Background
Mesenchymal hamartoma is the second most common benign liver tumor in children, with 20% of the cases diagnosed during the neonatal period. The exact etiology in still unclear, and most investigators believe that it is a developmental anomaly rather than a true neoplasm. The presentation of these tumors is highly variable depending on the lesion’s size, ranging from small asymptomatic lesions to very large tumors with life threatening complications. Radical surgical excision, whenever possible, is the gold standard for treating these lesions to avoid the problems of local recurrence and possible malignant transformation. We present the rare occurrence of an entirely solid, giant hepatic mesenchymal hamartoma in a 3-week-old male newborn and discuss the mode of presentation, as well as the diagnostic and therapeutic approach.
Case presentation
A 3-week-old male newborn was referred to our institution with huge abdominal distension and respiratory distress. Imaging studies confirmed the presence of a very large solid intraabdominal mass occupying the majority of the abdominal cavity and abutting the inferior aspect of the right lobe of the liver, but did not reveal the diagnosis. At laparotomy, a huge solid mass was found attached to the right lobe of the liver. Complete excision was done, and histopathological examination confirmed the diagnosis of mesenchymal hamartoma.
Conclusion
Although rare, mesenchymal hamartoma of the liver can present as a neonatal surgical emergency. Emergency intervention is required in symptomatic patients. Radical surgical intervention is possible and is the treatment of choice to relieve the patient’s symptoms and avoid future complications.
Background: Lymphatic malformations are rare benign cystic tumors that result from localized disordered embryologic development of the lymphatic system and can develop anywhere in the body, predominantly in the head and neck. These lesions are classified according to the diameter of the largest cystic cavity within the lesion into microcystic and macrocystic types. Historically, surgical excision has been considered the mainstay of treatment and still remains the first therapeutic option of choice for many surgeons particularly for giant macrocystic lesions. Several alternative therapeutic modalities emerged including intralesional sclerotherapy and laser therapy with encouraging results. The study is designed to assess the effectiveness and safety of surgical excision as an initial therapeutic option in the management of these malformations. Results: Asymptomatic mass with parental cosmetic and functional concerns was the mode of presentation in six (66.7%) patients. Seven (77.8%) patients were presented before the age of 2 years. Six (66.7%) of the patients had their lesions in the neck. Complete surgical excision was achieved in eight (88.9%) patients without any evident significant injury to vital neurovascular structures. None of the patients had any difficulties with breathing, swallowing, or phonation and cosmetic results were satisfactory in the majority (88.9%) of them. Conclusion: Radical surgical excision of giant macrocystic lymphatic malformations in children is possible in experienced hands. It is an effective and safe initial therapeutic option and gives satisfactory esthetic and functional results.
Background: Sirenomelia is a rare congenital malformation characterized by fusion of the lower limbs giving a characteristic mermaid-like appearance to the affected fetus. It is commonly associated with gastrointestinal, genitourinary, cardiovascular and musculoskeletal system defects. Objective : To report the clinical manifestations of an extremely rare and complex malformation along with the associated anomalies. Case report: A case of sirenomelia was reported in a one of a set of twin delivered at term by caesarean section to a 39 year old Iraqi mother. According to the search that has been done, it seems that this is the first reported case in this country. The following associated anomalies (imperforate anus, absence of external genitourinary orifices, esophageal atresia with tracheo-esophageal fistula, intestinal atresia and bilateral renal agenesis) were observed. Conclusion: Sirenomelia is a rare and fatal congenital malformation. Still there is some controversy regarding its etiology, however there is an increasing belief that this complex malformation is distinct from the caudal regression syndrome. Survival depends on the presence of normal renal function
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