Complete Resolution of Retroperitoneal Lymphangioma with a Single Trial of OK-432 in an Infant.

Retroperitoneal lymphangioma is extremely rare. Although these neoplasms are benign, they can grow progressively with subsequent compression and infiltration of the adjacent structures. Surgical excision is demanding when the lesion surrounds vital structures and it is generally fraught with a high recurrence and morbidity rate. We report the case of a huge retroperitoneal lymphangioma in a newborn treated successfully with intracystic injection of OK-432.

Endoscopic retrieval of a duodenal perforating teaspoon.

Foreign objects ingestion occur commonly in pediatric patients. The majority of ingested foreign bodies pass spontaneously the gastrointestinal tract and surgery is rarely required for extraction. Endoscopic removal of foreign bodies larger than 10 cm has not yet been described. We present the case of a 16 years old bulimic girl that swallowed a 12 cm long teaspoon in order to provoke vomiting. The teaspoon perforated the duodenum. However, it was removed during gastroscopy and the site of perforation was closed endoscopically. This particular case shows the importance of endoscopy for retrieval of large foreign bodies, and the possibility to endoscopically close a perforated duodenal wall.

Extrinsic colonic obstruction by congenital fibrous band in an infant.

The finding of a congenital fibrous band during laparotomy for intestinal obstruction is extremely rare. Preoperative diagnosis is challenging and no characteristic radiological findings have been described. We report the case of a premature baby in whom incomplete intestinal obstruction was due to a congenital band originating from the duodeno-jejunal flexure and extending across the ascending colon.

Successful management of congenital chylous ascites with early octreotide and total parenteral nutrition in a newborn.

Congenital chylous ascites (CCA) is a rare disease that results from maldevelopment of the intra-abdominal lymphatic system. Few cases have been described and no gold standard treatment has been defined so far. Octreotide, a somatostatin analogue, has been used for the treatment of CCA, but always after a failed conservative approach with fasting, total parenteral nutrition (TPN) or medium chain triglyceride (MCT) feeds. We report the case of a newborn with CCA treated by fasting, TPN and octreotide for a period of 15 days until the abdominal distension was successfully reduced at which point treatment was switched to an MCT formula. On day 25 the patient was breastfed and was discharged on day 33. No recurrence of chylous ascites was noted. Our experience highlights the successful treatment with TPN and octreotide as the first step for the conservative approach of CCA in a newborn, reducing the length of treatment and hospitalisation.

Intradiaphragmatic hybrid lesion in an infant: case report.

Hybrid lesions have elements of both congenital cystic adenomatoid malformation and bronchopulmonary sequestration. We report an unusual case of an infant treated for an intradiaphragmatic hybrid lesion. Although computed tomography remains the criterion standard imaging examination for planning the operation, the exact localization of such lesions may be discovered only at surgical exploration.

Cribriform-morular variant of papillary thyroid carcinoma in an 8-year-old girl: a case report with immunohistochemical and molecular testing.

The description of the histological features and the immunohistochemical and molecular analyses of a case of cribriform-morular variant of papillary thyroid carcinoma in an 8-year-old girl with a family history of adenomatous polyposis is presented. The neoplasm was multifocal and bilateral, with a mixed pattern of solid, trabecular, and morular areas. The neoplasm showed angioinvasive behavior, extracapsular infiltration with extension to the perythyroidal muscles, and lymph node metastases. Tumor cells were positive for CAM 5.2, cytokeratins 5/6, TTF-1, HBME-1, galectin-3, and ß-catenin. In addition, the molecular tests did not reveal BRAF mutations, RET/PTC rearrangement, APC mutation, or KRAS mutation.

Invasive fetal therapies: approach and results in treating fetal ovarian cysts.

