[Performance of prenatal diagnosis and postnatal development of congenital lung malformations]. / Performance du diagnostic anténatal et évolution postnatale des malformations pulmonaires congénitales.

OBJECTIVES: For many diseases, the comparison of prenatal diagnosis with a histopathological reality is not always possible. Fetal lung pathology, with its high rate of surgery in postnatal, allows this assessment. This study proposes an approach to the reliability of prenatal diagnosis and analysis of the postnatal development of all children in care for congenital pulmonary malformation (CPM). METHODS: This is a retrospective study of all cases of CPM diagnosed in Poitiers University Hospital from 1995 to 2011. Cases diagnosed prenatally were identified and the diagnostic accuracy was studied by histology when cases had surgery. The postnatal development of prenatally diagnosed cases is described and compared to children who did not receive prenatal diagnosis. RESULTS: Among the 45 cases of CPM supported at the Poitiers University Hospital, 30 had received prenatal diagnosis of isolated CPM. The diagnostic concordance between antenatal ultrasound and the final diagnosis is κ=0.67 (CI95% [0.38 to 0.94]). The sensitivity of ultrasound was 90% (CI95% [55-99.7]) in our series for the diagnosis of CAMP (cystic adenomatoid malformation pulmonary). We found a sonographic disappearance of lesions in 4 children, 1 child in regression, stable lesions in 21 cases. Four children showed an increase in volume of the malformation, with signs of poor tolerance in 3 cases. After birth, children who received a prenatal diagnosis were no more symptomatic than those whose diagnosis was made postnatal: 21 (70%) versus 11 (73%; P=1) respectively. Similarly, they often received prophylactic surgery: 18 (60%) versus 2 (13%) respectively (P<0.01) and less often suffered post-surgery complication: 3 (10%) versus 10 (67%) respectively (P<0.01). The number of children monitored was not significantly different in the two groups. CONCLUSION: Prenatal diagnosis allows for the precise nature of the lesion in 90% of cases in 2013 and had no impact on symptomatology at birth. When prenatal diagnosis is possible, preventive surgery probably reduces the occurrence of emergency surgery.

[Current place of curettage in the management of giant congenital nevi: report of 29 patients]. / Place actuelle du curetage dans la prise en charge des nævi géants congénitaux: à propos de 29 cas.

SUBJECT: The management of giant nevi remains discussed to date. Curettage, early superficial technique, allows a lightening of the lesions. We wanted to define the current interest in its use, from results obtained after 20 years of experience. PATIENTS AND METHODS: Twenty-nine patients were treated by curettage between 1991 and 2011. Surgery consisting of excision of the superficial dermis was performed between the 1st and 7th week of life in an average of 1.8 procedures. The cosmetic result was judged subjectively. RESULTS: Healing was achieved in 7 days. We noted a few local complications but no general complications. The aesthetic results, with initial variable lightening, induced an overall parental satisfaction in 100 % of cases. A secondary heterogeneous and varying pigmentation was observed in all cases. CONCLUSION: To date, we believe that curettage is indicated for the treatment of giant nevi in some localization difficult to treat by conventional techniques (eyebrow, those associated with many satellite nevi) or those with a psychological impact too important to support a therapeutic delay.

[Pediatric keratocystic odontogenic tumor and nevoid basal cell carcinoma syndrome. Predictive factors for recurrence and aggressiveness]. / Tumeurs kératokystiques odontogènes de l'enfant et syndrome de Gorlin. Comment expliquer les récidives et l'agressivité des lésions ?

