Fatal cardiac tamponade as a late complication of central venous catheterization: a case report.

Central venous catheterization is a reliable technique in neonatal surgery. Nevertheless, the rate of mechanical catheter-related complications remains high. We report a neonate with gastroschisis in which the successful placement of a central venous catheter was followed later by a cardiac tamponade with a fatal outcome. This complication occurred without perforation of the cardiac wall. A similar finding has been reported in only one other pediatric patient. Vigilant observation is required in any neonate with a central venous line in place to prevent the occurrence of this life-threatening event.

Risk factors for latex allergy in patients with spina bifida and latex sensitization.

BACKGROUND: Some subjects with spina bifida (SB) and latex sensitization (cutaneous and/or serum IgE) can have clinical reactions, while others may have no symptoms after the exposure to latex products. OBJECTIVE: This study was carried out to determine the risk factors associated with latex allergy in patients affected with SB and latex sensitization. METHODS: Fifty-nine consecutive subjects affected with SB, besides answering a questionnaire, underwent a skin-prick test (SPT) to latex and the determination of the specific serum IgE (RAST CAP) to latex. The total serum IgE concentration was determined and SPT to common aero- and food-allergens, skin tests (prick + prick) with fresh foods (kiwi, pear, orange, pineapple, tomato, banana) and RAST CAP to the same foods tested by the prick + prick technique, were also performed. RESULTS: Fifteen out of the 59 subjects (25%) were sensitized to latex according to the presence of IgE to latex detected by SPT (nine patients) and/or RAST CAP (13 patients). Five out of the 15 sensitized patients (33%) suffered from clinical reactions to latex (urticaria, conjunctivitis, angioedema, rhinitis, bronchial asthma) while they were using latex gloves and while inflating latex balloons. In the 15 sensitized patients, the presence of specific latex seric IgE > 3.5 kU/L (>/= class 3), a positive latex SPT, an elevated total serum IgE, and a positive prick + prick and/or a positive RAST CAP to foods, were significant (P < 0.05) risk factors associated with latex symptoms. The other factors tested (age, gender, months of intermittent bladder catheterization, surgical procedures, SPT reactivity and clinical reactions to aero-allergens and food-allergens, skin tests for fresh foods, positive RAST CAP to foods) were not significantly different in symptomatic and asymptomatic patients. CONCLUSION: Significant risk factors for symptoms to latex in patients with SB and latex sensitization were a presence of specific IgE to latex > 3.5 kU/L, a more frequent positive latex SPT, elevated total IgE, and one or more positive prick + prick and/or RAST CAP to fresh foods.

Prevalence of and risk factors for latex sensitization in patients with spina bifida.

PURPOSE: We determined the prevalence of and risk factors for latex sensitization in patients with spina bifida. MATERIALS AND METHODS: A total of 59 consecutive subjects 2 to 40 years old with spina bifida answered a questionnaire, and underwent a latex skin prick test and determination of serum IgE specific for latex by RAST CAP radioimmunoassay. We also noted the relationships of total serum IgE skin prick tests to common air and food allergens. In addition, skin prick plus prick tests were also done with fresh foods, including kiwi, pear, orange, almond, pineapple, apple, tomato and banana. RESULTS: Latex sensitization was present in 15 patients (25%) according to the presence of IgE specific to latex, as detected by a skin prick test in 9 and/or RAST CAP in 13. Five latex sensitized patients (33.3%) had clinical manifestations, such as urticaria, conjuctivitis, angioedema, rhinitis and bronchial asthma, while using a latex glove and inflating a latex balloon. Atopy was present in 21 patients (35.6%). In 14 patients (23%) 1 or more skin tests were positive for fresh foods using a prick plus prick technique. Tomato, kiwi, and pear were the most common skin test positive foods. Univariate analysis revealed that a history of 5 or more operations, atopy and positive prick plus prick tests results for pear and kiwi were significantly associated with latex sensitization. Multivariate analysis demonstrated that only atopy and a history of 5 or more operations were significantly and independently associated with latex sensitization. CONCLUSIONS: A fourth of the patients with spina bifida were sensitized to latex. Atopy and an elevated number of operations were significant and independent predictors of latex sensitization in these cases.

[Portable bladder ultrasonography: a new technic for the urological management of pediatric spinal cord damage]. / L'ecosonografia vescicale protatile: una nuova tecnica per il management urologico dei pazienti neurolesi in età pediatrica.

