Liver replacement with reduced size donor organs.

End stage liver disease in children can be treated with orthotopic liver transplantation (OLT). Nevertheless, the expansion of this therapy in Europe has been limited because of the shortage of appropriate size-matched donors. One possible technical solution is the OLT of a liver graft previously reduced in size by in situ resection preceding the harvesting procedure. To study the impact of this technique we examined two different operative procedures performed on Landrace pigs. Group 1 consisted of 20 standard donor/recipient weight matched OLT. In group 2, 15 OLT were performed using right lateral and medial lobes (55% of the original donor liver). The donor/recipient weight ratio in the group was 2:1. Cold ischemia times were 90 +/- 16 min for group 1 and 98 +/- 9 min for group 2. It is emphasized that in contrast to all the other reports using resected liver grafts for OLT, the donor resection in our study was always performed in situ under normothermic conditions, preceding the harvesting procedure. This was designed to reduce the cold ischemia time. No significant technical problems were encountered. The biochemical results of group 2 pigs compared to group 1 demonstrate an analogous, postoperative course. This might be explained by regenerative stimuli acting on the resected liver tissues and enhancing their metabolic function. These data support the conclusion that resected adult donor liver grafts may be used for pediatric transplant recipients.

Prenatal diagnosis and management of some fetal intrathoracic abnormalities.

Nine cases of fetal intrathoracic anomalies detected in utero and followed to birth are reviewed. There were 6 congenital diaphragmatic hernias (CDH), one congenital pleural effusion and two isolated cysts of the lung. All these conditions were potentially responsible for neonatal respiratory distress and received early intensive treatment after maternal transport and delivery had been arranged in a center with thoracic surgical facilities available. The risks of a delayed or missed diagnosis were thus avoided, especially for CDH. Despite intensive, traditional, respiratory support, started in the delivery room, mortality among prenatally detected cases of CDH was paradoxically high (83%), compared to mortality among 7 cases of CDH not detected in utero, referred in the same period to our Institution, and symptomatic within 6 h from birth (63%). With prenatal diagnosis the total number of CDH cases referred to a surgical center before birth increases. Many cases which would never have been treated in the past because of death before referral and treatment for severe pulmonary hypoplasia not compatible with life are thus observed and sometimes treated. Nevertheless, lung development continues to be a determining factor for survival even when intensive treatment at birth is available. Responsiveness to therapy is unpredictable before birth and proposed antenatal treatment is still far from being a realistic option. For the other three newborns, where a pleural effusion and pulmonary cysts were found, prenatal diagnosis helped to start appropriate treatment and to prevent neonatal hypoxia in two of them. In the third case, with an incommunicant, isolated pulmonary cyst, the outcome would have been favourable even without a prenatal diagnosis.

Endoscopic treatment of chronic idiopathic pancreatitis in children.

The purpose of this study was to analyze the preliminary results obtained with endoscopic treatment of children with chronic idiopathic pancreatitis. The disease appears to be caused by a deficit of pancreatic stabilizing proteins that leads to precipitation of solutes contained in the pancreatic secretions; these precipitates are the cause of inflammation. The possible role of a congenital malformation in the pathogenesis of this disease is being regarded with increasing scepticism. Between October 1991 and April 1994, five cases of chronic pancreatitis were referred to the Division of Pediatric Surgery of the Policlinico "A. Gemelli" of Rome. The age range of the patients (3 boys, 2 girls) was 3 to 14 years (mean, 10.8 years); all had a history of acute attacks and had blood chemistry findings compatible with pancreatitis. None of the patients' families had a history of pancreatic disease. All diagnoses were confirmed by endoscopic retrograde cholangiopancreatography, and the disease was staged according to the criteria of Cremer et al. Endoscopic pancreatic sphincterotomy, with or without removal of calculi, was performed in four cases (2 in the authors' hospital, 2 in another institution). Three of the children have had no further symptoms (mean follow-up period, 20 months), and their growth and weight gain have been normal. The fourth child, a 3-year-old girl, has had two episodes of pain since treatment. These attacks, both of which subsided spontaneously, probably were caused by the passage of protein plugs through the sphincterotomy. The fifth patient received no treatment because she had remained asymptomatic since the first examination (28 months ago). The short-term effects observed in these children indicate that endoscopic treatment of chronic idiopathic pancreatitis is associated with a high rate of success with respect to pain control. Because of the short follow-up, the effects on endocrine and exocrine function have not been assessed. No deaths or complications occurred. Conservative treatment of this type can be repeated if pain recurs, an important factor given the chronic nature and early onset of this disease, and it does not preclude the possibility of subsequent surgery. The authors' experience indicates that this approach should be considered the initial treatment of choice for children with chronic pancreatitis.