INTRODUCTION: Keratocystic odontogenic tumors (KOT), as complications in Nevoid Basal Cell Carcinoma Syndrome (NBCCS), occur early (before 20 years of age) and are usually more aggressive. The aim of this retrospective study was to determine the clinical, histological, and genetic phenotype, of these lesions and to define predictive features of aggressiveness. PATIENTS AND METHODS: We retrospectively studied five patients presenting with one or several KOT with NBCCS. We collected their clinical, radiological, and therapeutic data, rate of recurrence or new localization. Anatomopathological examinations were reviewed systematically. Somatic PTCH, SMO and SMAD 4 sequencing were completed. RESULTS: The average age at diagnosis was 11.2 years. The average number of KOT was 3.2 most often located in the molar region. All the cysts were enucleated. Anatomopathological examination revealed the presence of satellite cysts and daughter cysts and epithelial expansion in more than 80% of cases. No somatic mutation was observed among KOT. DISCUSSION: KOT develop in the first 10 years, in patients presenting with NBCCS, and recurrence is observed in the second and third decade. KOT are typically aggressive and have a tendency to recur, especially in patients with NBCCS. Anatomopathological examination may be predictive of the lesion's aggressiveness. Understanding the genetic and immunological mechanisms should open the way for new medical treatment.

The return of the solitary testis.

PURPOSE: To assess what is done and what is recommended concerning fixation of the solitary testis. MATERIAL AND METHOD: We conducted an e-mail survey of current practices in 28 pediatric surgery departments in 28 university or general hospitals in France. We then reviewed what evidence could be found in the literature. RESULTS: All surgeons fix the contralateral solitary testis following intravaginal torsion. Sixteen out of 28 fix the contralateral solitary testis following extravaginal torsion, 13/28 in cases of monorchia, and 8/28 following orchiectomy for trauma or tumour. Five surgeons have observed one case each of torsion of a solitary testis, and three have witnessed testicular necrosis following orchiopexy. CONCLUSION: There is no consensus regarding fixation of the remaining testis in the literature. Scientific evidence does not show clearly whether fixation is necessary, regardless of the clinical situation. However, if fixation is performed it should be done using the dartos pouch sutureless technique.

[Pylephlebitis in the child: a challenging diagnosis]. / Pyléphlébite chez l'enfant: un diagnostic difficile.

Pylephlebitis or septic thrombophlebitis of the portomesenteric veins is a complication of intra-abdominal infections. The disease is rare in children and the diagnosis is often delayed. The morbidity of pylephlebitis is relatively low, although there is a risk of residual thrombosis. We report on 2 cases of pylephlebitis in a 12-year-old girl and a 13-year-old boy, following undiagnosed appendicitis. In the 1st case, the young girl had been misdiagnosed with Salmonella infection and was given antibiotics; in the 2nd case, the boy had retrocecal appendicitis that was clinically subacute. An accurate diagnosis was finally made in both cases by CT scan. Both children evolved satisfactorily following appendectomy, long-term antibiotics, and anticoagulation. Clinically, the severe sepsis associated with pylephlebitis is at the forefront. Physical examination is often normal and therefore of little help; the knowledge of a preceding abdominal infection leads to further radiological investigations. Biologically, there are pronounced signs of infection. CT is the preferred exam for diagnosing pylephlebitis, as it can also show the underlying cause of the intra-abdominal sepsis or possible complications. Doppler sonography is recommended more for follow-up of the portal vein thrombosis. Treatment of pylephlebitis associated with appendicitis always includes long-term antibiotics. An appendectomy is always performed either at the time of diagnosis or later. The need for anticoagulation therapy in children is controversial. However, most pediatricians recommend its use, beginning as soon as possible, to be continued until normalization of portal vein flow.

Management of a ruptured infected abdominal aortic aneurysm and a spondylodiscitis due to Gemella haemolysans.

Infected aneurysm of the abdominal aorta is an uncommon and highly lethal disease, with a >80% rate of death. It is uncommonly associated with spondylodiscitis. Management of this rare association of pathology remains controversial. This report details the case of a patient with a large vascular past history, presenting a scarce association of disease: a ruptured infected aneurysm of the abdominal aorta with a spondylodiscitis due to Gemella haemolysans. The patient recovered quickly and durably, thanks to an unusual cooperation of both vascular surgeons and orthopedists: He underwent an in situ graft replacement and a front vertebral fixation in a single-step surgery. Good immediate and midterm outcome shows a new way of treatment in this rare but severe situation.