Rectal Biopsy for Hirschsprung's Disease: A Multicentre Study Involving Biopsy Technique, Pathology and Complications.

BACKGROUND: The heterogeneity of rectal biopsy techniques has encouraged us to search for a surgical and pathological standardisation of this diagnostic technique to exclude Hirschsprung's disease. The different amounts of information on the anatomopathology report prompted us to compile a template for the anatomopathology report for diagnostic rectal biopsies for surgical colleagues and pathologists working on Hirschsprung's disease. METHODS: We gathered the anonymous biopsy information and its pathology information from five hospitals for all patients in which rectal biopsies were taken to diagnose Hirschsprung's disease over two years (2020-2021). RESULTS: Of the 82 biopsies, 20 suction (24.4%), 31 punch (37.8%) and 31 open biopsies (37.8%) were taken. Of all biopsies, 69 were conclusive (84.2%), 13 were not (15.8%). In the suction biopsy group, 60% were conclusive and 40% were not; for punch biopsy, the values were 87% and 13%, respectively and for open biopsy, 97% and 3%. Inconclusive results were due to insufficient submucosa in 6/8 suction biopsies, 4/4 punch biopsies and 0/1 open biopsies. An insufficient amount of submucosa was the reason for an inconclusive result in 6/20 cases (30%) after suction biopsy, 4/31 (12.9%) cases after punch biopsy and 0 cases (0%) after open biopsy. We had one case with major postoperative bleeding post suction biopsy; there were no further adverse effects after biopsy. CONCLUSIONS: Diagnostic rectal biopsies in children are safe. Non-surgical biopsies are more likely to give inconclusive results due to smaller amounts of submucosa present in the specimen. Open biopsies are especially useful when previous non-surgical biopsies are inconclusive. An experienced pathologist is a key factor for the result. The anatomopathology report should specify the different layers present in the specimen, the presence of ganglion cells and hypertrophic nerve fibres, their description and a conclusion.

Thyroglossal duct cysts in children: a 30-year survey with emphasis on clinical presentation, surgical treatment, and outcome.

Background: Thyroglossal duct cysts (TGDC) are one of the most common congenital anomalies found in the anterior neck region of children. Sistrunk's procedure, described in 1920 already, is still considered as the gold standard. However, clinical reality shows that in a minority of patients, marsupialization and simple cyst excision are still performed as well. Cyst recurrence is the most feared complication. The main goal of this retrospective study is to determine risk factors of recurrence. Furthermore, data on presentation characteristics, management and outcome were collected as well. Methods: The data of 104 patients aged between 0 and 16 years who underwent surgery for TGDC at the University Hospital of Brussels between 1986 and 2016 were retrospectively analyzed. We focused on aspects of clinical presentation, intra- and postoperative treatment and long-term follow-up. Results: Overall recurrence of TGDC was seen in twelve of the 104 cases (11.5%). Eight out of these 12 showed a preoperative infection, 4 out of 12 had intra-operative cyst rupture. Five out of the 12 patients had not been treated by the Sistrunk procedure, but by cyst excision or marsupialization only. Non-adherence to the Sistrunk procedure appeared to be the only significant risk factor of TGDC recurrence. Conclusion: Our study shows that Sistrunk's operation for thyroglossal duct cyst in pediatric patients is significantly superior in reducing the risk of cyst recurrence compared to other surgical treatments. Preoperative infection and cyst rupture did not influence the recurrence rates.

Early experience with single-incision laparoscopic surgery for the placement of a gastrostomy in a 10-year-old girl: a case report.

INTRODUCTION: Access procedures for alimentation have been performed both endoscopically and surgically. In patients in whom endoscopic gastrostomy feeding tubes cannot be placed, single-incision laparoscopic surgery gastrostomy is an alternative method. This minimally invasive approach is a new technique performed through a single umbilical incision and without the need for additional laparoscopic ports. CASE PRESENTATION: In this article we present a case of single-incision laparoscopic surgery gastrostomy performed with conventional laparoscopic instruments in a 10-year-old girl of Caucasian ethnicity who was not a candidate for a percutaneous endoscopic gastrostomy tube because of esophageal varices due to her advanced-stage cystic fibrosis with liver cirrhosis and portal hypertension. She also had an umbilical hernia, which was repaired during the same procedure through the same incision. Access and pneumoperitoneum were obtained through the umbilicus with the single-incision laparoscopic surgery port. The selected site for the feeding tube in the stomach was exteriorized through this incision and a feeding tube was placed. The stomach was returned into the abdomen. The fascial defect, and thus also the hernia, was repaired, and the 2cm umbilical incision was closed with endocutaneous sutures. The total operative time was 25 minutes. Our patient's intra-operative and post-operative course was uneventful. We were able to use the feeding tube on the first post-operative day with good intestinal function. Our patient and her parents were pleased with the cosmetic result. CONCLUSIONS: The single-incision laparoscopic surgery procedure seems to be a less invasive alternative to open placement of gastrostomy. This approach has the possible advantages of reduced post-operative pain, faster return to normal function, reduced port site complications, improved cosmesis and better patient satisfaction.

Laparoscopic repair of diaphragmatic Morgagni hernia in children: review of 3 cases.

This article retrospectively reviews the laparoscopic repair of Morgagni hernias in 3 children. The surgical procedure was performed by closing the defect using extracorporeal, interrupted, nonabsorbable sutures. Recovery was uneventful in all 3 patients. There were no recurrences and the chest radiograph stayed normal during the postoperative follow-up.

