Nasal polyposis in children.

Nasal polyposis is a chronic inflammatory disease affecting the nasal cavity and the paranasal sinuses. It is a relatively common disease, occurring in 1-4 % of the general population, but it is rarely described in the pediatric population. Most of the published series include children presenting with other underlying systemic diseases, mainly cystic fibrosis. The aim of the present study was to describe the characteristics of the patients suffering from nasal polyposis, evaluated at the Pediatric Clinic of the University of Pavia (Italy) over the last 17 years. 56 consecutive pediatric patients referring to our Pediatric Department had a diagnosis of nasal poyposis over the last 17 years. All children underwent allergy evaluation, nasal endoscopy, CT scan of the paranasal sinus, and Functional Endoscopic Sinus Surgery. The mean age of the present cohort was of 11.8 years and most of the patients were male. 50% of the patients presented with unilateral, polyposis, mostly with a diagnosis of antrochoanal polyp. 4 patients presenting with bilateral polyposis suffered from cystic fibrosis. Main symptoms at diagnosis included nasal obstruction, snoring and rhinorrhea 32% of the patients presented at least a positivity to skin prick test, for major inhalant and food allergens. Nasal polyposis in children could represent an alert sign for other underlying systemic diseases. Nasal endoscopy should therefore be prescribed when a diagnosis is suspected. To properly treat a patient presenting with nasal polyposis, it is necessary to integrate medical and surgical skills through a multidisciplinary approach.

Accurate placement of central venous catheters in pediatric patients using endocavitary electrocardiography: reassessment of a personal technique.

To avoid the need for radiological control in the assessment of the proper location of central venous catheters (CVC), a particular use of endocavitary electrocardiography (EC-ECG) was proposed 10 years ago. The aim of this study is to reassess our experience with this method. EC-ECG assumes that the CVC, when filled with normosaline and connected to a standard electrocardiograph, behaves like an exploring electrode. The approach of the catheter tip to the right atrium is then detected by a slightly increasing negative P wave. When the tip reaches the exact level of the sinus node, the P wave suddenly deepens. After a preliminary test of the reliability of the technique versus the standard method in 50 CVC placements verified by both EC-ECG and chest x-ray, we have placed 807 CVCs in children using EC-ECG only. There have been no false-positive and one false-negative test result (lead connector misplacement). In 17 cases in which intrinsic deflection was not detected, the catheter tip was found to be wrongly positioned; all the remainder CVCs have been successfully positioned. For 10 years this technique has proved to be a simple, safe, quick, inexpensive and highly reliable method to assess the correct positioning of the CVC.

Conservative surgery of bladder-prostate rhabdomyosarcoma in children: results after long-term follow-up.

Between 1968 and 1982, 10 children (7 boys and 3 girls) with a mean age of 3.2 years affected by bladder (8 cases) and prostate (2 cases) rhabdomyosarcoma (RMS)--all histologically embryonal--were seen. Three (1 with bone metastases at diagnosis and 2 with stage III tumor) underwent radical surgery; all died because of progression of the disease. Seven children were treated with conservative surgery: 3 solid type bladder tumors were removed together with a narrow strip of apparently healthy bladder wall, sparing 30% to 70% of the bladder; 3 botryoid type bladder tumors were removed bluntly and implantation sites fulgurated; in 1 prostate tumor osmotic cytolysis of the neoplastic mass was achieved with multiple injections of hypertonic saline solution (NaCl 30%). Surgery was followed by chemotherapy with a vincristine actinomycin D and cyclophosphamide protocol and radiotherapy (20 to 39.5 Gy). Six of the seven are alive and disease free with a normal functional bladder after 10 to 21 years. The validity of a primary conservative surgical approach with preservation of the bladder, followed by adjuvant therapy, is emphasized.

Long-term follow-up in children treated for retroperitoneal malignant tumours.

Thirty-two long-term survivors after treatment with surgery, radiotherapy and chemotherapy for neuroblastoma (NRB) and Wilms' tumour (WT) were re-evaluated after a period of more than 10 years (mean 16 yr 1 mo, range 27 yr 2 mo/10 yr 5 mo). In addition to routine clinical and laboratory studies, all patients underwent auxometry, echocardiography, spinal X-ray, abdominal US and kidney radionuclide scans. Neither obvious physical signs nor cutaneous toxicity were noted at inspection. No dysmenorrhoea or sexual disorders were mentioned. Haematological data turned out to be all in the normal range, except for TIBC/UIBC ratio (out of range in 70 % of cases); gammaGt (62.5 %); A/G ratio (12.5 %); ALT (37.5 %). Auxometrical data were all in the normal range; in particular, results of mean weight and height were just slightly over the 50 degrees centile. Spinal X-rays revealed mild kyphotic deformities (68.9 %), pelvic obliquity and tilting (62.5 %), rotation of the vertebral bodies (34.3 %) and compensatory curves of the spine (21.8 %). No impairment of cardiac functions was noted at echocardiography. No alteration of the hepatic structure was detectable at US scans. Marked impairment of the renal function (20 % and 1.5 % residual function) was detected at radionuclide imaging in 2 NRB patients who had not undergone nephrectomy, with the kidney having almost disappeared in one. In conclusion, the study emphasises the need for careful and prolonged follow-up for the detection of sequelae than can appear even many years after the initial treatment, and the wisdom and the need for a continued search for less aggressive protocols; as long as the cure rate is not compromised, less aggressive protocols should aim for a satisfactory overall survival with a reduced incidence of sequelae and hence a better quality of life.

