Fatih Andiran

Chest wall hamartoma: An alarming chest lesion with a benign course

Chest wall hamartoma is a very rare mass lesion of the chest wall that is manifest at birth or in early infancy with deformity of the thoracic wall and/or varying grades of respiratory distress. The authors report on an 11-month-old infant presenting with chest wall hamartoma who was treated by en bloc excision of the lesion including the involved ribs. They emphasize the importance of early and complete excision of the lesion even in minimally symptomatic patients to avoid lethal respiratory complications, more severe postoperative orthopedic problems, and malignant transformation.

SYSTEMIC HARMFUL EFFECTS OF INGESTION OF HOUSEHOLD BLEACHES

Household bleaches that contain sodium hypochlorite and sodium hydroxide are the common domestic agents used all around the world, which are frequently accidently ingested by children. Commercial household bleaches are known to have minor adverse effects when ingested, as being mucosal irritants, emetics and weak corrosives. However, our clinical observations of pneumonitis among household bleach ingested by patients without a history of aspiration foretell some possible systemic toxic effects of household bleaches. An experimental study on rats was designed to evaluate systemic effects of household bleaches that contain 4% sodium hypochlorite and less than 0.05% sodium hydroxide on lungs, livers, kidneys and intestines after 2, 4, 6, 12, 24 and 48 hours of administration via intragastric route. Prominent congestion and some interstitial mononuclear cellular infiltration were observed in the lungs, the livers and the kidneys of the rats after administration of household bleaches. Additionally, the lungs showed expansion of the alveolar spaces. While erosive changes were present in the stomachs, the intestines were normal. These histopathological changes were especially prominent at early periods of systemic administration. In the second part of the study, to assess whether these findings would hold for intravenous administration of household bleaches, another group of rats were given intravenous administration of household bleach and after 4 hours of intravenous administration of household bleach, the same histopathological changes above were observed in the lungs, kidneys and livers. The study indicates that household bleaches may have systemic effects prominent during early periods of ingestion, although the clinical importance remains to be clarified.

Duplicate exstrophy of the bladder

Duplicate exstrophy of the bladder is a rare, distinct congenital anomaly without additional major anomalies of the urinary tract. A 15-year-old boy with duplicate exstrophy is reported on, and the possible role of bladder sequestration in the development of classical musculoskeletal deformities in this anomaly is discussed.

Surgical Treatment of Childhood Inflammatory Myofibroblastic Tumors.

Aim A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives of childhood inflammatory myofibroblastic tumors (IMTs). Patients and Methods Patients who underwent surgical treatment for IMT between 2000 and 2015 were evaluated for age, sex, presenting symptoms, physical examination findings, diagnostic methods, treatment modalities, histopathologic findings, and results of surgical treatment during long‐term follow‐up. Results Eleven patients who underwent surgical treatment were included in the study. Male:female ratio was 7:4 and the mean age of the patients was 6.09 years (1‐10 years). Presenting symptoms were respiratory difficulty, cough (n = 7, 63.3%), abdominal pain, vomiting (n = 2, 18.8%), loss of body weight (n = 1, 9.09%), palpable mass (n = 1, 9.09%), and rectal bleeding (n = 1, 9.09%). Ultrasonography (n = 4, 36.3%) and computed tomography (n = 9, 81.1%) were used for diagnosis. Localizations of tumors were lungs (n = 5, 45.4%), mediastinum (n = 2, 18.1%), spleen (n = 1, 9.09%), neck (n = 1, 9.09%), colon (n = 1, 9.09%), and rectum (n = 1, 9.09%). The mean size of mass was 6.6 cm (2‐12 cm) and six patients were diagnosed with preoperative biopsy. Lung lobectomy (right lower lobe; n = 3, right middle and lower lobe; n = 2), total resection of mass with adjacent bowel (n = 2), partial splenectomy (n = 1), total resection of neck mass (n = 1), and incomplete resection (n = 2) were the choice of surgical treatment. Incomplete resection was performed in masses closely adjacent to atrium and mediastinal structures. In histopathologic evaluation, surgical margins were free of tumor in four cases, positive in six cases, and were not reported in one case. Anaplastic lymphoma kinase (ALK) positivity was detected in six cases, negative in two cases, and was not evaluated in three cases. Two cases who had residual mass with positive ALK received chemotherapy. Mean follow‐up time was 68.2 months (5 months to 12 years). During follow‐up, there was no recurrence or distant metastasis. Ten patients survived and one patient was lost to follow‐up. Conclusion IMT is a rare tumor of childhood with a spectrum of clinical findings because of variable localization. Surgical treatment is the first choice of treatment. Patients with residual mass and ALK positivity may require medical treatment. In our series, long‐term survival of patients was favorable in patients with total resection.

