Management of Priapism in Sickle Cell Patients: Experience of the Urology Department of the University Hospital Centre of Libreville

Introduction: Priapism is a rare pathology, known since antiquity. Sickle cell disease is the main aetiology in Africa. The aim of our work was to report our experience in its treatment. Material and Methods: This is a prospective, descriptive study carried out at the urology department of the Libreville University Hospital from January 2018 to December 2020. All sickle cell patients admitted to urology for priapism were included. The parameters studied were socio-demographic, clinical and therapeutic parameters as well as the evolution after treatment. Result: We collected 19 priapisms in sickle cell patients. The average age was 20.9 years with extremes of 4 and 53 years. Fifteen patients were homozygous SS. All patients had stasis priapism. The average consultation time was 22.4 hours. All patients had perioperative medical management combining hyperhydration, analgesia and antibiotic prophylaxis. A vasoactive drug was administered to 13 patients. Sixteen patients had a puncture of the corpus cavernosum. A distal cavernosal-spongiosum shunt under penile block was performed in 6 patients. The outcome was favorable from the outset in 12 cases, marked by complete detumescence of the corpus cavernosum. Partial detumescence was noted in 7 patients with the need for a new puncture of the cavernous body. A complication such as edema of the penis was in only one of our patients. A recurrence was noted in 2 patients. After an average follow-up of 6 months, no sequelae erectile dysfunction was observed. Conclusion: Priapism is a frequent complication among sickle cell patients in Libreville. Medical management associated with a cavernous puncture with administration of vasoactive drugs allows a favourable evolution without after-effects.

Laparoscopic Orchidopexy after 4 Weeks of Testicular Traction with Preservation of Blood Supply: A New Concept for the Treatment of Intra-Abdominal Cryptorchidism: Original Article

Background: Testicular atrophy is recurrent after orchidopexy for intra-abdominal cryptorchidism due to damage to vessels. Fowlers-Stephens and Shehata methods proposed staged orchidopexy, but are still associated to a rate of atrophy greater than 10%. Purpose: We set out to present a new technique that preserves testicular vessels. Our study is aimed at investigating testicular vitality after surgery. Patients and Method: We reviewed files of boys who underwent orchidopexy for 2 years in our department. Those who have been operated for intra-abdominal cryptorchidism with the new technique and aged between 6 months and 14 years old were included in our study. We excluded boys who have been operated before 6 months and after 14 years old and those who did not respect their follow-up plane. Ultrasound (US) outcomes were used to measure testicular volumes and blood flow at each postoperative visit. The Sample population was divided into group A and group B representing testes that were followed respectively for 12 and 24 months. Results: 22 boys with 25 testes were included in our study;11 testes in group A and 14 testes in group B. In each group, we noted a significant difference of UDT volumes from the third month after surgery with respective P-values 0.05. There was no statistical difference between UDT and contra lateral testes volumes 12 months after surgery in group A and 24 months in group B with respective P-values of 0.07 and 0.72. All volume differential indexes were Conclusion: This method offers a new perspective in performing safe orchidopexy for intra abdominal undescended testes.

Large Conventional Osteosarcoma of the Proximal Humerus in a 13-Year-Old Child: Case Report

Introduction: Osteosarcoma is the most common primary malignant bone tumor in children. It is highly aggressive and has a poor prognosis. A late presentation modifies and makes difficult the management affecting the survival of children. We report the case of a large conventional osteosarcoma in a 13-year-old girl. Case Presentation: Adolescent girl admitted for painful swelling of the left shoulder with absolute functional impotence of the thoracic limb and severe anemia. The painful swelling was thought to have been caused by a minor trauma that had occurred six months previously. The patient’s general condition was poor, and she presented with a large, shiny, painful mass over the shoulder and upper 2/3 of the left arm, measuring 28 cm long by 28 cm wide and 57 cm in circumference, and a large fistulous axillary adenopathy. CT scan showed a tumour lesion of the left humerus with liver and lung metastases, raising suspicion of osteogenic osteosarcoma. The tumor was classified according to TNM staging: T2N1M1(a + b). Management was modified when uncontrolled bleeding developed. It consisted of an extended amputation of the left thoracic limb. Pathological analysis showed a high-grade conventional osteosarcoma. Quality improvement was obtained for thirty days, followed by the onset of dyspnea. The evolution was towards death at forty days post-operatively. Conclusion: Osteosarcoma is a highly aggressive cancer. Delayed treatment leads to a fatal outcome. Early diagnosis is one of the challenges to be met in order to improve survival.

