SM Journal of Pediatric Surgery

Archive Articles

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Hepatopulmonary Fusion in a Newborn Diagnosed during Surgical Repair of Right Congenital Diaphragmatic Hernia

Hepatic Pulmonary Fusion (HPF) is a rare malformation in which adhesion between the liver and the right lower lobe of the lung occurs. This situation is frequently associated with right Congenital Diaphragmatic Hernia (CDH), and often only discovered during surgical repair of the defect.

Few cases have previously been reported in the literature [1]. In this paper we are reporting a case of HPF diagnosed fortunately in a tow days old female baby, at time of surgical repair of right side CDH and we discuss the modalities of diagnosis and the difficulties encountered during surgery.

Rechid Khemakhem¹*, Mohamed Raiz Elhaq Rao¹, Refeat Mohamed Sadek¹, Adel Metwali Fadhl¹, Majdi Gueessa², Mohamed Yousef², and Houda Rahay²


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Intrauterine Foreign Body in a Child

Foreign bodies in the genital tract are reported more commonly in women. Symptoms are not specific. In our report a child consulting for chronic pelvic pain, was diagnosed with intrauterine foreign body and managed successfully. Early diagnosis and emergent treatment may avoid serious complications. Removal under scope control is a safe therapeutic way.

Saloua Ammar¹*, Mohamed Jallouli¹, Ahmed Kotti¹, Hayet Zitouni¹, Mahdi Ben Dhaou¹, and Riadh Mhiri¹


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Apert Syndrome: Case Presentation

Apert syndrome belongs to acrocephalosyndactyly group of disorders, and it is a autosomal dominantly inherited rare disorder characterized by craniosynostosis, midline facial hypoplasia, severe symmetrical cutaneous syndactyly of the hands and the feet, as well as central nervous system, heart and kidney abnormalities. It is caused by a mutation of the fibroblast growth factor gene located in the 10th chromosome (10q25-26). Clinical presentation of the disease was first described in 1906 by Apert, and it is an easily recognizable disorder with particularly typical physical examination findings. In the light of the current literature, this article reviews and presents a case of Apert syndrome diagnosed based on physical examination findings and chromosomal analysis.

Atiye Fedakâr¹* and Zakir Sakc²


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Accessory Urethra, Accessory Phallus, and Accessory Scrotum: Varied Clinical Scenarios

Introduction: We presented 4 cases managed and operated with varied clinical scenarios; accessory urethra, accessory phallus and accessory scrotum, the study encountered these rare anomalies describing our findings for both anatomical and morphological variations.

Methods: All studied cases were operated in single pediatric surgery unit. All workup was done on basis of clinical presentation accordingly. All patients arranged for chromosomal karyotyping and evaluation of SRY and ATL1 gene loci on X and Y chromosome respectively using polymerase chain reaction amplification PCR. All sites excised sent for histopathology.

Results: Case 1: An 8-months old infant was evaluated for a history of purulent discharge from the opening of the sinus in the midline pre pubic area on the dorsal radix of the penis. Case 2: A 3- days old baby was presented with a mass in the gluteal region with bisexual ambiguity. On evaluation, both vaginal structure and welldeveloped phallus (small penis attached parasitic twin caudally) were present. Case 3: A 4-months old male infant presented with caudal mass (cystic perineal structure) grossly resembling testicle with well prominent phallus covering the anal opening. Case 4: A 2-year old male baby presented with a neighboring ipsilateral bulging peduncular perineal mass resembling phallus measuring 4.7 cm in length and 1.3 cm in width with narrow meatus and a history of drippling urine from this phallus like.

Conclusion: Crucial workup must be submitted including clinical, radiological, chromosomal karyotyping and cytogenic study to asses and resolve such scenarios

Mohammed Joudi Aboud¹*