MPFL reconstruction and tibial tuberosity transposition in patients with patellar instability: May it troubleshots also trochlear dysplasia?

PURPOSE: This study aimed to highlight short- and medium-term outcomes of combined medial patello-femoral ligament (MPFL) reconstruction and anterior tibial tuberosity (ATT) transposition surgery in patients with recurrent patellar instability and different degrees of trochlear dysplasia. METHODS: Between January 2014 and May 2019, 25 patients with patellar instability underwent a surgical procedure combining the lowering/transposition of the ATT and the MPFL reconstruction. Each patient were preoperative assessed by Kujala score, International Knee Documentation Committee (IKDC), Tegner activity level scale. The assessment of instability predisposing factors was carried out with patellar height, tibial tuberosity-trochlear groove (TT-TG) distance, trochlear dysplasia, sulcus angle, patellar tilt and MPFL injuries. Functional outcomes were evaluated with Kujala, IKDC and Tegner scores at 3, 6 and 12 months after surgery. RESULTS: The average age of the patients was 20 years (range 13-43 years). Pre- operative Caton-Deschamps index was pathological in 10 (40%). Sulcus angle was elevated in 13 patients (52%) and TT-TG distance was irregular in 17 patients (68%). Trochlear dysplasia was present in 13 patients (9 type A, 3 type B, 1 type C according to Dejour's Classification). No re-dislocation occurred during the follow-up. There was a significant increase in the Kujala, IKDC and Lysholm scores after 3, 6 and 12 months, and the results were compared for the different follow-up times and patient's trochlear dysplasia degree. CONCLUSION: This prospective observational longitudinal study identified good clinical outcomes in patients who underwent MPFL reconstruction and ATT transposition for patellar instability. Finally, the different risk factors for patellar instability examined, particularly the presence of trochlear dysplasia, did not significantly influence the final functional results, which range from good to excellent without re-dislocation episodes.

Acute treatment of medial collateral ligament tears: short to mid-term results.

The aim of this study is to describe the results of a consecutive set of patients treated in acute for the surgical repair of medial collateral ligament (MCL) tears with a mean follow up of 63.78±43.25 months (4- 136). This is a retrospective observational study. From January 2011 to December 2016, 56 patients within the average of 31.75±13.27 (13-55) years old at the time of injury underwent medial compartment repair in an acute setting. The sample size of our study is therefore made up of 26 patients. Patients have been evaluated with functional scores: IKDC (international knee documentation committee evaluation form), the KOOS (Knee injury and osteoarthritis outcome score) and clinical assessment. The Tegner Activity Score was evaluated retrospectively at the 12 months follow-up. The mean KOOS value at the final follow-up were 91.25±9.65 (72-100) for pain, 85.68 ± 12.34 (57-100) for the symptoms category, 94.5±8,07 (75-100) for the activity of daily life, 71.87±22.86 (35-100) for the sport category and 76.37±18.55 (38-100) for the quality of life. At the last follow up the mean IKCD value was 77.68±15.95 (55-98). The mean difference in the Tegner Activity Score between the preoperative time and the postoperative time was 1.06±1.12 with a 95% Confidence Interval 0.46-1.66. The functional outcomes underline how the surgical approach to the medial capsule-ligament compartment of the knee is a reliable treatment to restore excellent joint function. Level of evidence III retrospective observational study.

Management of symptomatic mesenchymal hamartoma of the chest wall: surgical resection only in symptomatic cases.

Mesenchymal hamartoma of the chest wall (MHCW) is a rare neonatal benign tumor with an estimated incidence of 1 in 3 000 among primary bone tumors, and 1 in one million in the general population. Traditionally, the treatment of choice was an "en bloc" resection, but surgery limited to symptomatic cases, is now suggested by most authors due to the numerous cases of spontaneous regressions. We report 2 patients of symptomatic MHCW, characterized by progressive respiratory distress, who underwent surgical treatment with prompt resolution of symptoms. Surgeons and neonatologists should be aware of this rare condition and its possible fatal or nearly-fatal complications.