Is bilateral congenital anorchia genetically determined?

Bilateral congenital anorchia (BCA) can be defined as complete absence of testicular tissue in a patient with male normal phenotype and karyotype. On the basis of familial occurrences of BCA a possible genetic aetiology has been hypothesised, i.e. mutations of the SRY gene which initiates the genetic cascade leading to testis development in mammals. The aim of the study is to assess this hypothesis. Eight boys affected by BCA have been studied; a normal monozygotic twin of one of the patients, a boy and a girl acted as controls. A normal 46, XY karyotype was detected in all patients; 3 had hypoplasia of the scrotum and 2 of the penis. Hormonal data were available for 5 patients: Prader's stimulation test to HCG showed in all lack of testosterone response, and 4 out of 5 had elevated FSH and LH levels. Complete absence of testicular tissue was confirmed in all by surgical exploration. DNA was sampled by Jeanpierre modified extraction method and amplification by polymerase chain reaction. The expected segment of 750 basepairs of the SRY gene, included between the two oligonucleotide primers Xes 10 and Xes 11, was found in all patients. SRY gene is present in our BCA patients as well as in normal boys, and therefore BCA does not seem related to an anomaly of the opening reading frame sequence of the SRY gene. Nevertheless, familial occurrences of BCA continue to suggest a genetic aetiology: further studies must therefore evaluate the possibility of punctiform mutations of the SRY gene, by direct sequentiation, and exclude abnormalities in the critical region DSS/AHC of the X chromosome, recently discovered as one of the loci involved in the differentiation of the male gonad.

Surgical treatment of necrotizing enterocolitis: when? how?

In 10 years (1981-1990) 28 out of 54 neonates (51.8%) with definite necrotizing enterocolitis (NEC) underwent surgery. Operation was performed at 13.5 +/- 8.8 (range 3-38) days of life, after 1.7 +/- 1.5 (range 1-6) days from the onset of symptoms. Aiming to perform laparotomy before the occurrence of perforation, surgery was liberally indicated in stage IIIa, according to Walsh-Kliegman. Explorative laparotomy (+peritoneal drainage in 2 cases) was performed in 4 patients with massive intestinal necrosis: all died within 3 days of surgery. In one neonate, only pneumatosis was present and resection was not considered mandatory. Intestinal resection and enterostomy was performed in 17 neonates, 5 of them with perforation; three developed an intestinal stenosis. Enterostomy was closed after 116.2 +/- 61.8 days (range 26-193); 11 patients (64.7%) are long-term survivors. Intestinal resection and primary anastomosis was performed in 6 babies, 3 of them with perforation. Postoperatively, 2 dehiscences and 1 stenosis were recorded, but all children survived. In our opinion, resection followed by primary anastomosis seems to be the most satisfactory surgical option.

[Medical treatment of cryptorchism. "Vanitas vanitatum et omnia vanitas"]. / La terapia medica del criptorchidismo. "Vanitas vanitatum et omnia vanitas...".

Aiming to study the effectiveness of medical therapy in cryptorchidism, a computerised Medline research from 1985 to 1999 on the thesaurus word "cryptorchidism" with the filter "drug therapy" was conducted. Of the 147 papers thus retrieved, only those dealing with more than 50 patients were considered. Factors studied were: ultimate aims of therapy, different drugs and/or associations used, suggested age of treatment, dosages and cycles, anatomical position of the testis, mono- or bilaterality of cryptorchidism. For every considered factor results heavily conflicting were found. According to the various Authors, medical treatment has different goals: not only to induce testicular descent but also to improve testicular trophism, to help in diagnosis and/or in surgical treatment, to increase postsurgical fertility, to reduce neoplastic risks, to reduce the psychological stress. About relative effectiveness of drugs, hCG seems to be more effective than LH-RH in most papers; the latter resulted slightly more effective than placebo (from 37% vs 18% to 9% vs 8%). Suggested age for treatment goes from less than 6 months to more than 6 years. In relation to the position, non palpable testes resulted almost uniformly not responding to the therapy, while testes in prescrotal position responded from 17% to 100%. According to the mono- or bilaterality of the lesion, results vary respectively from 58% vs 50% to 14% vs 64%. Relapse rate as well shows a variability from 10% to 63%; effectiveness of a second treatment in such cases goes from 0% to 100%. Drawing definite conclusions from this analysis turned out to be impossible because of the steady high variability in published results, leaving at the end of the day an unappealing sensation of "vanitas" (vacuity).

