Pectus Arcuatum: A Pectus Unlike Any Other Abdellaoui, Sarah; Scalabre, Aurélien; Piolat, Christian ...
Journal of pediatric surgery,
09/2023, Volume:
58, Issue:
9
Journal Article
Peer reviewed
Pectus arcuatum is often mistaken for a type of pectus carinatum. However, pectus arcuatum is a unique clinical form of pectus caused by premature obliteration of the sternal sutures (manubrial ...sternum, four sternebrae and xiphoïd process), whereas pectus carinatum is due to abnormal growth of the costal cartilage. In order to better describe pectus arcuatum, we analysed the files of patients with pectus arcuatum followed in our centers.
Multicenter retrospective study of young patients’ files diagnosed with pectus arcuatum.
The clinical diagnosis of pectus arcuatum was made in 34 patients with a mean age at diagnosis of 10.3 years (4–23 years).
A chest profile X-ray or a CT scan was performed in 16 patients (47%) and confirmed the diagnosis of PA by the presence of a sternal fusion. It was complete in 12 patients.
A malformation was associated in 35% of cases (Noonan syndrome 33%, scoliosis 25% or cardiopathy 16%). 11 patients (32%) had a family history of skeletal malformation.
Orthopedic treatment was initiated in 3 patients without any success. 11 patients underwent surgical correction, which was completed in 7 of them.
The diagnosis of pectus arcuatum is based on clinical experience and if necessary, on a profile chest X-ray showing the fusion of the sternal pieces. It implies the search for any associated malformations (musculoskeletal, cardiac, syndromic). Bracing treatment is useless for pectus arcuatum. Corrective surgery, based on a sternotomy associated with a partial chondro-costal resection, can be performed at the end of growth.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Testicular transposition (TT) before scrotal external radiotherapy (RT) is poorly reported in children with cancer, with only rare case reports published. TT surgical techniques, dosimetric ...parameters, and testicular functions are retrospectively reported in 12 children, median age 12.8 years, after scrotal RT for sarcomas. TT has low morbidity and allows a dramatic RT dose decrease in the healthy testicle. Endocrine functions seem preserved while more follow‐up is needed to assess fertility. Though a rare situation, TT should be discussed in children and young adult cases when a scrotal high‐dose RT is needed.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Background
Chemotherapy for non‐seminomatous germ cell tumours (NSGCT) exposes to dose‐dependent toxicities. The TGM13‐NS protocol (EudraCT 2013‐004039‐60) aimed to decrease the chemotherapy burden ...compared to the previous TGM95 protocol while maintaining the 5‐year event‐free survival (EFS) at 80% or more.
Procedure
Patients less than 19 years of age with disseminated NSGCT were enrolled (May 2014 to May 2019) and stratified into four groups: two intermediate‐risk (IR: localised tumour with low tumour markers TM) groups treated with VBP (vinblastine–bleomycin–cisplatin): three courses for IR1 (ovarian tumour any age/testis tumour less than or equal to 10 years) and four courses for IR2 (extragonadal tumour 10 years or less) groups, and two high‐risk (HR: metastatic and/or high TM) groups treated with etoposide–cisplatin and either ifosfamide (VIP) or bleomycin (BEP): three courses for HR1 (ovarian tumour any age/testis tumour less than or equal to 10 years and low TM/testis tumour more than 10 years and very low TM) groups and four courses for HR2 (remainder) groups.
Results
One hundred fifteen patients were included: median age of 12.8 years (0.4–18.9); tumour sites: 44 ovaries, 37 testes and 34 extragonadal. The 5‐year EFS and overall survival (OS) were 87% (95% CI: 80–92) and 95% (89–98), respectively (median follow‐up: 3.5 years, range: 0.2–5.9), similar to those of the TGM95 protocol (5‐year EFS 89% (84–93), 5‐year OS 93% (89–95), p = .561). The 5‐year EFS were 93% (95% CI: 80–98), 88% (71–95) and 79% (62–90) for ovarian, testicular and extragonadal tumours, respectively. The 5‐year EFS varied (p = .02) according to the risk groups: 90% (66–97), 64% (30–85), 95% (72–99) and 87% (74–94) for IR1, IR2, HR1 and HR2, respectively. TM decline adjusted to tumour site, and alpha‐fetoprotein (AFP) level revealed a prognostic impact of time to normalisation on EFS: HR = 1.03 (1.003–1.007).