Acute splenic torsion in children: which is the best treatment? A case report.

Wandering spleen is a clinical entity which rarely affects children and adolescents. This condition can be asymptomatic or responsible of chronic pain, but it appears as a surgical emergency when an acute twisting occurs. The risk of post-splenectomy sepsis in the pediatric population suggests a conservative approach whenever possible, and also in case of acute torsion, most authors prefer to preserve the spleen and perform a splenopexy. The Authors describe a case of a child with acute splenic torsion, in whom a conservative surgical approach was initially adopted. The conservative option has to be balanced with the risk of prolonged thrombocytopenia, multiple transfusions and a possible second procedure to remove the spleen.

Complementary and Alternative Medicine (CAM) in paediatric rheumatology: a European perspective.

OBJECTIVES: To analyse the use of complementary and alternative medicine (CAM) in children with rheumatic diseases, treated at a paediatric rheumatology centre in Italy. METHODS: Parents of children with different kinds of chronic rheumatic diseases anonymously completed a questionnaire about their children's past or current use of CAM. Two groups of patients were analysed: Group A consisted of children who were still attending the centre; Group B consisted of children who had not attended the clinic for more than one year. RESULTS: 150 completed surveys were analysed: 22 paediatric patients (14.7%), 10/100 in group A and 12/50 in group B, used CAM to treat their diseases. The therapies used the most were homeopathy, herbal remedies, vitamins and minerals. We observed a significantly greater use of CAM among patients who had not attended the clinic for more than one year (24%) as compared to those who were regularly checked (10%) (p=0.02). Parents' use of CAM was significantly related to its use for their children (p=0.001). A poor outcome, probably related to the exclusive use of alternative treatments, was observed in three out of six patients who had completely stopped using traditional immunosuppressive drugs. CONCLUSIONS: Physicians should be aware of the use of CAM particularly in patients who skip their regular check-ups. The use of CAM to treat childhood rheumatic conditions in Italy seems to be less frequent than in North America.

PAX2 gene mutations in pediatric and young adult transplant recipients: kidney and urinary tract malformations without ocular anomalies.

Heterozygous humans for PAX2 mutations show autosomal dominant papillorenal syndrome (PRS), consisting of ocular colobomas, renal hypo/dysplasia and progressive renal failure in childhood. PAX2 mutations have also been identified in patients with isolated renal hypo/dysplasia. Twenty unrelated children and young adults with kidney and urinary tract malformations and no ocular abnormalities were retrospectively recruited for PAX2 mutational analysis. All patients had undergone renal transplantation after end-stage renal disease. We identified two new sequence variations: (i) a deletion causing a frameshift (c.69delC) and (ii) a nucleotide substitution determining a splice site mutation (c.410+5 G/A) by predictive analysis. Therefore, we suggest PAX2 molecular analysis to be extended to all patients with congenital malformations of kidney and urinary tract (CAKUT).

[Application of the ROLL technique in a case of bilateral multifocal breast cancer]. / Aplicación de la técnica ROLL en un caso de cáncer de mama multifocal bilateral.

The increasing number of non-palpable breast lesions and the trend to perform less aggressive surgical procedures have prompted the development of radioguided surgery in breast lesions, alone (ROLL) or with sentinel node detection. We present a 55-year-old woman with four mammary lesions diagnosed by magnetic resonance. The ROLL technique allowed not only their characterisation but also the study of the lymphatic drainage of malignant lesions. Moreover, it was useful as a guide for surgical biopsy of the benign lesions. The combination of different colloid size allowed an accurate study of each lesion and avoided radical surgery. The applications of radioguided surgery are ever increasing. Individualisation of each indication will benefit a greater number of patients.

Hematometra in uterus didelphis: a pitfall in teenagers with lower abdominal mass. Report of two cases.

