Safety of laparoscopic fundoplication in children under 5 kg: a comparative study.

BACKGROUND: Laparoscopic fundoplication in children under 5 kg is still debated. Our objective was to evaluate the safety and efficacy of laparoscopic fundoplication (LF) in children under 5 kg. METHODS: We reviewed the cases of 96 children treated by laparoscopic fundoplication between 2005 and 2014. Thirty-five patients had a weight of 5 kg or less at the time of LF (Low Weight Group) and 61 patients had a weight between 5.1 and 10 kg (High Weight Group). The pre-operative, peri-operative, post-operative data regarding surgery and anesthesia were compared between groups. RESULTS: Mean weight was 3.9 ± 0.8 kg in the LWG and 7.8 ± 1.5 kg in the HWG. Children in the LWG were more prone to pre-operative respiratory management (40% mechanical ventilation and 42.9% oxygen therapy). The operating times (82 ± 28 min for LWG and 85 ± 31 min for HWG) and respiratory parameters during the procedure (PCO2) were comparable between groups. Post-operative complications were 1 gastric perforation with peritonitis and 1 small bowel obstruction in the LWG, 2 cases of gastric perforation with peritonitis in the HWG. Mean follow-up was 67 ± 44 months. Significant recurrence of GERD requiring a redo fundoplication was noted in 3 patients in the LWG and 1 patient in the HWG. CONCLUSION: Laparoscopic fundoplication is a safe procedure in infants ≤ 5 kg without increase of post-operative complications, recurrence, or mean operative time.

Celio-mesenteric trunk associated with giant omphalocele: surgical consequences / Tronco celíaco-mesentérico asociado con onfalocele gigante: consecuencias quirúrgicas

We report the first case of a newborn presenting with a celio-mesenteric trunk variation associated with a giant omphalocele. The celio-mesenteric trunk was unexpected and unseen during the staged surgical closure. After partial reintegration of the liver, the newborn presented refractory hypovolemia with anuria, leading to redo surgery. This procedure revealed ischemia of the liver and necrosis of the entire gastrointestinal tract except the colon. Despite treatment, including liver externalization, the infant did not survive. The autopsy revealed a celio-mesenteric trunk, a rare anomaly characterized by a common origin of the celiac axis and the superior mesenteric artery from the aorta. This association may explain the dramatic consequences of the staged closure procedure. Awareness of the association of celio-mesenteric trunk and omphalocele would allow the surgeon to take extra care during this delicate surgery.

Presentamos el primer caso de un recién nacido que presenta una variación del tronco celíaco-mesentérico asociada con un onfalocele gigante. El tronco celíaco-mesentérico fue inesperado y no se vio durante las etapas del cierre quirúrgico. Después de la reintegración parcial del hígado, el recién nacido presentó hipovolemia refractaria con anuria, lo que condujo a la repetición de la cirugía. Este procedimiento reveló isquemia del hígado y necrosis de todo el tracto gastrointestinal excepto el colon. A pesar del tratamiento, incluyendo la externalización hepática, el bebé no sobrevivió. La autopsia reveló un tronco celíaco-mesentérico, una rara anomalía caracterizada por un origen común del tronco celíaco y la arteria mesentérica superior, a partir de la aorta. Esta asociación puede explicar las dramáticas consecuencias del procedimiento durante las etapas del cierre. El conocimiento de la asociación de tronco celíaco-mesentérico y onfalocele permitiría al cirujano tomar especial cuidado durante esta delicada cirugía.

The Fisher Technique for Correction of Penile Torsion in Children: Who Are the Candidates?

OBJECTIVE: To report our experience in the treatment of penile torsion with a special reference to the Fisher technique. PATIENTS AND METHODS: We reviewed 30 cases of congenital penile torsion treated from 2009 to 2015 in a single center. Sixteen patients presented a moderate rotation of 45°-90° and 14 had a severe rotation with an angle greater than 90°. Chordee was present in 17 cases and distal glanular hypospadias in 15 cases. Median age at surgery was 10 months. In 14 cases, degloving along with skin realignment, alone, allowed correction of the torsion. In 16 cases, the Dartos flap (Fisher technique) was used. RESULTS: Out of the 30 patients, 29 had a good result, with a complete correction of the torsion. Only 1 patient had a residual torsion of 30°. Complications were minor and consisted of skin excess in 11 patients; 1 single case needed to be reoperated for that skin complication. The need for the Dartos flap procedure was significantly correlated to the degree of torsion (P = .001): it was used in 4 out of 16 patients (25%) with a torsion of less than 90°, and in 12 out of 14 patients (86%) with a torsion of 90° or more. CONCLUSION: Most of penile rotations less than 90° can be corrected by skin degloving and realignment. The Fisher technique is a simple and safe procedure for correction of persistent rotation after skin degloving; such cases have usually a penile rotation greater than 90°.