Treatment of intraosseous arteriovenous fistulas of the extremities.

Intraosseous arteriovenous fistulas of the extremities are rare malformations frequently associated with severe systemic hemodynamic alterations. In many cases, it is quite difficult to eliminate these anomalous vascular structures, but the possibilities for successful treatment are much greater when surgery is combined with interventional radiology. Selective embolization of the malformed vessels can be produced with a variety of agents that are injected into afferent arteries, via percutaneous puncture or through direct surgical access. The intraosseous portion of the fistula should be resected at the time of embolization or later. The authors describe the successful treatment of three patients having intraosseous arteriovenous fistulas of the upper extremities, who have had follow-up for 2 to 10 years.

Recurrent peptic stenosis of the esophagus: treatment with a self-expanding metallic stent.

A 12-year-old neurologically impaired boy with recurrent peptic stenosis of the esophagus was treated successfully with use of a self-expanding metallic stent that remained for 3 months.

Accessory lower limb in a newborn with multiple malformations.

Accessory lower limbs are rare anomalies that are associated with other severe malformations. The authors describe a case of this type and discuss the pathogenetic and classification problems surrounding these malformations in the light of the limited number of the other cases reported in the literature.

Thyroid tumors in children and adolescents: preoperative study.

Several studies indicate that in young patients (less than 21 years of age at the time of diagnosis), the prognosis of thyroid carcinoma (TC) is more favorable than in older patients. However, a more radical treatment approach is recommended in children and adolescents due to the higher prevalence of local lymph-node involvement in these cases. Since the extent of primary surgical treatment is closely related to the overall prognosis, preoperative diagnosis becomes essential in the management of thyroid neoplasms in young patients. In this retrospective study (1987-1998), we analyzed a surgical series of 50 children and adolescents with thyroid nodules in an attempt to establish the role of diagnostic studies in detecting malignant lesions prior to surgery. Our diagnostic protocol for evaluating thyroid nodules was based on clinical evaluation, measurement of thyroid-hormone and thyroglobulin (TG) levels, anti-TG and anti-TPO antibody titers, calcitonin, CEA, and TPA levels, sonography, scintigraphy, and fine-needle aspiration cytology (FNAC) of the thyroid nodules and any enlarged lymph nodes. Eleven of the 15 cases of histologically confirmed carcinoma were preoperatively identified as malignant lesions with the aid of FNAC. The authors conclude that the preoperative work-up of children and adolescents with thyroid nodules requires the collaboration of an experienced team of professionals, and recommend FNAC as the initial test.

H-type rectovaginal fistula associated with the Currarino triad.

We describe a case of H-type rectovaginal fistula associated with the Currarino triad (anorectal stenosis, sacral defect, presacral mass). Presenting symptoms included passage of feces per vaginam, signs of intestinal subocclusion without perianal inflammation, left leg paresis and foul-smelling urine. An anterior sacral meningocele was repaired at the age of three months. At age 18 months the fistula was excised through a perineal approach after creation of a protective colostomy. Diagnostic and therapeutic aspects of this malformation are discussed.

[A giant lymphangioma of the trunk with a fatal outcome]. / Linfangioma permagno del tronco ad esito infausto.