Müllerian malformations represent a wide spectrum of anomaly of the female reproductive tract, usually detected at birth or at puberty. The presence of uterus didelphys and obstructed hemivagina, associated with ipsilateral renal agenesis, is known as Herlyn-Werner-Wunderlich syndrome, a rare congenital anomaly. Two cases of uterus didelphic and hematometra caused by vaginal septum are reported; the girls had a delayed diagnosis due to absence of a specific clinical feature. Clinicians must be aware of the existence of these malformations. An adequate radiological study is mandatory to assess the anatomic conformation, and to optimize the therapeutic procedures.

Polypoid lesion of the gallbladder in childhood: case report and literature review.

Gallbladder polypoid lesions are rare in the pediatric patient and sometimes represent an incidental finding. A 13 year old male was referred to the Padua Hospital Pediatric Department for an obesity. A routine abdominal ultrasound (US) detected a gallbladder polypoid lesion 6 mm in diameter, initially considered a gallbladder adenoma. Investigation did not detect any other biliary tract abnormality. After seven months, the asymptomatic patient underwent a follow-up US which revealed the disappearance of the polypoid mass. The following concerns are raised: what is the size of the polypoid mass that should be considered for surgery? How does the presence of symptoms worsen the diagnosis and lead to preferring a surgical approach (cholecystectomy) over an echographic follow-up?

Radioguided occult lesion localisation in breast cancer using an intraoperative portable gamma camera: first results.

PURPOSE: The role of the radioguided occult lesion localisation (ROLL) technique in breast cancer has been increasing in recent years. One of the important drawbacks of such a technique is radiotracer spillage within the mammary gland that makes the precise lesion resection difficult, and this requires the use of a hook-wire collocation to reach the lesion. The possibility of obtaining an intraoperative image of the specimen could help to confirm whether the lesion is correctly removed. Some types of portable gamma cameras have been designed, but up to now, intraoperative use has been confined to surgery of parathyroid adenomas and sentinel lymph node location. The aim of the study was to value the usefulness of an intraoperative gamma camera to assess the resection of non-palpable breast lesions. MATERIALS AND METHODS: The study involved 42 women diagnosed with non-palpable early breast cancer. Lymphoscintigraphy was performed on the day before surgery by injection of (99m)Tc-labelled nanocolloid. During surgery a gamma probe was used to guide the surgeon, and afterwards images of the surgical bed and the tumoral specimen were acquired by means of a portable gamma camera, fitted with a pinhole collimator. A (99m)Tc pointer was used to draw an outline image around the specimen. RESULTS: On lymphoscintigraphy, radiotracer was concentrated in 31 cases. During surgery, all lesions were removed. In the images acquired by the portable gamma camera, the lesion was centred inside the surgical specimen in 23 of 42 cases, non-centred in 15 and in contact in 4 cases. Congruence of 60% was found between the intraoperative images and the histopathological results. The posterior margin was the most frequently involved. The whole acquisition time for the tumoral specimen with its margins was 5 min at most. CONCLUSION: The use of portable gamma cameras in theatre is in an early phase. The short period of time required during the surgical procedure will allow the surgical team to improve this technique until it can replace hand-held probes. The intraoperative acquisition of such images can predict the involvement of surgical margins, avoiding future surgical procedures.

[Serious dog bite lesions in childhood: presentation of 3 cases and literature review]. / Lesioni gravi da morso di cane in età pediatrica: descrizione di 3 casi e revisione della letteratura.

Animal bite lesions in the pediatric patient are mainly accounted for by dog bites in 80% to 90% of the cases. They often present a favorable prognosis but serious lesions do account for 5% to 20% of the total incidence. We will present three particular clinical cases which required urgent surgical treatment as well as a review of the current literature to include both medical and surgical treatment methods for this kind of lesion.

Case for diagnosis: 4-month-old infant with increasing cough, hemoptysis, and anemia.