Adrenocortical tumor with precocious puberty in a 2-month-old girl.

Adrenocortical tumor is a rare childhood tumor with a median age at onset of 3.2 years. Virilization is the most common sign. Laparotomy is the reference treatment and has a favorable course. The diagnosis of adrenal tumor can be difficult. The main parameters of malignant tumors are size and metastasis. Analysis of TP53 mutation can facilitate final diagnosis. We report a case of virilizing adrenal tumor that developed in a 2-month-old girl, and which was treated with laparoscopic adrenalectomy.

Q-island flap urethroplasty: 1-stage procedure for reconstruction of Y-type urethral duplications in children.

PURPOSE: We review our experience with Q-flap urethroplasty in children with Y-type urethral duplication. MATERIALS AND METHODS: Six children with Y-type urethral duplication underwent 1-stage urethral reconstruction using Q-flap urethroplasty between 1988 and 2012. The ventral hypospadiac urethra was always the functional one, and, therefore, was used for urethroplasty. RESULTS: Mean age at surgery was 5 years (range 1 to 13). All 6 patients had an excellent cosmetic result, with a normal appearing penis, a large apical meatus and no skin chordee. All 6 voided normally. None of the 6 children required reoperation. The only complications were urethral stenosis in 2 patients and lower urinary tract infection in 3. Cosmetic and functional results were analyzed during a mean followup of 8 years (range 1 to 12). CONCLUSIONS: Q-flap urethroplasty provides a long penile skin flap that allows 1-stage reconstruction in children with Y-type urethral duplication, with good cosmetic and functional results.

Laparoscopic treatment of renal cancer in children: a multicentric study and review of oncologic and surgical complications.

OBJECTIVE: The aim was to report a multicentric study with a longer follow-up to evaluate the laparoscopic radical nephrectomy in children with renal cancer. MATERIAL AND METHODS: This was a retrospective multicentric study, from October 2005 to January 2012, of children who underwent a laparoscopic radical nephrectomy for small renal malignant tumors. RESULTS: Seventeen children were included in this study. Sixteen underwent chemotherapy before surgery according the SIOP (Société Internationale d'Oncologie Pédiatrique) protocol and one was treated by surgery only for a carcinoma. All except one could be treated by laparoscopy; the biggest tumoral size was 8 cm. The median hospital stay was 3 days (2-10). The pathologic examination showed 15 Wilms' tumors, one clear cell sarcoma and one TFE3 renal cell carcinoma. With a median follow-up of 42 months (range 12 and 77 months) after laparoscopic radical nephrectomy, 15 children had no oncological complications (port site or local recurrence, pulmonary metastasis) and one had a local recurrence without intraoperative tumoral rupture. The child with TFE3 renal cell carcinoma died 4 years after surgery from brain and lung metastases without local recurrence. No small bowel obstruction occurred. CONCLUSIONS: Radical nephrectomy in children for Wilms' tumor or other renal cancer can be safely performed laparoscopically and our indications can be summarized, for trained laparoscopic surgeons, by small tumors under about 8 cm diameter, especially without crossing the lateral edge of the vertebra on the CT scan at the time of surgery.

Hypercalcemia in a child with juvenile granulosa cell tumor of ovary: Report of an unusual paraneoplastic syndrome and review of the literature.

► Hypercalcemia is an extremely rare paraneoplastic syndrome in children. ► Small cell carcinoma is the commonest ovarian tumor associated with hypercalcemia. ► Small cell carcinoma must be ruled out because of poor prognosis. ► We report the only third case of JGCT associated with paraneoplastic hypercalcemia.

Congenital megaprepuce: a 12-year experience (52 cases) of this specific form of buried penis.