Noninvasive measurement of bladder volume demonstrates how a new technology can improve the management of pediatric patients with spinal cord injured. The bladder-scan was used to perform volume and post-void residual urine measurement. The aim of our study is to verify the reliability of Bladder Manager Tc 5000 in these patients.

[Severe chronic constipation: limitations of medical therapy and indications for surgical intervention. II]. / Stipsi cronica severa: limiti della terapia medica e indicazioni all'intervento chirurgico. Parte seconda.

Severe chronic constipation is a common problem in children. Most patients have functional constipation, only few (approximately 5%) have an underlying organic disease who requires surgical treatment. Anatomic problems with a painful defecation (anal rhagades, fistulas, hemorrhoids) or with difficult defecation (anal stenosis, tumor) require only a careful physical examination. Other organic problems due to neurogenic and myogenic colonic abnormalities require extensive investigations, including histological and histochemical study, for diagnosis. Ultrashort Hirschprung's disease, neuronal intestinal dysplasia type B and hypoganglionosis represent different levels of a developmental problem of the enteric nervous system. Surgical treatment is recommended in these cases. After surgery is recommended a long period of medical treatment to avoid a functional constipation.

[A flowmetric study after a Mathieu-Righini intervention in medial-distal hypospadias]. / Studio flussometrico dopo intervento di Mathieu-Righini nelle ipospadie medio-distali.

Uroflowmetric long-term data (mean follow-up 4.5 years) of 64 nonselected patients successfully treated for mid-distal hypospadias with Mathieu-Righini procedure were analyzed. None of our patients needed meatal dilation after primary procedure. Four patients (6.3%) presenting a plateau pattern and a maximum flow rate less than -2 standard deviations were considered to have meatal obstruction. These patients were successfully treated with meatoplasty. We conclude that functional characteristics of the neourethra are quite equivalent to a normal urethra in most of boys and adolescents after meatal based flap urethroplasty. On the other hand, in order to prevent the possibility of unknown strictures postoperative uroflowmetric controls are strongly recommended.

Evolution of urodynamic patterns in posterior urethral valves.

A retrospective, long-term urodynamic study was performed in order to follow the evolution of the urodynamic patterns in 55 unselected patients previously affected by posterior urethral valves (PUV). The mean maximum cystometric capacity (MCC) values progressively increased over time and, on long-term follow-up, were just above 2 standard deviations (SD). The mean compliance values were clearly reduced in the first urodynamic studies after valve ablation, and only after a mean of 5 years follow-up did they approach the lower limits of normal. The small compliance and capacity (SCC) group showed two trends of evolution: a more numerous subgroup tended toward progressive normalization while a second subgroup (<20%) showed mean compliance values below normal limits, with reduced (-2 SD) MCC persisting at long-term follow-up. The number of patients in this group decreased over time. In contrast, we were able to show a significant increase in patients with myogenic failure. In this group scheduled voiding using the Valsava maneuver in conjunction with a regimen of double or triple micturition was usually succesful in modifying the course, normalizing MCC, reducing residual urine, and also eliminating incontinence. Finally, initial urodynamic investigations in the fulguration and vesicostomy groups showed a much higher percentage of SCC bladders in the latter group (83.5% vs. 35%). However, at long-term examinations the urodynamic parameters were nearly identical in both groups, showing that temporary bladder defunctionalization does bot adversely affect future detrusor activity. No direct relationship between urodynamic abnormalities and renal insufficiency could be shown, however, the majority of patients with reduced glomerular filtration rates still showed urodynamic dysfunction at long-term follow-up. In the authors' opinion, serial urodynamic investigations in association with serial evaluation of the evolution of upper urinary tract and renal function are mandatory for correct PUV management and provide useful guidelines for avoiding incorrect treatment and obtaining better long-term results.

Evolution of upper urinary tract and renal function in patients with posterior urethral valves.

