Amyand's hernia in a 3-month-old infant: A case report.

Amyand's hernia is a rare condition characterized by an inguinal hernia containing the appendix, which can lead to complications. It is more common in children and it can be challenging to diagnose due to its location, often being mistaken for other conditions like strangulated hernias, orchitis-epididymitis, or testicular torsion. Imaging, including computed tomography and sonography, plays an important role in diagnosis, which is usually made intraoperatively. A case concerning a 3-month-old boy with a large acute scrotum that had been evolving for 4 days was presented. Clinical examination revealed a hard inguinal mass and a large scrotum with signs of inflammation. An inguinoscrotal ultrasound found a herniated appendix, suggesting the diagnosis of Amyand's hernia, which was confirmed after surgery.

Primary hydatid cyst of the pancreas of the child: a case report.

Primary pancreatic hydatid lesions are very rare with an incidence of less than 1% in the adult population. We report an observation of a 5-year-old girl who consulted for isolated abdominal pain occurring for 2 weeks without vomiting, transit disorders or jaundice and evolving in a context of conservation of the general condition and apyrexia. Clinical examination and preoperative imaging have suggested the diagnosis of a choledochal cyst or duodenal duplication rather than a hydatid cyst of the pancreas due to the presence of a cystic hepatic image projecting into the liver hilum. During the procedure, a hydatid cyst was found occupying the head of the pancreas. Primary hydatidosis of the pancreas in children is extremely rare. Possible sources of infection include: blood diffusion, local spread via biliopancreatic ducts and peripancreatic lymphatic invasion. In the endemic areas, hydatid disease should be mentioned in the list of differential diagnoses of cystic lesions located around the biliopancreatic junction in children.

Closed reduction of a traumatic hip dislocation in children: case report.

Traumatic dislocation of the hip in children is a rare disease. It only represents 5% of hip dislocations in all age groups. Before 10 years, the mechanism is often a minimal domestic accident; after 10 years, the dislocation occurs with the waning of an accident of the public highway. It is different from that of the adult by its rarity, its ease of reduction and better prognosis. This is an emergency trauma: risk necrosis of the femoral head (If delayed reduction). We report a rare case of a 3 year old boy, who suffered from bipolar trauma after a fall near his height of his house causing him a detachment of the right humerus and post-traumatic dislocation of the left hip. The diagnosis was clinically confirmed by the results of standard radiographs and CT scans of the pelvis. The consultation period to emergencies was 5 hours after the trauma. We performed an hour after a closed reduction under general anesthesia for hip dislocation with establishment of a splint pelvic-pedal for analgesic keep for three weeks. The radiological outcome was satisfactory. Peeling Salter I humerus was reduced by orthopedic manner and immobilized by thoracoabdominal plaster to keep for a month. The child was discharged the next day. Reviewed in consultation after a month, the clinical examination showed a steady left hip. Traumatic dislocation of the hip in children is a rare diagnosis, the management should as urgent as possible to overcome the different possible subsequent complications dominated by coxa magna.

[Bean's syndrome in children: about two cases]. / Syndrome de Bean chez l'enfant: à propos de deux cas.

Diffuse angiomatosis or Bean's syndrome is a rare disease characterized by venous malformations mainly involving the skin and the digestive tract which can result in hemorrhage of variable severity. This study reports the case of two children aged 5 and 9 and a half years respectively with diffuse angiomatosis who had been treated in the Department of Emergency Paediatric Surgery over the years. The diagnosis was based on rectal bleeding and/or melenas causing severe anemia requiring regular transfusions in both patients as well as skin angiomas occurrence at the level of the limbs. Radiological evaluation showed the presence of multiple lesions at the level of the jejunum and ileum consistent with small intestinal angiomatosis in the child aged 9 and a half years. It didn't show abdominal lesions in the child aged 5 years. The two patients were admitted to the operating block. Angiomas were surgically individualized. Some of them were actively bleeding. Enterotomy was performed. Postoperative sequelae was marked by the stop of the bleedings. This study aims to update the current understanding of this rare pathology as well as the benefit of surgical treatment in controlling the complications caused by this pathology and in reducing the frequency of transfusions.

A novel mutation in the endothelin B receptor gene in a moroccan family with shah-waardenburg syndrome.

Waardenburg syndrome (WS) is a neurocristopathy disorder combining sensorineural deafness and pigmentary abnormalities. The presence of additional signs defines the 4 subtypes. WS type IV, also called Shah-Waardenburg syndrome (SWS), is characterized by the association with congenital aganglionic megacolon (Hirschsprung disease). To date, 3 causative genes have been related to this congenital disorder. Mutations in the EDNRB and EDN3 genes are responsible for the autosomal recessive form of SWS, whereas SOX10 mutations are inherited in an autosomal dominant manner. We report here the case of a 3-month-old Morrocan girl with WS type IV, born to consanguineous parents. The patient had 3 cousins who died in infancy with the same symptoms. Molecular analysis by Sanger sequencing revealed the presence of a novel homozygous missense mutation c.1133A>G (p.Asn378Ser) in the EDNRB gene. The proband's parents as well as the parents of the deceased cousins are heterozygous carriers of this likely pathogenic mutation. This molecular diagnosis allows us to provide genetic counseling to the family and eventually propose prenatal diagnosis to prevent recurrence of the disease in subsequent pregnancies.