OBJECTIVE: Congenital megaprepuce is a specific form of buried penis. Children affected by this malformation usually suffer from a subjective difficulty in voiding which is the main indication for early surgical correction. The aim of this study was to report a single center's 12-year experience in the treatment of megaprepuce in children, describing the surgical procedure we used to treat it and the results. PATIENTS AND METHODS: We retrospectively reviewed the charts of all children who underwent congenital megaprepuce repair between January 1999 and August 2011 in our institution. Fifty-two children were operated during the study period. Our single surgical technique, not an original one, consists of fixing the penile shaft at the base of the penis, and widely reducing the inner prepuce. RESULTS: Four children (8%) underwent revision surgery but according to our criteria a very good result was observed in only 23 patients, and a less good but still acceptable result in 25 patients. CONCLUSION: Our 12-year experience in the surgical treatment of congenital megaprepuce demonstrated satisfying results from a safe and simple surgical technique, but to achieve the optimum result remains difficult.

Acute gastric volvulus: an unreported long-term complication of pericardial drainage.

We report the case of a girl who had tetralogy of fallot (TOF) repaired at birth without any associated diaphragmatic hernia. At the age of 2½ years, she experienced an upper gastrointestinal occlusion. At laparoscopy an organoaxial gastric volvulus was observed related to a peritoneal adhesion secondary to pericardial drainage that had been performed at the time of the TOF repair. After reduction of the volvulus, a phrenofundopexy was done. Postoperatively, the child has remained asymptomatic with a follow-up of 24 months. There are few cases of pericardial drainage complications documented in the literature but none in the pediatric population. Based on this observation, we advise that during pericardial drainage tube placement, the peritoneal cavity be carefully avoided to prevent formation of intra-peritoneal adhesions and the risk of gastric volvulus.

Quality of life in adults with bladder exstrophy-epispadias complex.

PURPOSE: We evaluated quality of life in adults with bladder exstrophy-epispadias complex to improve patient and parent counseling. MATERIALS AND METHODS: We evaluated quality of life in a multicenter study using the SF-36® Medical Outcome Study questionnaire and a local questionnaire. A total of 47 patients born in 1957 to 1990 were followed due to bladder exstrophy-epispadias complex at the 4 participating hospitals. RESULTS: Nine women and 16 men were included in analysis, including 15 who underwent staged reconstruction for bladder exstrophy-epispadias complex and 10 who underwent primary or secondary urinary diversion. Nine patients were married and 2 were in a long-term partnership. A total of 22 patients achieved a high school degree with examination success and 17 worked full time. Six women and 12 men achieved penetrative intercourse. A total of 13 children were born to 3 women and 7 men. Quality of life scores in our patients were less than the norm based scores on 2 of the 8 health concepts, including limitations in physical activity due to health problems and general health perception. Results were statistically different among patients depending on dryness, voiding and urinary reconstruction/diversion. Patient scores did not differ in regard to gender, number of interventions, sexual life, cosmesis or renal function. CONCLUSIONS: Patient scores were less than norm based scores on only 2 health concepts and patients were generally well integrated into society. Urinary diversion was usually well tolerated. It remains a therapeutic option when incontinence interferes with social life or renal function is compromised.

Portal venous gas and thrombosis complicating superior mesenteric artery syndrome (Wilkie's syndrome) in a child.

We report a case of a 10-year-old girl presenting with portal venous gas and thrombosis associated with superior mesenteric artery syndrome. To our knowledge, this is the first reported case of superior mesenteric artery syndrome complicated by gastric wall pneumatosis, portal venous gas, and thrombosis in childhood. Although these complications usually lead to bowel resection in adults and result in a high mortality rate, our pediatric patient was successfully treated nonoperatively with intensive care management and jejunal tube feedings. Presence of portal venous gas may occur in superior mesenteric artery syndrome in children and does not necessarily lead to bowel injury, allowing conservative medical management as a first-line treatment.

Severe bladder dysfunction revealed prenatally or during infancy.

OBJECTIVE: Although thought to be an acquired condition, non-neurogenic neurogenic bladder may sometimes be a congenital dysfunction, revealed before toilet training. We report our experience with the condition diagnosed prenatally or during early infancy. PATIENTS AND METHOD: We retrospectively reviewed cases of severe bladder dysfunction with upper-tract impairment, without neurological or obstructive pathology, in children diagnosed before toilet training: five with prenatal diagnosis of severe hydro-ureteronephrosis (group 1) and six with signs of bladder dysfunction during infancy (group 2). RESULTS: Follow up of group 1 showed decompensation toward severe bladder dysfunction, diagnosed after either toilet training or ureteral reimplantation (n=3). After a median follow up of 14 years (0.5-20), four were on clean intermittent catheterization with bladder augmentation and one required sphincteric re-education with good result. Two of the five had chronic renal failure. In group 2, six children (two females) presented at median age of 20 months (2-30) with indirect signs of bladder dysfunction, including vesicoureteral reflux (n=4) and/or hydro-ureteronephrosis (n=4). After a median follow up of 11 years (5-20), three were on clean intermittent catheterization (two Mitrofanoff channels), and three underwent bladder augmentation. Three children had chronic renal failure of whom one underwent renal transplant. CONCLUSION: These cases of severe bladder dysfunction were initially misdiagnosed. In both groups, follow up revealed severe dilatation of the upper tract and secondary renal impairment. Antenatal diagnosis of bilateral pyeloureteral dilatation may be the first sign of early bladder dysfunction.