The authors describe the case of a 2-month-old male brought to the emergency room in a preagonal state cause by extensive septic necrosis of an enormous lymphangioma located on the left lateral trunk. In spite of emergency treatment the child expired shortly after admission. Our observation of this case prompted a review of the literature on the treatment approaches currently used for these tumors. Emphasis is placed on the necessity for early treatment in order to avoid infectious complications that, if neglected, can lead to unmanageable and possibly fatal sepsis.

[Abdominal masses in pediatric age; clinical aspects and diagnostic approach in 52 cases (author's transl)]. / Masse addominali in età pediatrica. Aspetti clinici e orientamento diagnostico in 52 casi.

A large series of malignant and benign conditions are generally collected under the term of abdominal masses. Their common aspect is the lack, in most of the cases, of peculiar clinical features which may help early differential diagnosis. In many cases the mass is detected late after a long period of vague, aspecific symptoms. 40% of these space occupying lesions of the abdomen are of malignant origin and delayed detection and investigation affect clinical course. Preoperative study of abdominal masses is a problem of primary importance in pediatric surgical practice. A changing attitude is registered towards many diagnostic procedures and the role of largely diffused techniques like angiography is controversial. The introduction of ultrasonography makes in many cases intensive radiologic investigation unwarranted and academic. The Authors discuss the real role and targets of preoperative investigations of abdominal masses and refer on their experience based on 52 cases, to underline some clinical aspects and analyse their diagnostic approach to this pathology.

[Congenital esophageal obstruction caused by complete mucous membrane: a clinical case]. / Obstrucción esofágica congénita debida a diafragma mucoso completo: caso clínico.

The authors report a case of membranous atresia of the esophagus. Diagnosis of this rare malformation was made intraoperatively, and resection and primary anastomosis were performed immediately. A brief review of the literature is included on the various types of esophageal atresia.

[Bilateral distal ureteral obstruction: unusual complication of appendicular abscess]. / Obstrucción ureteral distal bilateral: una rara complicación del absceso apendicular.

We describe the case of a 6-year-old boy who presented post-renal anuria and renal failure five days after appendectomy and drainage of a periappendicular abscess. Only mild dilatation of the urinary tract was observed on ultrasound and small calculi were documented at the ureterovesical junction bilaterally. Diuresis was restored by the insertion of uretercatheters. Awareness of this complication and immediate treatment can avoid permanent impairment of renal function.

[Cryptorchidism: a study of histological changes in undescended testis related to age]. / Criptorchidismo: studio delle alterazioni istologiche nei testicoli ritenuti in rapporto all'età.

A total of 46 testicular biopsies were examined to define the histological characteristics of undescended testicle. Nine of the biopsied organs were normally positioned testicles from deceased pediatric patients (aged 2 months-14 years), and the remaining 37 were undescended testes from children with various degrees of cryptorchidism (aged 18 months-14 years). As reported by others, the degree of histological damage was directly proportional to the age of the subject. Close correlation between histological findings and the level of descent was not observed.

[Natural history of fetal uropathies. Experience in 40 cases]. / L'evoluzione naturale delle uropatie fetali. Esperienza su 40 casi.

Appropriate management of fetal uropathies depends on accuracy in assessing the severity of urinary tract obstruction and renal damage, and in predicting the potential for recovery after surgical correction. A review of 40 fetuses aged between 20 an 39 weeks referred to us for prenatal counseling for a suspected anomaly of the urinary tract, has been made. Reliability of our diagnostic resources has been retrospectively evaluated on the basis of clinical, ultrasonographic and radiological postnatal data. Conservative approach has been adopted in all our cases. None intrauterine surgery was attempted; pre term delivery was advised only in two cases. Our results confirm poor prognosis of fetuses with marked decrease of amniotic fluid within the 24th week of gestational age. After this date the amount of amniotic fluid alone may not be a completely reliable prognostic factor. Ultrasonographic aspect of fetal kidneys and, in some occasions, chemical analysis of fetal urines may accomplish our prenatal study and help to predict the degree of neonatal renal function and potential of survival.

