The fetus was then returned in the uterus and, after amniotic fluid restoration with warmed saline, the hysterotomy was sutured in double layer (polyglycolic 5-0). Maternal abdomen was closed in 1 layer (polyglycolic 4-0) and the skin in a continuous overlying fashion (silk 3-0). The abdominal cavity of the 2 adult male rabbits was entered through a right subcostal incision. Partial liver resection was performed, and abdominal and skin closure followed the same techniques used for the pregnant does. The treated livers were then sampled in toto at 24, 48, 72 hrs and 4 days after surgery from the fetuses, and at 7 days from the adult rabbits. Histological stains were: H & E; Van Gieson; Masson; Alcian Bleu; PAS. Fetal histology showed a low inflammatory reaction poor in PMN cells with minimal deposition of collagen and a high amount of glycogen in the hepatocytes. The inflammatory response to resection was much more evident in the adult samples as much as the abundant intra and extra-cellular deposition of collagen associated to a minor amount of intracellular glycogen. The peculiar features of liver regeneration in the fetus, deserve further experimental studies.

[Haemophilus influenzae type b in meningitis: antibiotic resistance in pediatric patients]. / Haemophilus influenzae tipo b: antibioticoresistenza in una casistica pediatrica di meningiti purulente (1985-1994).

A retrospective study on 357 children admitted to four Pediatric Infectious Disease Centers in Rome, affected by acute meningitis, during 10 years period, between January, 1, 1985 and December, 31, 1994 was carried out. Haemophilus influenzae type b was detected in 110 patients; all children aged between 1 month and 5 years; the maximum incidence (74.5%) was observed in patients under two years. The following diagnostic criteria were utilized: Gram stain of CSF; Latex test on CSF, blood, urine; CSF and blood cultures. The in vitro sensitivity of 65 isolates was tested by using the Kirby-Bauer method. We detected 15.3% of strains resistant to Ampicillin and 1.5% resistant to CAF. We also observed a high number of Hib strains resistant to Erythromycin and Cotrimoxazole. Only one strain Ceftriaxone resistant was isolated, confirming the high in vitro sensibility Hib to III generation cephalosporins that still remain the first choice drugs in Hib meningitis.

[Otomastoiditis caused by Mycobacterium avium: a case report]. / Otomastoidite da Mycobacterium avium: descrizione di un caso.

Non tuberculous Mycobacterial (NTM) Infections mainly affect immunocompromised patients, appearing as disseminated or pulmonary disease. In immunocompetent children the most common form of infection with NTM is cervical adenitis. Ear infection seems to be a rare disease. We present a case of otomastoiditis caused by Mycobacterium avium in a 15 months old child, immunologically normal. Patient was referred for persistent right otitis unresponsive to routine medical therapy. TC scan of the ear and temporal bones revealed: soft tissue in external auditory canal, Eustachian canal, and middle ear overlying ossicles with erosion of tegmen tympani. Tuberculin skin test was positive with 5 units PPD and culture yielded M. avium. The patient undergo timpanomastoidectomy and medical therapy with antituberculous drugs and Steroids, subsequently he was given Clarithromycin and Rifabutin. M. avium is an ubiquitous low grade pathogen found in soil, water, dust and food. There is no evidence of direct transmission. Only a few cases of otomastoiditis due to M. avium have previously been reported. The case presented underlines the importance of microbiological investigations. When a NTM infection is suspected surgeons and infectious diseases specialists should cooperate to find an optimal treatment regimen of this unusual disease.

[Bacterial meningitis and CSF cytokines]. / Meningiti batteriche e citochine liquorali.

Aim of study is the determination of concentrations of two important cytokines: TNF alfa and IL8 in children with bacterial meningitis to establish a correlation between infection, CSF concentration of cytokines and neurological sequelae. TNF alfa and IL8 concentrations in CSF have been measured by quantitative immunometric enzyme assay during the course of the disease. In the purulent meningitis we observed that CSF concentrations of these cytokines decreased to undectable values 24 to 48 hours after beginning of the antibiotic therapy. Conversely, in the 3 patients with mycobacterial meningitis (TBM) the concentrations of IL8 were higher for a longer period, being detectable in the CSF between 4 and 8 weeks after the beginning of the specific treatment. We found no significant differences of the values of IL8 in children with neurological sequelae compared with children without sequelae.

[Stenosis of the colon in childhood]. / La stenosi del colon in età pediatrica.

The authors report 6 cases of colonic stenosis (three males and three females, range of age 9 days-4 months) observed from 1982 to 1985 in the Department of Pediatric Surgery in Bambino Gesù Hospital. Three out of six have been admitted with the diagnosis of necrotizing enterocolitis soon after birth. The authors have observed 2 cases of colonic stenosis in neonatal age, 1 case aged month, both suffering from Hirschsprung's disease. A file for the diagnosis has been performed for all patients. Stenosis have been observed in the descending colon, sigmoid colon, rectosigmoid tract, multiple stenosis were present in 2 patients. In 5 patients the surgical treatment has been the removal of stenotic tract. In three patients a colostomy on transverse colon has been performed followed by abdominal perineal intestinal lowering. The authors stress the importance of anorectal manometry among investigations usually performed to exclude Hirschsprung's disease.

Appendiceal abscess and right ovarian teratoma: a rare association.

Many pediatric patients presenting to the Emergency Department with acute abdomen are subject to one or even more than one imaging modalities in order to determine the reason for the patient's symptoms. Most of the times, imaging can render an accurate diagnosis and help clinicians and surgeons for the decision making plan and further management. In some circumstances, image findings are equivocal, non specific or simply misinterpreted and a correct diagnosis cannot been made preoperatively. Authors present a unique case of an unexpected acute appendicitis found during surgical exploration performed in order to remove an ovarian tumor in an eight-year-old girl.