Rare cause of recurrent urticaria in childhood and its unusual presentation: Solitary mastocytoma on vulva.

Dear Editor, The underlying cause of recurrent urticaria cannot be identified in some patients despite intensive investigation. Solitary mastocytoma is a rare cutaneous mastocytosis characterized by mast cell hyperplasia limited to a particular area on skin. It frequently appears as a solitary mass lesion but less commonly as a macule, plaque or nodule. Mastocytomas may involve any region of the body surface. They are usually asymptomatic, but pruritus, urticaria and flushing may occur with triggers such as trauma or certain drugs leading to mast cell degranulation. The diagnosis is based on clinical appearance of the lesion and pathological findings. Here, we report a child with recurrent urticaria who was consequently diagnosed with solitary mastocytoma on the vulva. An otherwise healthy, 9-year-old girl with recurrent urticaria for five episodes since the previous year was admitted. Her urticarial episodes were always starting with itching in the genital area and then generalized to the body, lasting for 10–30 min. She had never had an anaphylactic reaction before. There was no family history of allergic diseases. Her physical examination was unremarkable except for a 2 cm 9 1 cm, slightly raised, solid lesion on the vulva with positive Darier’s sign (Fig. 1a–b). Laboratory evaluation including complete blood count and basal tryptase levels were within normal limits. Skin prick tests to common allergens were negative. Abdominal ultrasonography was normal. Excisional biopsy of the solid lesion revealed numerous mast cells with round, oval nuclei and large, pale eosinophilic granular cytoplasm in the papillary and reticular dermis (Fig. 1c). Metachromatic staining was observed in cytoplasmic granules with Giemsa and Toluidine blue stains (Fig. 1d). The diagnosis of solitary mastocytoma

Results of intraoperative gamma probe survey and frozen section in surgical treatment of parathyroid adenoma in children

Abstract Aim To evaluate the use of intraoperative gamma probe survey (IGPS) and frozen section (FS) in the surgical treatment of parathyroid adenoma (PA) in children. Patients and methods Children operated for PA between 2000 and 2015 were evaluated for age, sex, clinical features, presenting symptoms, diagnostic methods and results of surgical treatment retrospectively. Results Seven patients operated for PA were included in the study. The mean age of the patients was 14.58years (11–17years) and male to female ratio was 4:3. Presenting symptoms were non-specific such as fatigue, weakness and abdominal pain ( n =4). Six patients (85.8%) had adenoma of single parathyroid gland whereas remaining patient has involvement of two glands (14.2%). Localizations of adenomas were left inferior ( n =3, 42.8%), right inferior ( n =2, 28.3%), right superior ( n =1, 14.2%), left superior ( n =1, 14.2%) and intrathymic ( n =1, 14.2%). In case with involvement of more than one gland, adenomas were localized at right superior/left inferior glands. The mean longest diameter of the adenomas were 14.8mm (4–23mm) and all of the cases were underwent USG and Tc-99m sestamibi scan. During surgical excision, FS were used in 5 cases, and 4 cases had IGPS after preoperative radioactive substance injection. In one patient, injection was performed directly into the adenoma and 3 patients had systemic injection. Patient who had injection directly into the involved gland had positive gamma counts in the adjacent tissues because of local spread of radioactive substance and diagnosis was also confirmed with FS. In two of the cases, adenoma excision was performed without FS with only IGPS. None of the patients had preoperative and postoperative complications. Conclusion PA is rare in children and appropriate gland excision can be obtained also by the help of IGPS after injection of radioactive substance, and frozen section examination as well as intraoperative parathormone (PTH) screening. IGPS is a helpful tool to define the target tissue during surgery in order to avoid extensive dissection. Since direct injection of involved gland may cause accumulation of radioactivity in the adjacent tissues, surgeons must be aware of advantages and disadvantages of different radiolabeling techniques.