From January 1972 to June 1993, 166 patients with posterior urethral valves (PUV) were treated in our surgical department, 59 with a milder form of PUV (upper urinary tract [UUT] complication rate 29%) and 107 with a severer form (UUT complication rate 96.3%). Only the latter group was studied for long-term (mean 9.3 years) evaluation of the UUT and renal function. A temporary vesicostomy was the primary treatment in 25 patients. Indications for temporary diversion were very young age and/or low birth weight, severe and bilateral UUT complications, and severe renal damage. All the other patients were treated by primary endoscopic valve fulguration. After removal of the lower urinary tract obstruction, vesicorenal reflux (VRR) resolved spontaneously or was ameliorated in 59.2% of the renal units. Spontaneous normalization or evident amelioration were found at long-term follow-up in nearly 70% of dilated, non-refluxing ureters. Ureteral reimplantation was performed on 41 of the 202 dilated or refluxing ureters (surgical rate 20.3%). The surgical failure rate requiring reoperation was 5% (2/41). The prerequisite for successful reimplantation was a large-capacity, stable, and compliant bladder. Ten nephroureterectomies were carried out for unilateral, massive VRR and renal dysplasia; 1 late nephrectomy was performed for arterial hypertension. The evolution of renal function showed statistically significant overall improvement, which was more evident in patients diagnosed and treated in the 1st month of life (P = 0.000) than in those treated between 1 and 12 months (P = 0.004) or after 1 year of age (P = 0.025). Renal function considerably improved in the vesicostomy group (P = 0.000). Thirteen patients (12.4%) are now either dead (2) or have end-stage renal disease (6) or chronic renal insufficiency evolving toward end-stage renal disease (5); 5 of these 13 were treated by vesicostomy in the first days or months of life, and at presentation the glomerular filtration rate (GFR) was less than 25 ml/min . 1.73 m(2). Determination of basic GFR and, even more, functional renal reserve is relevant in predicting the long-term evolution. In the author's opinion, vesicostomy is the procedure of choice in very ill newborns or infants. Aggressive management with early surgical reconstruction is rarely justified, because frequently UUT complications resolve spontaneously or clearly improve, and their surgical treatment has limited and very precise indications.

Neuropathic urinary incontinence in pediatric patients: management with artificial sphincter.

From June 1982 to November 1990, 37 patients, aged 13 to 19 years (35 males and 2 females), were treated in our division for neuropathic urinary incontinence with an artificial sphincter. All patients were treated before sphincter implantation with drugs, transurethral sphincterotomy in boys, and bladder flap urethroplasty was carried out in females during the surgical procedure. The cuff was placed at the bladder neck in 33 cases, and in 4 cases at the urethral bulb. The sphincter was activated about 3 weeks after implantation and after 2 months in two patients with associated bladder augmentation. Operative and perioperative complications occurred in four cases during the initial phase of our experience. Later we had to perform 19 revisions on 14 patients due to mechanical and surgical failure (reoperation rate, 0.38). Thirty-three patients are presently dry (90% of successes). Postoperatively, normal upper urinary tract conditions were found in all patients except two.

[Renovascular hypertension in childhood]. / Ipertensione reno-vascolare in età pediatrica.

One to two per cent of children and up to 11% of adolescent have arterial hypertension. In most cases children and adolescent are not recognized to be hypertensive because physicians do not routinely measure blood pressure. Often the diagnosis is recognized only when the pediatric patients develop a complication: seizure, stroke, heart failure or paraplegia. Renovascular hypertension in children and adolescents is more common than all of the other causes combined, except for coarctation of the aorta. The diagnosis is not so easy and includes the usual history, physical examination (signs and symptoms of coarctation of the isthmic or abdominal aorta or of an abdominal mass or of one of the adrenal causes of hypertension), laboratory studies, abdominal ultrasound study and chest x-ray. Sometime a CAT can be usefull. The next steps are the early and rapid-sequence IVP, renal angiography and peripheral and renal renin activity. The management of renovascular hypertension in children and adolescent includes a conservative approach (percutaneous transluminal renal angioplasty or renal embolization), rarely used in pediatric age, and the surgical treatment. This latter includes all the surgical procedures of renal revascularization and, in unilateral renal parenchymal diseases, the nephrectomy or a partial nephrectomy. The postoperative results are very good in a high percentage of cases. In bilateral cases, the revascularization surgical procedures improve or normalize also the impaired renal function.

[Primary intestinal lymphangiectasis. A case treated surgically]. / Linfangectasia intestinale primitiva (L.I.P.). Un caso di trattamento chirurgico.

The authors describe a rare case of primary intestinal lymphangiectasis resolved with surgical treatment. Usually the natural course of the disease is relatively mild and medical nutritional treatment can be sufficient. In this case the lymphatic intestinal anomaly was generalized to the entire small intestine but a distal ileal segment was particularly involved. The surgical resection of this intestinal tract resolved the symptomatology.

