Porous Ceramic Sternal Prosthesis Implantation in a 13-Year-Old Patient Presenting with Metastatic Ewing's Sarcoma

Ewing's sarcoma is the second most frequent primary malignant bone tumor in adolescents and young adults. Locations on the thoracic wall represent up to 20% of primary and secondary locations. We present the case of a 13-year-old patient treated with the use of a radiolucency porous bioceramic prosthesis as a sternal replacement for a wide tumor resection in an oncologic context. Focal radiation therapy was not possible due to the high risk of severe myocardial injuries caused by the sternal location of the tumor. The sternum CERAMIL® (I.CERAM, Limoges, France), in porous alumina (Al2O3) has already been implanted into adults in sternal replacement during its invasion by a tumor or its infectious destruction. There were no complication concerning the surgery. The last follow-up at 2 years postoperatively reveals a satisfactory clinical situation with any functional thoracic complaint and nor any functional respiratory symptoms. The porous alumina sternal prosthesis offers a reliable alternative for sternal replacement indications for children in an oncologic context.

Posterior Cloacal Variant with Clitorolabial Transposition and a Rectoperineal Fistula

We present a case and discuss the management of a posterior cloacal variant not as yet described in the literature. A 5-week-old infant presented to our institution with a posterior cloacal variant and transposition of the clitoris and labia. After initial radiological investigations, staged operative intervention was performed over a 1-year period. This included an initial laparotomy (with drainage of hydrocolpos and formation of a colostomy), a left ureteric reimplantation and a posterior sagittal anorectoplasty due to a rectoperineal fistula. The child is under continued long-term follow-up by our specialist pediatric surgical team.

Pelvic Venous Congestion Secondary to a Circumaortic Renal Collar in an Adolescent Female: Report of a Case

We report a 13-year-old girl who presented with a recurrent abdominal pain that started after her menarche. The abdominal palpation revealed tenderness over the left ovarian point. The laboratory study, ultrasonography, and abdominal X-ray were normal. The computed tomography and magnetic resonance imaging showed a double left renal vein with a retroaortic component, an increased left parauterine circulation, and ipsilateral ovarian vein engorgement. A diagnostic and therapeutic phlebography allowed a selective catheterization of a group of pelvic varicose veins draining to the left ovarian and to the internal iliac veins. There were no complications during the procedure and the symptoms disappeared 2 days later. Circumaortic left renal vein may cause hematuria, proteinuria, pelvic congestion syndrome, and massive hemorrhage during surgery. A conservative treatment is recommended for patients without gynecourological/renal symptoms or with mild hematuria. The endovascular treatment by gonadal venous embolization is safe and effective.

Communicating Bronchopulmonary Foregut Malformation Type IB: Diagnostic and Surgical Challenges

Communicating bronchopulmonary foregut malformations (CBPFM) are extremely rare. We present a complex case of type IB CBPFM with esophageal atresia and distal tracheoesophageal fistula (EA/TOF), duodenal atresia/annular pancreas (DA/AP), and intestinal malrotation who underwent primary repair for EA/TOF on day 3. Bilious aspirates on day 8 prompted an upper gastrointestinal (GI) contrast revealing a duodenal obstruction and communication between the right lung lower lobe and the esophagus (T8-T9 level). DA/AP and malrotation were repaired by a gastrojejunostomy and Ladd's procedure. A repeat contrast swallow identified a 2nd communication from the esophagus into the right lower lobe (T5-T6 level) raising the suspicion of a recurrent TOF. Computed tomography (CT) thorax confirmed above findings with an anomalous blood supply to right lung. An exploratory thoracotomy identified a three-lobed lung. However, the lower lobe was enlarged and connected in two separate locations to the esophagus. The child recovered after the disconnection of the esophageal connections and partial right lower lobectomy. CBPFM are extremely rare anomalies requiring a high index of suspicion, use of an upper GI contrast series, and CT scans for diagnosis. The treatment of choice is resection of the affected lung and disconnection of the esophageal communications.

Preoperative Embolization Facilitates Segmental Resection of Pulmonary Sequestration in an Infant

The most common congenital lung malformations are congenital pulmonary airway malformations and pulmonary sequestrations. Many surgeons advocate resection to prevent complications of infection, malignancy, and pneumothorax. The standard of care is lobectomy, but segmentectomy and embolization alone have been reported. These methods avoid the complications of lobectomy but are not widely practiced due to concerns about incomplete resection or involution of the lesion. We present a novel approach to the treatment of a pulmonary sequestration in a 7-month-old male using preoperative embolization followed by a sublobar pulmonary resection. The embolization clearly demarcated the affected lung intraoperatively, thereby facilitating complete removal of the lesion with a segmental lung resection rather than complete lobectomy.