Conclusion
Risk‐adapted and globally decreased chemotherapy burden maintains excellent outcomes, exclusive of the IR2 group, which warrants more intensive chemotherapy.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Introduction
Pediatric adrenal cortical tumors are characterized by a wide spectrum of behavior. Questions remain regarding intermediate disease stages with isolated tumor rupture or relapse.
...Objectives
To describe clinical characteristics, treatment strategy, and outcome of patients depending on disease stage, tumor rupture, or in case of a refractory tumor, to discuss optimal management.
Material and methods
Pediatric patients with histological material reviewed and treated between 2000 and 2018 in 23 French oncology centers were included.
Results
Among 95 cases, 59% of patients had stage I tumors (n = 55), 16% had stage II tumors (n = 16), 19% had stage III tumors (n = 17), and 5% had stage IV tumors (n = 5) (missing data: 2). Overall, 27% of patients (n = 25) had an unfavorable histology. Initial tumor resection was performed for 90% of patients (n = 86). Systemic therapies included mitotane in 20 cases and chemotherapy in 13 cases. Among 17 stage III patients, 12 had microscopic residual tumor due to an initial biopsy (n = 5), intraoperative rupture (n = 8), or surgical resection with microscopic residue or tumor spillage surgery (n = 1) (two patients with two modalities). After a median follow‐up of 96 months (25‐119), four early progressions and two relapses occurred. A total of seven patients died, including five of disease. Stage III diseases due to microscopic residual disease correlated with a worse prognosis: 5‐year progression‐free survival 44% (95% CI, 22‐87%) versus 82% (95% CI, 73‐91%) for the whole cohort (P < .0001). Among the 14 patients with refractory disease, only 3 were alive and free of disease after multimodal second‐line therapy.
Conclusions
Stage III diseases due to a microscopic residual tumor have a dismal prognosis, arguing for the systematic use of adjuvant therapy. Patients with a relapsed disease should be included in experimental studies.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Background
Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10%–20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi‐ or ...contralateral ovaries. Ovarian‐sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed.
Design
This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. A national retrospective review of girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, second events and their management were retrieved.
Results
Overall, 350 children were included. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases, respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty‐nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: eight cases including one malignant tumor; contralateral: 18 cases; both ovaries: three cases). A large palpable mass, bilateral forms, at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas the type of surgery or approach did not.
Conclusion
This study is a plea in favor of OSS as the first‐choice treatment of OMT when possible. Close follow‐up during the first 5 years is mandatory considering the risk of 8.3% of second events, especially in cases with risk factors.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Purpose
To examine the clinical presentation, treatment and results in children and adolescents with primary mediastinal (PM) and retroperitoneal (RP) germ cell tumors (GCTs).
Methods
The TGM95 trial ...for malignant GCTs was conducted in France between 1995 and 2005 to evaluate a strategy adapted to prognostic factors with cisplatin‐based chemotherapy and surgical management. We reviewed patients with TGCTs at PM and RP sites.
Results
Among 239 patients, there were 16 patients with PM and 5 with RP tumors, which represent 9% of all patients, highlighting the rarity of these extragonadal locations. A bimodal demographic distribution was observed (11/21 patients <5 years old and 7/21 patients >12 years old). A majority of patients presented with bulky tumors that required urgent care with neoadjuvant chemotherapy. In all patients, elevation of alpha‐fetoprotein indicated a yolk sac tumor component. Human chorionic gonadotrophin was elevated in five patients (four adolescents), suggesting a choriocarcinoma or seminoma component. The diagnosis was based on elevation of these tumor markers in addition to imaging. Chemosensitivity was observed for a majority of patients. An aggressive surgical approach allowed a microscopic complete resection in 12/15 patients with PM tumors and 4/5 with RP tumors. Overall, 14/16 and 4/5 patients survived, respectively. Three adolescents died of tumor progression.
Conclusion
In children with mediastinal or RP GCTs, the prognosis is favorable when a strategy of delayed aggressive surgery is performed after cisplatin‐based chemotherapy. Younger patients have a better prognosis. Relapses were observed only in adolescents and could not be cured.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
This first observation of donor-transmitted coccidioidomycosis in a pediatric liver-transplant recipient underlines a rare condition in transplanted patients in a nonendemic area. This transmission ...was observed after a liver split, the patient being contaminated by the left liver while the right-liver recipient was not.