[Ectopic ureter]. / L'uretere ectopico.

Ectopic ureter is quite a rare malformation, and it can be more or less serious depending on its anatomy (monolateral or bilateral ectopy, duplicated ureter, single ureter) and the associated malformations. It's more common in female patients. Ectopic ureteral orifice can be either intravesical (bladder neck) or, more often, extravesical. Ectopia in women is more frequently found in the urethra and the vaginal vestibule; much less frequently in vagina and in uterus. In men it is mostly found in the posterior urethra; in the male genital tract is very rare. In most cases ectopic ureter is associated with pyelo-ureteral complete duplication: the upper kidney usually works badly or doesn't work at all, due to renal dysplasia or pyelonephritis. More rarely ureteral ectopia affects a single urinary system and can be monolateral or, in the most serious forms, bilateral. The main clinical signs are urinary incontinence accompanied by regular micturition (more exactly pseudoincontinence) in the female patients, and urinary infection. In the male there is no urinary incontinence. Authors report their experience about 54 ectopic ureters in 51 children. Treatment is always by surgery: the choice has to be made between conservative or radical attitude. 38 total exeresis of the ectopic excretory pathway (upper heminephroureterectomy or nephroureterectomy), 9 ureteral reimplantations, 1 uretero-pyeloanastomosis have been done. Outcome is always satisfactory in monolateral forms, more uncertain in bilateral forms with single ectopic ureter, due to incontinence and reduced bladder capacity problems and the possible associated renal failure.

[Retroperitoneal lymphangioma. Radiologic aspects and diagnostic protocol]. / Linfangioma retroperitoneale. Aspetti radiologici e protocollo diagnostico.

The authors analyze the radiological aspects and the staging problems of the retroperitoneal lymphangioma, a rare neoplasma of infancy. Echography and computer-assisted tomography contributions are particularly emphasized.

[Dilatation of esophageal stenosis using a balloon catheter]. / Dilatazione delle stenosi esofagee con sonda a palloncino.

Treatment of esophageal strictures is discussed. Authors describe technique and results of transluminal balloon dilatation in nine cases of esophageal stenosis.

[Surgery of malformations of extrahepatic bile ducts in childhood]. / La chirurgia delle malformazioni delle vie biliari extraepatiche in età pediatrica.

The actual hypothesis on the etiology and pathogenesis of neonatal hepatitis, intrahepatic and extrahepatic biliary atresia and choledocal cyst is that these disorders can be different results or permissible outcomes of a single basic process: infantile obstructive cholangiopathy. This hypothesis can explain the failure of many infants with operable extrahepatic biliary atresia to do well following surgically successful anastomosis. Very possibly no surgical mode of therapy will cure a significant fraction of infants with biliary atresia (correctable and non-correctable types) because the basic disease process actually produces portal fibrosis and destroys intrahepatic bile ducts as well. However, since the obliterative process can resolve, even if not usually completely, surgical procedures of conventional or of hepatic-portoenterostomy type should be considered for all infants who are found to have biliary atresia. Probably biliary atresia is more in need of preventive or prophylactic measures than of new surgical procedures.

[Description of a case of thanatophoric dysplasia with cloverleaf skull]. / Descrizione di un caso di displasia tanatofora con cranio a trifoglio.

This report presents the clinical, radiographic, and autopsy findings in a newborn infant with thanatophoric dysplasia and cloverleaf skull who died soon after birth. The possible genetic mechanisms of transmission of both malformations are discussed.

[Care of the newborn infant with respiratory distress syndrome using continuous negative pressure. 5 years' experience]. / Assistenza al neonato con sindrome di distress respiratorio (RDS) mediante la pressione negativa continua (CNP). 5 anni di esperienza.

Continuous Negative Pressure (CNP) was utilized as sole form of respiratory assistance in the treatment of 40 newborn infants with Respiratory Distress Syndrome (RDS). The average duration of CNP therapy was less than 7 days in all cases. 38 of these infants survived without sequelae (95%); an infant developed severe bronchopulmonary dysplasia (2.5%); another infant died from intracranial hemorrhage (2.5%). CNP appears to be an effective respiratory support in the treatment of neonatal RDS, when used early in spontaneously breathing infants weighing greater than 1500 grams.