[Destiny of prepuce between Quran and DRG]. / Il destino del prepuzio fra Corano e DRG.

Ritual male circumcision is one of the oldest operations ever performed (Egyptian mommies of the III millennium b.C. have been found circumcised), which has been practiced for thousands of years by Moslems, Jews and various tribes in Africa, America, and Australia; today some 25% of the total world male population is circumcised. Steadily increasing number of Moslem immigrants raised also in our country this issue, up to now completely unknown to Italian culture. In the year 2000 in Italy 21.735 live births from immigrant parents, > 50% of Islamic faith, were recorded: it is therefore easily foreseeable that pediatricians and pediatric surgeons will be increasingly requested to perform this procedure, on the other hand still forbidden by Italian laws and N.H.S. regulations. This paper deals with some anthropological, religious, psychological and legal-economical aspects of ritual male circumcision, in order to offer data for a deeper insight of this complex issue.

Partial nephrectomy for Wilms tumor.

We report a case of a left Wilms tumor that was treated successfully by partial nephrectomy. The advantages and disadvantages of this approach are discussed.

Turcot's syndrome with intestinal lymphoma in a child: an unusual case of triple tumor.

Turcot's syndrome, the association of brain tumor (usually glioblastoma, medullo-blastoma, or astrocytoma) and colonic polyps, is a very rare condition of which about 20 cases have been reported. It has been described only once previously with cancer in a third organ system. In this paper, we report a child affected with colonic polyposis and astrocytoma (i.e., Turcot's syndrome) associated with intestinal non-Hodgkin's lymphoma.

Fortuitous diagnosis of cystic fibrosis at laparotomy for acute appendicitis.

Two children aged 11 and 9 years were operated upon for acute appendicitis and were discovered to have cystic fibrosis: the histologic pattern of the removed appendix was typical for this disorder. Appendicitis may thus be the first presenting sign of occult cystic fibrosis.

[Intestinal atresia: what is changed in the last 30 years]. / L'atresia dell'intestino: cosa è cambiato negli ultimi 30 anni.

The changes occurred in the management of intestinal atresia in the last 30 years are presented. On the basis of a series of 55 neonates (29 M/26 F; 14 premature; mean body weight 2640 g, range 1340-4100 g; 23 duodenal, 30 small bowel, 2 colon) four aspects of this malformation are discussed; epidemiology, diagnostics, therapy and prognosis. Epidemiology. 40% incidence reduction is detected; unchanged the relative incidence of Down neonates with duodenal atresia. Diagnostics. Prenatal US scan useful in 4 of 8 cases only; emphasized the usefulness of plain X-ray abdomen. Perioperative treatment. Dramatic impact of the artificial nutrition on the management of short bowel syndrome, with 7 cases (residual small bowel from 75 to 9 cm, mean 40 cm) successfully treated. Surgical technique. Confirmed the validity of the one-stage termino-terminal anastomosis. Prognosis. Significant improvement in the last three decades respectively with 50%, 87% and 93% of long-term survivors. In the first decade 6 over 8 deaths were related to the atresia, while in the last 10 years the only death recorded was related to an associated severe cardiac malformation.

Laparoscopic antireflux surgery in neurologically impaired children.

From May 1996 to April 2002, 48 laparoscopic fundoplications were performed after failure of medical treatment in 47 neurologically impaired infants and children affected by gastroesophageal reflux. Indications for surgery included vomiting, recurrent upper airway infections, failure of medical therapy, feeding difficulties with failure to gain weight, and instrumental (barium swallow and pHmetry) diagnosis of gastroesophageal reflux. A standard approach was adopted, with minimal access modifications according to the patients' characteristics. In two patients, laparoscopic surgery had to be converted to open surgery because of severe kyphoscoliosis and accidental left emidiaphragm perforation. In another patient undergoing a laparoscopic Nissen fundoplication, a re-do laparoscopic operation was performed. Postoperative analgesia was administered during the first 12 h, and fluid intake and feeding were begun on days 1 and 2, respectively. All patients clinically improved except two; a paraesophageal hernia developed in one, and a stenosis developed in the other. We strongly believe that laparoscopic fundoplication can be successfully adopted in neurologically impaired children as well as in pediatric patients as a whole, with the same advantages and far fewer drawbacks than are expected in adults.