A 4-month-old caucasian infant presented non-productive cough, fever associated with hemoptysis, and increasing anemia. He had mild tachypnoea; routine lab tests were normal. The thoracic HRCT scan showed a very large mass in the right lung adherent to the thorax wall, well defined and limiting the medium and upper lobe; the mass was well vascularized, and with central hypodensic areas. Fiberoptic bronchoscopy and bronchoalveolar lavage (BAL) cytology were normal. The definitive histology of the mass showed the presence of inflammatory cells admixed with fibroblasts and rare Touton giant cells in the lesion suggestive of a juvenile xanthogranuloma (JXG) of the lung.

Thoracoscopic surgery in the management of pediatric malignancies: a multicentric survey of the Italian Society of Videosurgery in Infancy.

BACKGROUND: Few reports have elucidated the role of minimally invasive surgery (MIS) for pediatric malignancies. This study aimed to review the results of a multicenter study on the management of thoracic tumors in children using MIS. METHODS: A 5-year retrospective review of all MIS procedures for the treatment of pediatric malignancies performed in seven centers belonging to the Italian Society of Videosurgey in Infancy is reported. The data from 145 pediatric oncologic patients (80 girls and 65 boys) ages 30 days to 17 years (median, 7.2 years) were analyzed. Of the procedures performed, 87 were laparoscopies (60%), 55 were thoracoscopies (38%), and 3 were lumboscopies (2%). This study focused only on the results of the 55 thoracoscopic procedures performed for diagnostic purposes in 19 cases (34.6%) and for therapeutic purposes in 36 cases (65.4%). RESULTS: The duration of surgery was 15 to 180 min (median, 65 min). Metastasectomies were performed for various etiologies in 31 of the 55 cases. Of the 55 patients, 5 underwent resection of a mediastinal tumor, and 19 underwent a diagnostic thoracoscopy. During a mean follow-up period of 25.6 months, 2 (3.6%) of the 55 patients experienced perioperative complications. CONCLUSIONS: The role of MIS in tumor resection for children is currently limited, but may be used in individual cases when the preoperative workup shows it to be feasible. Its indication is strictly dependent on the thoracoscopic experience of the surgeon and the tumor site for preoperative imaging techniques. When the indication for thoracoscopy is correct, this approach has high therapeutic applicability (65.4% in our series). Our preliminary experience shows that careful patient selection and an appropriate level of technical skill make thoracoscopy a reasonable and safe option for the treatment of pediatric malignancies.

Fibrous Histiocytoma-like Spindle-Cell Proliferation in the Nipple After Body-Piercing.

We report the case of a 19-year-old pregnant woman who presented with a nipple tumor. The lesion consisted in a spindle-cell proliferation with histologic features similar to those of fibrous histiocytoma, with a highly vascularized stroma. Although it showed low mitotic activity, scattered marked atypical cells with prominent nucleoli were identified, thus raising concern about the benign nature of the tumor. Immunohistochemical evaluation revealed that the spindle cells were diffusely positive for vimentin, focally positive for CD68, and negative for all the other tested antibodies. The patient had a total excision of the lesion and she is free of disease after 30 months. To our knowledge this is the first reported case of a lesion of this type in the nipple after body-piercing.

Further experience with OK-432 for lymphangiomas.

This study includes all the children treated with OK-432 for lymphangioma at our institute. Twenty-nine children treated between 1999 and 2003 are reported for the first time: twelve cases regressed completely, eight cases regressed more than 50% and seven remained unchanged; two cases were lost at follow-up. The outcome was related to the size of the cysts, the larger ones having a better prognosis. The adverse reactions are discussed and the methods of treatment are described in detail. Fifteen children, treated before 1999 and already reported, are reviewed after a long-term follow-up. Four had a recurrence: one regressed spontaneously and three needed further treatment. The other 11 had no complaints. Even considering the risk of recurrence, OK-432 therapy remains our first line therapy for lymphangiomas, avoiding surgery in most cases.

Clinical axillary recurrence after sentinel node biopsy in breast cancer: a follow-up study of 220 patients.