Skip Segment Hirschsprung Disease Managed by Pull-Through of the Right Colon

Hirschsprung disease is the most common neurocristopathy in children, resulting in the congenital loss of enteric ganglia. Rare reports of skip lesions have previously been reported in the literature. We present a case of skip lesions known prior to surgery and managed by pull-through of the right colon that allowed the preservation of the colon.

Congenital Anterior Urethrocutaneous Fistula with a Persistent Urethral Groove

Congenital anterior urethrocutaneous fistula (CAUF) is a rare penile anomaly with only 63 cases reported in the literature. The anomaly can present in isolation or in association with chordee or hypospadias. We report the case of an 8-month-old boy with CAUF that resembles the embryological urethral groove. On examination, a wide urethral groove was noted to cover the midshaft of the penis with a well formed urethra extending proximally and distally and with a normal glandular anatomy, a wide glandular meatus, and a complete foreskin. The urethral groove was tubularized and covered in layers. Surgery was complicated with early superficial skin dehiscence not affecting the urethral repair.Refashioning of the skin was then performed. A satisfactory aesthetic and functional outcome was observed at 7 years' follow-up. Defining the anatomy of CAUF and distal urethra is key in management of these children.

Posterior Mediastinal and Cutaneous Back Hemangiomas in Infants: A New Association

Infantile hemangiomas (IHs) are common vascular tumors. In most cases, a benign course with favorable outcome can be anticipated. IH typically present as cutaneous lesions either with a localized or diffuse segmental distribution. Segmental hemangiomas in the face may be associated with brain and cardiac anomalies (PHACES syndrome), whereas airway involvement has been reported to be associated with hemangiomas in the “beard” area. Multiple cutaneous hemangiomas may be associated with visceral hemangiomas (commonly in the liver).In this report, we present a new association where deep paravertebral hemangiomatous lesions were observed to be associated with cutaneous back hemangiomas in two consecutive cases.

Advantage of Using 8K Ultra-High-Definition Television System for Kasai Portoenterostomy for Biliary Atresia

Kasai portoenterostomy (KPE) is currently the first-line treatment for biliary atresia. Many pediatric surgeons have reported that the dissection of the fibrous remnant at the porta hepatis is one of the most important components of this procedure. Furthermore, laparoscopic portoenterostomy is being increasingly used to treat biliary atresia.An advantage of laparoscopic surgery is that surgeons can more easily identify microbiliary ducts, owing to the magnification. We report the case of a 61-day-old girl on whom we performed an exploratory laparotomy and diagnosed type III biliary atresia using intraoperative cholangiography. For the first time, we performed an open KPE using an 8K ultra-high-definition television system. This allowed us to clearly view the porta hepatis and to successfully perform the portoenterostomy.

Hemi-Clamshell Approach for Fetal Lung Interstitial Tumor Resection in a Neonate: A Case Report

Fetal lung interstitial tumor (FLIT) is a rare primary lung mass in neonates. Classical incisions, such as posterolateral thoracotomy or median sternotomy, do not provide optimal exposure of the operative field for the resection of pediatric thoracic giant tumors. Herein, we report a rare case of a FLIT in a full-term male neonate, with complete resection achieved using a hemi-clamshell approach, which provided the required visualization of the operative field. The neonate was transferred to our hospital because of mild respiratory distress, which developed 18-hour after normal vaginal delivery. A mass in his right chest, without a midline shift, was observed on chest radiographs. Computed tomography showed a well-circumscribed solid anterior cervicothoracic mass, with a uniform density and no apparent cysts, diagnosed as a primary thoracic giant tumor. Once the patient was clinically stabilized, we proceeded with right upper lobectomy, using a hemi-clamshell approach, full sternotomy, and anterolateral thoracotomy, on postnatal day 22.Histopathologic examination revealed an 8.5 × 6.5 × 4.0 cm solid mass within the right upper lobe, which was diagnosed as a FLIT. His postoperative recovery was uneventful. The patient was followed up for 1 year, with no complaints or symptoms and no postoperative shoulder dysfunction. Gross total resection of primary thoracic giant tumors can be accomplished in neonates with optimal exposure of the chest cavity using a hemi-clamshell approach.