OBJECTIVE: To show the validity of prenatal invasive surgical intervention when a fetal ovarian cyst is diagnosed, compared to a wait and see attitude, in order to avoid possible prenatal and postnatal complications. PATIENTS: Fourteen cases of intra-abdominal cysts monitored in our center between April 2005 and November 2010. All cases were first diagnosed in the third trimester, and were monitored for the remainder of the pregnancy and after delivery (2 months-3 years postnatally). SURGICAL INTERVENTION: Upon maternal and fetal cutaneous anesthesia performed trans-amniotically, the cystic fluid (mean contents 43.85 cc, DS 46.27) was extracted for cytological, biochemical, and hormonal examination. RESULTS: Thirteen cases of intra-abdominal cysts (92.8%) were fetal ovarian cysts. Ninety-two percent of pregnancies bearing such a condition were successfully concluded (n = 12). Sixty-nine percent concluded in vaginal delivery (n = 9). None experienced maternal and/or fetal complications. Every drained cyst had an estradiol concentration higher than 10,000 pg/ml. CONCLUSIONS: The aspiration of ovarian cysts exceeding a 40 mm diameter, performed as early as possible, allows a good longitudinal treatment of this fetal affection, thus avoiding torsion, tissue necrosis, and invasive postnatal surgery, as well as giving hope of future gestational capability to the fetus/newborn.

Solitary Peutz-Jeghers Polyp in a Paediatric Patient.

Hamartomatous polyps of Peutz-Jeghers are mostly found in patients affected by Peutz-Jeghers syndrome (PJS), but they can be rarely encountered in the general population. It is unclear whether a solitary Peutz-Jeghers polyp (PJP) is an incomplete form of PJS or a separate entity. We report a case of solitary PJP in a paediatric patient in whom the other features of PJS were absent. The patient underwent laparotomy due to small bowel intussusception secondary to an ileac polyp. Histological examination showed the characteristic features of PJP, but the patient did not fulfill the WHO criteria for PJS diagnosis (negative family history for PJS and absence of mucocutaneous pigmentation); moreover analysis of the STK11/LKB1 gene did not reveal any genomic abnormality. The clinical and investigative findings in our case suggest that the solitary PJP can be considered a different clinical entity from PJS.

Peculiar type 1 congenital pyloric atresia: a case report.

Pyloric atresia (PA) is a very rare condition. Its incidence is approximately 1 in 100,000 newborns and constitutes about 1% of all intestinal atresias. We describe the neonatal course of a peculiar case of type 1 pyloric atresia, in which the pyloric membrane was connected to a second duodenal membrane through a virtual duodenal lumen in a premature newborn. The atypical variant required an unusual side to side gastroduodenostomy. We emphasize the importance of a prompt diagnosis to avoid potentially fatal complications and to warrant a good outcome even in the presence of a strange form of PA in the neonatal period.

Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2.

Schwannoma is a benign neoplasia of the peripheral nerve sheath. Its localization in the gastrointestinal tract, and in particular in the esophagus, is very rare. According to the existing literature esophageal schwannoma has been reported so far only in adult patients. We report the case of an 11 year old patient with neurofibromatosis, type 2, who underwent surgical excision of a plexiform schwannoma of the esophagus.

[Esophageal atresia. Personal experience and review of the literature]. / Atresia esofagea: nostra esperienza e revisione della letteratura.

INTRODUCTION: A long gap between the esophageal pouches in esophageal atresia is a relatively rare finding; it adds a significant challenge in the surgical correction and final outcome. MATERIAL AND METHODS: We retrospectively reviewed all cases of long gap esophageal atresia managed at our institution between 2000 and 2006, focusing on antenatal period, delivery weight, maurity, associated malformations, initial management and definitive repair. RESULTS: Sixteen patients with esophageal atresia were observed. Five of them (31%) presented a long gap. Delayed reconstruction was achieved in 4 children at an average age of 63 days (range 40-95 days). Primary repair was possible at birth in one case. All children had a direct anastomosis with one or more Livaditis myotomies in four cases. DISCUSSION: Surgical management of long gap esophageal atresia remains controversial. Most authors believe that elongation of the native esophagus provides a better functional outcome. In particular conditions, when this goal cannot be achieved, esophageal substitution is the last resort. Many techniques have been proposed to obtain esophageal elongation; although all the procedures give acceptable results, none of them has been unanimously accepted by pediatric surgeons. The circular or spiral myotomy is still the most commonly used technique to lengthen the esophagus in the repair of long gap atresias. CONCLUSIONS: In our limited experience circular myotomy (single or double) has proven an effective and reliable technique which allows direct repair of the esophagus even in the presence of initial gaps of 4 vertebral bodies.