The ‘Unsigned highway’:An alternative route for portal vein anastomosis for non-malignant portal vein thrombosis during pediatric re-transplantation

To the Editor:Non-malignant portal vein thrombosis(PVT)remains an important issue in liver transplantation due to the technical challenges in re-establishing the portal flow to the liver graft.The prevalence of complex PVT(i.e.Yerdel grade 4)was reported to be around 2.0%[1].In the early history of liver transplantation,PVT was regarded as a contraindication.

Late Presentation of a Congenital Intrinsic Duodenal Obstruction in a Patient with Anorectal Malformation

Anorectal Malformations are known to be associated with various other congenital anomalies including duodenal atresia. An association of congenital intrinsic duodenal obstruction causing partial duodenal obstruction in a patient with anorectal malformation is not described in literature. We describe a case of delayed presentation of congenital intrinsic duodenal obstruction in a child with high-type anorectal malformations causing diagnostic dilemma.

Congenital Cystic Adenomatoid Malformation: A Case Report with Clinical, Radiological, Histological, and Surgical Features

Background: Congenital cystic adenomatoid malformation (CCAM) is a congenital anomaly of lung development, accounting for approximately 25% of congenital lung lesions. Respiratory distress often occurs during the neonatal period, and in 80% to 85% of cases, the diagnosis is made before the age of 2 years following respiratory infections. Case Report: We report a case of MAKC diagnosed in the neonatal period. The diagnosis was based on clinical, radiological and histological elements. Our patient underwent surgical resection. Histological examination confirmed the diagnosis of MAKC without any sign of malignancy. The postoperative evolution was good. Conclusion: Clinicians and pathologists should recognize the early discovery of MAKC in neonatal age. The clinical diagnosis strongly guided by the radiological approach is confirmed by the pathological anatomy insofar as the therapeutic sanction is surgical in the majority of the cases.

食管闭锁和失弛缓样食管运动障碍

A 14-year-old boy presented with regurgitation, malnutrition, and chronic lung insufficiency with a history of successful repair of esophageal atresia and tracheoesophageal fistula in the newborn period. Barium swallow and manometry results showed achalasia. Hellar operation with antireflux procedure resulted in complete symptomatic relief. Histology and immunohistochemistry including PGP9.5, MAP5, cKit, and nNOS of myotomy specimen showed intact innervation. Although esophageal dysmotility after esophageal atresia repair usually is caused by gastroesophageal reflux and incoordination of peristalsis, the possibility of achalasia should also be considered, because a casual relationship between esophageal atresia and achalasia may exist.

Complicated Left-Sided Amyand’s Hernia in an 18-Month-Old Boy: A Case Report and Literature Review

The rare finding of the vermiform appendix within an inguinal hernia sac is known as Amyand’s hernia. It was first described by Claudius Amyand in 1735, in a right inguinal hernia. A much rarer find is a left-sided Amyand’s hernia. This is a report of a case of complicated left-sided Amyand’s hernia in an eighteen month old male child. He presented as an emergency with an obstructed inguino-scrotal hernia and the diagnosis of Amyand’s hernia was made intra-operatively. He made uneventful recovery after surgery. Treatment options depend on findings during operation and clinical status of the patient.

Perspectives on perioperative management of children’s surgical conditions during the COVID-19 pandemic in low-income and middle-income countries:a global survey

Background Many organizations have issued recommendations to limit elective surgery during the coronavirus disease 2019(COVID-19)pandemic.We surveyed providers of children’s surgical care working in low-income and middle-income countries(LMICs)to understand their perspectives on surgical management in the wake of the COVID-19 pandemic and how they were subsequently modifying their surgical care practices.Methods A survey of children’s surgery providers in LMICs was performed.Respondents reported how their perioperative practice had changed in response to COVID-19.They were also presented with 26 specific procedures and asked which of these procedures they were allowed to perform and which they felt they should be allowed to perform.Changes in surgical practice reported by respondents were analyzed thematically.Results A total of 132 responses were obtained from 120 unique institutions across 30 LMICs.117/120 institutions(97.5%)had issued formal guidance on delaying or limiting elective children’s surgical procedures.Facilities in LICs were less likely to have issued guidance on elective surgery compared with middle-income facilities(82%in LICs vs 99%in lower middle-income countries and 100%in upper middle-income countries,p=0.036).Although 122(97%)providers believed cases should be limited during a global pandemic,there was no procedure where more than 61%of providers agreed cases should be delayed or canceled.Conclusions There is little consensus on which procedures should be limited or delayed among LMIC providers.Expansion of testing capacity and local,context-specific guidelines may be a better strategy than international consensus,given the disparities in availability of preoperative testing and the lack of consensus towards which procedures should be delayed.