Abstract Objective Androgens have a positive effect on penile growth in children, but they may also have a repressive effect on the healing process. The aim of this prospective study was to compare ...the outcomes of onlay urethroplasty with and without preoperative androgen stimulation in patients with severe hypospadias. Patients and method Of 300 severe hypospadias cases treated at a single institution, 126 operated on by the same surgeon had complete follow-up data, and 30 of these received preoperative androgen treatment (human chorionic gonadotrophin and/or systemic testosterone) 1–24 months before surgery. Results Thirty-five patients presented with a complication (27.7%) of whom 26 (20.6%) had a fistula or dehiscence. Among patients on androgen stimulation there was a 30% healing complication rate (9/30) whereas for those without this was 17.7% (17/96). When androgenic treatment was given > 3 months prior to surgery the healing complication rate was 21.7% (5/23), and when < 3 months prior to surgery the rate reached 57% (4/7). Mean follow up was 41 months (10–97). Conclusion Although the numbers were too small in this series to reach statistical significance, the tissular interactions of androgens in the healing process reported by dermatologists should alert the hypospadiologists and lead to a further prospective study to define the optimal protocol for stimulation of the penis in specific cases without affecting outcome.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Abstract Objective To compare the outcomes of three different urethroplasty techniques (onlay, buccal mucosa, Koyanagi type I) used in the reconstruction of severe hypospadias. Patients and methods ...Over 10 years (1997–2007), 300 severe hypospadias cases were treated with a mean follow up of 2 years (1–105 months); 203 were operated by the same surgeon of whom 184 completed follow up. Three main techniques were used according to the quality of the urethral plate: onlay urethroplasty (133), buccal graft urethroplasty (25) and Koyanagi type I (26). The mean age at surgery was 36 months (8–298); 76 required preoperative androgen stimulation (onlay 37, buccal 11, Koyanagi 26); 18 required a corporoplasty to straighten the penis (onlay 13, buccal 3, Koyanagi 2). Results Thirty-eight onlay (28.5%); 14 buccal (56%); 16 Koyanagi (61.5%) urethroplasties had a complication. The fistula rate was 15% for the onlay group; 32% for the buccal mucosa group; 19.2% for the Koyanagi cases. The dehiscence rate was, respectively, 11.3%, 20% and 42.3%. The stricture rate was, respectively, 1.5%, 20% and 34.6%. Urethrocele was found in seven Koyanagi patients. Final functional and cosmetic results were satisfactory in 126/133 (94.7%) onlay, 20/25 (80%) buccal and 14/26 Koyanagi (53.8%) urethroplasties. Primary cases had better results (89%) than redo cases (75.9%). Patients submitted to preoperative androgen therapy developed more complications (onlay: 40.5% vs 23.9%; buccal: 70% vs 43.7%). Conclusion Two striking results are the low number of severe hypospadias cases requiring an additional corporoplasty, and the increased complication rate found in androgen-stimulated patients. The excellent results of the onlay procedure could be related to the use of dorsal preputial tissue, which in hypospadias is characterized by a well-balanced protein platform compared to the ventral tissues.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of laparoscopic splenectomy (LS) and can lead to bowel ischemia or portal hypertension. In childhood, this ...complication is reported in 5–10 % of the cases whereas it can be up to 50 % in adult population. Our aim was to evaluate PVT incidence after LS and associated risks factors. A retrospective chart review identified 37 children who underwent elective LS from 2005 to 2013. The main indications were spherocytosis or sickle cell disease. Median age and weight were respectively 7.4 years and 25.1 kg. Thromboembolic prophylaxis was not routinely given. Duration of surgery was 129 min and hospital length of stay 4 days. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases. Post-operative course was uneventful in all but one patient. She was a 17 year-old girl previously operated for an ovarian tumor with hyperandrogenism. Histopathology revealed a splenic lymphoma. At day 4, a systematic USS showed a PVT extending in the portal branches. Therapeutic low molecular weight heparin was used and then transitioned to fluindione for 3 months. Follow-up USS performed at 1 and 4 months demonstrated complete resolution of the PVT. PVT after pediatric LS is a rare event in our series. Clinician should be cautious in oncologic cases and if very large spleen or if thrombocythemia >650.10
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/L is present. If detected early, PVT can be treated efficiently. We therefore recommend a systematic USS during the first postoperative week.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