PURPOSE: The aim of this study was to evaluate the frequency of false-negative (FN) sentinel node procedures in patients with breast cancer and the subsequent clinical outcome in such patients. METHODS: A total of 325 breast cancer patients underwent sentinel lymph node biopsy at our institution between June 1998 and May 2004. A 2-day protocol was used to localise the sentinel node with the injection of 99mTc-nanocolloid. There were two phases in the study: the learning phase (105 patients) and the application phase (220 patients). In the learning phase, a complete lymphadenectomy was always performed. In the application phase, sentinel nodes were studied intraoperatively and lymphadenectomy was performed when considered warranted by the pathological intraoperative results. RESULTS: The median follow-up duration in the 220 patients studied during the application phase was 21.2 months (range 4-45 months). In this phase a total of 427 sentinel nodes were obtained (range 1-5 per patient, median 1.99), with 66 positive sentinel nodes in 56 patients (26%). The lymphadenectomies performed were also positive in 25% of cases (14 patients). We observed a total of two false-negative sentinel lymph node results (3.45%). One of them was found during the surgical excision of non-sentinel nodes, and the other presented as an axillary recurrence 17 months postoperatively (1.72% clinical false-negative rate). The latter patient died 1 year after the first recurrence. CONCLUSION: After a median follow-up of 21.2 months we observed only one clinical recurrence among 220 patients. Our results indicate that adequate local control is achieved by application of the sentinel node protocol.

[Comparative study of the different radiotracer administration route to locate the sentinel node in breast cancer]. / Estudio comparativo de las diferentes vías de administración del radiotrazador para la localización del ganglio centinela en el cáncer de mama.

AIM: To assess the results of Sentinel Lymph Node (SLN) detection in breast cancer patients depending on the radiotracer injection route, especially evaluating which route that makes it possible to identify the highest percentage of SLNs and its detection in the inner mammary chain. METHODS: We prospectively studied 120 breast cancer patients. Three groups of 40 patients each were established depending on the radiotracer injection route. 1) Subdermal or subareolar route: 18 T1 and 22 T2, mean tumor diameter size of 1.76 cm. 2) Peritumoral route: 16 T1 and 24 T2, mean tumor diameter size of 1.86 cm. 3) Intratumoral route: 20 T1 and 20 T2, mean tumor diameter size of 1.61 cm. The day before surgery, a lymphoscintigraphic study was performed in all cases and intraoperative SLN resection was carried out with the aid of a hand-held gamma probe. RESULTS: The SLN was identified in 38/40 cases (95%) of group 1, in 35/40 cases (88%) of group 2 and in 38/40 cases (95%) of group 3. No SLNs in the mammary chain were observed in patients who underwent subdermal/subareolar injection of the radiotracer. On the other hand, SLNs were visualized in this location in 4/35 patients with peritumoral injection (11%) and in 8/38 patients with intratumoral injection (21%). CONCLUSION: The superficial (subdermal, subareolar) injection technique is more suitable when an unnecessary lymphadenectomy has to be prevented in cases without axillary metastases. The deeper injection technique (peritumoral, intratumoral) should be use when, moreover, we seek accurate staging or plan non-systematic mammary chain irradiation.

Kidney transplantation in children with reconstructed bladder.

There is some controversy about the safety of renal transplantation in patients with an augmentation cystoplasty because of the possibility of urinary tract infection in immunosuppressed patients leading to pyelonephritis and graft loss. Nevertheless, it is now well known that in patients with a small volume and poorly compliant bladder, reconstructive bladder surgery (augmentation cystoplasty or continent reservoir) creates a low-pressure and compliant reservoir, which protects the upper urinary tract and restores a functional lower urinary tract. Graft survival is not adversely affected when a kidney transplant is drained into a reconstructed bladder. When bowel segments are used for augmentation, a voiding modality with clean intermittent self-catheterization does not increase the risk of urinary tract infections, even in immunosuppressed patients.