The outcome of prenatally diagnosed renal tumors.

PURPOSE: We assessed the incidence of perinatal morbidity and evaluated the outcome in children with prenatally diagnosed renal tumors in a retrospective multicenter study. MATERIALS AND METHODS: A review of the records of patients from 20 institutions identified 28 children with prenatally diagnosed renal tumors. Prenatal findings, clinical charts, and radiological, surgical and pathological reports were reviewed in this study. RESULTS: There were 26 congenital mesoblastic nephromas and 2 Wilms tumors. One or more complications were identified in 20 of the 28 cases (71%) during the perinatal period. Polyhydramnios was observed in 11 fetuses (39%), 2 presented with hydrops fetalis and 7 presented in acute fetal distress requiring emergency cesarean section, of which 1 died in utero before delivery. Median gestational age of the 27 neonates born alive was 35 weeks (range 29 to 39), including 13 (46%) who were pre-term (less than 34 weeks of gestation). Complications at birth included hemodynamic instability in 3 newborns, of whom 2 underwent emergency surgery, respiratory distress syndrome in 8 (30%) and hypertension in 6 (22%). Surgical complications occurred in 7 patients (26%), including tumor rupture in 1 and intraoperative bleeding with postoperative death in 1. At a median followup of 42 months (range 2 to 105) 26 of the 27 children were in complete remission. CONCLUSIONS: Fetal renal tumors have an excellent oncological outcome but a high risk of perinatal complications. Prenatal diagnosis should allow planning the delivery at a pediatric tertiary care center to avoid a potentially life threatening condition in neonates in the first hours of life.

[Impalpable testis: complete inguinal exploration by laparoscopy]. / Testicule impalpable: exploration inguinale complétée éventuellement par une laparoscopie.

OBJECTIVE: To prospectively evaluate an inguinal approach to surgery for impalpable testis possibly assisted by laparoscopy during the same operation. MATERIAL AND METHODS: From January 1996 to March 2002 each case of unilateral impalpable testis was operated via an inguinal incision and laparoscopy was performed during the same procedure whenever the inguinal canal was devoid of any testicular structures. Sixty boys were included in this study. Anatomical features were evaluated and testes were classified according to their trophicity and their site. We evaluated the number of laparoscopies and the factors justifying this procedure. Orchidopexy was performed via an inguinal approach with extensive retroperitoneal dissection to release spermatic vessels, when necessary. A one-stage Fowler-Stephens operation was performed when the vascular pedicle remained too short despite this dissection. The results of orchidopexy were evaluated by physical examination with a minimum follow-up of 4 months. RESULTS: In this series of 60 cases, we identified 19 viable testes. Fourteen were situated in the abdomen, 3 in the inguinal canal, and 2 in a perineal ectopic position. The other cases were considered to present testicular atrophy. Laparoscopy was performed in 23 boys (38%) in whom no viable or atrophic testis was demonstrated via the inguinal incision. Sixteen of the 19 viable testes were descended by preserving the spermatic vessels with a good result. Two Fowler-Stephens operations gave a good result. One testis was removed. CONCLUSION: An inguinal incision, possibly completed by laparoscopy, appears to be a simple and reliable method of evaluation and treatment of impalpable testis.

Total pneumonectomy as a salvage procedure for pulmonary arteriovenous malformation in a newborn: report of one case.

Pulmonary arteriovenous malformations are infrequent lesions diagnosed rarely in the neonatal period. A case of arteriovenous malformation involving an entire lung in a newborn is described. Because of an untractable cyanosis with heart failure, an emergency left total pneumonectomy had to be done at 18 hours of life. Cyanosis disappear immediately, and the newborn recovered normal cardiac function.