Complete epididymal separation presenting as polyorchidism.

We describe an unusual case of a 21-month-old boy with complete separation of the testis and epididymis. Two homogeneous structures were observed in the scrotum of a newborn boy in addition to a third structure. Polyorchidism was suspected, but follow-up sonographic studies showed a decrease in the echogenicity of the left scrotal structure. Surgical exploration revealed the testis and epididymis to be completely separated, with no duplicated testis. Orchidopexy was then performed.

Evaluation of satisfaction level by parents and children following pediatric anesthesia.

BACKGROUND: Research in the field of patient satisfaction first took place in the 1980s in the USA, and later in Europe, aimed at meeting competitive requirements in the field of health care. Very few studies of this type exist in regard to pediatric anesthesia. Our aim was to develop a rapidly interpretable questionnaire to measure the level of parental satisfaction when their children undergo surgery and provide information on those factors triggering anxiety in these children. METHODS: Over a period of 18 months 179 parents of children in pediatric surgery responded to 214 questionnaires designed to investigate principally the emotional/behavioral spheres as well as the comfort provided. RESULTS AND CONCLUSIONS: We found that parents generally expressed a high degree of satisfaction which was good and correlated significantly with environmental comfort and postoperative observations performed by anesthetists and nursing staff. In the children, lack of fear at the moment of being anesthetized, and lack of anxiety on the day preceding surgery, were attributed to the serenity transmitted by the anesthetist and nurses. Significant anxiety resulted from the fear of an unpleasant impact with the operating room.

Cystic dysplasia of the rete testis associated to cryptorchidism: a case report.

A case of cystic dysplasia of the rete testis (CDRT) is reported. Nine months after right orchidopexy, a 10-year-old boy presented a palpable mass in the ipsilateral hemi-scrotum. Preoperative ultrasound showed a cystic lesion involving almost the whole testis. Radical orchidectomy was performed and at gross examination a hilar multiloculated cystic lesion was confirmed; microscopic examination showed cystic spaces lined by benign appearing monostratified epithelium with atrophy of the surrounding testicular parenchyma. These findings were consistent with the diagnosis of cystic dysplasia of the rete testis. Immunohistochemical investigation evidenced a pattern similar to that of a normal rete testis. Renal ultrasound and DTPA scintigraphy performed after pathological diagnosis documented the absence of the ipsilateral kidney. CDRT is a rare cause of scrotal swelling in the pediatric patient. When diagnosis is suspected, based on clinical and sonographic findings, conservative surgery may be considered.

Laparoscopic approach to müllerian derivatives in a male with mixed gonadal dysgenesis.

Müllerian derivatives are a frequent finding in patients with external genital ambiguity. In cases in which their removal is indicated, traditional surgical approaches are both invasive and associated with risks. We report a case of mixed gonadal dysgenesis in which a large prostatic utricle was successfully removed via laparoscopy.

Sacrococcygeal teratoma. Outcome and management. An analysis of 17 cases.

AIMS: The authors compare their experience of 17 cases of sacrococcygeal teratoma (SCT) with the literature in an attempt to clarify the natural history of this tumor and to identify factors related to its prognosis and management. METHODS: The obstetrical, neonatal and surgical data were analyzed for 17 cases of SCT observed between July 1985 and December 1998. RESULTS: Three fetuses died in utero or shortly after birth. In the remaining 14, the tumors were removed. Twelve of the infants are currently tumor-free, with good sphincter control and lower-limb function. The remaining two died: one had a malignant tumor, and the other had a recurrence of an embryonal carcinoma. Recurrent tumors (mature histotypes) were also removed from two of the 12 patients who survived. CONCLUSIONS: Benign SCTs generally have favorable prognosis. Negative prognostic factors for SCT include solid tumors, those detected early in pregnancy, malignant histotypes, polyhydramnios, placentomegaly, and fetal hydrops.