[Radioisotope study of urinary deformity pathology in childhood]. / Lo studio radioisotopico della patologia malformativa urinaria in età pediatrica.

The role of radionuclide investigation of urinary tract pathology in pediatric patients (computerized radionuclide urography and radionuclide cystogram) is discussed. Advantages of reliable morphological and functional study with a non invasive, low radiation procedure are emphasized.

[Echography in the study of renal pathology in childhood]. / L'ecografia nello studio della patologia renale in età pediatrica.

Results of US study in 30 children with various renal lesions are reported, and compared with clinical and surgical features. Different ultrasonographic aspects are discussed with special interest on cogenital abnormalities. US is proposed as first choice investigation in newborns with antenatal ultrasonographic demonstration of renal lesions and in all patients where a mass of renal origin is suspected. Renal function, nevertheless, must be investigated with radiologic and/or radioisotopic techniques. US have still a large indication in short term follow-up of renal lesions where surgical treatment is not indicated.

[Dumbbell neurogenic tumors in children]. / Tumori neurogenici a clessidra in età pediatrica.

Dumbbell neurogenic tumours are unusual neoplasms in the pediatric age group. Six cases all in children under 10 years of age, have been reviewed with respect to diagnosis, management and results. Neurologic deficits have been found in 4 patients only; in the other 2 cases the tumours were occasionally discovered. Myelograms, sometimes associated with CT scan, allowed a correct diagnosis in all the patients. Initial treatment consisted in all the cases in laminectomy or laminotomy with total excision of the extradural tumour. The primary paravertebral tumours have been removed in one patient during the same neurosurgical operation, and in other three patients at a second stage. Two patients did not require the second operation: one recovered after chemotherapy and the other died few days after the first surgical procedure. Five patients are still alive: two, who had the tumour occasionally discovered, are free from disease; among the other 3 patients, presenting with neurological symptoms, 1 is free from disease without neurological symptoms and 2 have still long lasting sequelae. The authors emphasize the role of early diagnosis to prevent neurologic abnormalities and to achieve better results.

Bilateral renal vein thrombosis as a complication of gangrenous appendicitis.

Bilateral renal vein thrombosis after an appendectomy has never been reported in the pediatric literature. We describe the case of a 10-year-old boy who developed this very unusual complication following appendectomy for gangrenous appendicitis with peritonitis. Color duplex Doppler is the most appropriate investigation to allow correct diagnosis and immediate medical treatment. Peritoneal dialysis is a simple and effective tool to prevent permanent damage to renal function.

[One-stage "forced" closure of large congenital defects of the abdominal wall with mechanical ventilation and curarization. Clinical evaluation]. / Chiusura "forzata" in tempo unico dei difetti congeniti maggiori della parete addominale con ventilazione meccanica e curarizzazione. Valutazione clinica.

Advantages of primary fascial closure of abdominal wall defects are mainly in reducing the number of staged procedures with related complications and the need of multiple operation. Nevertheless correction of large defects still remains a challenge to pediatric surgeon. Postoperative paralysis and mechanical ventilation after intraoperative milking of intestinal content and abdominal muscles stretching have been reported to reduce the risks of "forced" primary closure. A series of 64 Omphalocele and Gastroschisis has been reviewed. Associated anomalies are still the main cause of mortality among Omphalocele. Prematurity plays a secondary role on survival of Gastroschisis cases; deaths were mainly due to sepsis. Primary respiratory insufficiency affected a large number of Giant Omphalocele cases (larger than 5 cm with herniated liver) and was associated to a restricted chest structure. All these cases died in the first weeks of life. Primary closure with or without postoperative paralysis and mechanical ventilation showed to reduce in a significant way the postoperative complication rate compared to staged procedures. Mortality and hospital stay were not significantly influenced by different kinds of surgical treatment among Omphalocele. Associated anomalies are an unavoidable limiting factor to survival. Among Giant Omphalocele the use of aggressive primary fascial closure with ventilatory support showed in our hands to be a safe procedure provided that a preoperative selection of cases on the basis of chest X-ray and blood gases has been made.