Long-term functional sequelae of sacrococcygeal teratoma: a national study in The Netherlands.

BACKGROUND: Long-term functional sequelae after resection of sacrococcygeal teratoma (SCT) are relatively common. This study determines the incidence of these sequelae associated clinical variables and its impact on quality of life (QoL). PATIENTS AND METHODS: Patients with SCT treated from 1980 to 2003 at the pediatric surgical centers in The Netherlands aged more than 3 years received age-specific questionnaires, which assessed parameters reflecting bowel function (involuntary bowel movements, soiling, constipation), urinary incontinence, subjective aspect of the scar, and QoL. These parameters were correlated with clinical variables, which were extracted from the medical records. Risk factors were identified using univariate analysis. RESULTS: Of the 99 posted questionnaires, 79 (80%) were completed. The median age of the patients was 9.7 years (range, 3.2-22.6 years). There were 46% who reported impaired bowel function and/or urinary incontinence (9% involuntary bowel movements, 13% soiling, 17% constipation), and 31% urinary incontinence. In 40%, the scar was cosmetically unacceptable. Age at completion of the questionnaire, Altman classification, sex, and histopathology were not risk factors for any long-term sequelae. Size of the tumor (>500 cm3) was a significant risk factor for cosmetically unacceptable scar (odds ration [OR], 4.73; confidence limit [CL], 1.21-18.47; P = .026). Long-term sequelae were correlated with diminished QoL. CONCLUSION: A large proportion of the patients with SCT have problems with defecation, urinary incontinence, or a cosmetically unacceptable scar that affects QoL. Patients who are at higher risk for the development of long-term sequelae cannot be clearly assessed using clinical variables.

Laparoscopic management of insulinoma in a child with multiple endocrine neoplasia type 1.

The diagnosis and surgical management of insulinomas associated with multiple endocrine neoplasia type 1 (MEN1) pose additional problems in children because of the long-term risk of recurrence of other pancreatic and non-pancreatic tumors. We report a diagnostic confirmation by laparoscopic ultrasound of an insulinoma and its successful removal by laparoscopic enucleation in an 8- year-old boy who was admitted to our hospital with a history of recurrent episodes of absences, headache, and visual and auditive disturbances diagnosed as hyperinsulinism-related hypoglycemia. Magnetic resonance imaging of the pancreas showed a small contrast-enhancing lesion in the body of the pancreas, suspected for insulinoma. MEN1 was genetically proven by direct DNA testing. A pancreatic tumor can arise before the age of 10 in patients with MEN1 and can be surgically treated by a laparoscopical approach.

Study of the factors associated with recurrence in children with sacrococcygeal teratoma.

PURPOSE: The aim of this study was to explore effects of (1) histological involvement of resection margins with microscopic residue, (2) incomplete removal of coccyx, and (3) tumor spillage on recurrence and on survival in children operated upon for sacrococcygeal teratoma (SCT). METHODS: Retrospective review of 70 patients treated between 1960 and 2003. RESULTS: Fifty-four girls and 16 boys presented with SCT diagnosed prenatally (12), at birth (37), or later (21). Thirty-six percent of tumors were Altman type I, 27% type II, 18% type III, and 18% type IV. Histologically, mature teratoma was observed in 48 patients, immature teratoma in 11, yolk sac tumor (YST) in 9, embryonal carcinoma in one, and mixed tumor in one. Eighty-four percent of patients solely underwent surgical extirpation. Six (8.5%) patients died. However, mortality for the group of 42 patients treated during the past 15 years was as low as 2.5%. Tumor recurrence was observed in 5 patients, 2 of whom died. Of 3 patients with initially mature teratoma, 1 showed local immature recurrence and 2 malignant recurrences. One of the latter died. Of 2 patients with initially immature teratoma grade I, one relapsed with a benign lesion and one with YST leading to death. Possible eliciting factors had been demonstrated in 3 patients. Histological analysis of resection margins showed tumoral involvement in 11 patients (and also in one patient after resection of a recurrent tumor). Only one of those with YST focus in the resection margin showed recurrence. Intraoperative tumor spillage presented in 2 patients, who both died of metastatic disease. Spillage of tumoral cyst fluid occurred in 6, none developed recurrence. One of 5 patients whose coccyx had not been removed died of metastatic disease. One with immature teratoma developed a benign recurrent tumor. The other 3 showed no recurrence. CONCLUSIONS: Microscopic involvement of the resection margins of mature or immature SCT is rarely associated with recurrence, provided there are no YST foci in the resection margins. A conservative attitude then appears to be justified. Spillage of cyst fluid was never associated with recurrence, unlike spillage of tumor and absence of removal of coccyx.

Ovarian germ cell tumors in children: a clinical study of 66 patients.

BACKGROUND: Ovarian germ cell tumors are rare in childhood. The aim of this study is to review clinical presentation, management, and outcome in a two-center series of girls with ovarian germ cell tumor. PROCEDURE: The records of 66 patients (median age 9 years) with histologically proven ovarian germ cell tumor (either benign or malignant), treated over a 44-year-span, were reviewed. RESULTS: Pain and an abdominal mass were the most frequent symptoms. The tumors were right-sided in 35, left-sided in 28, and bilateral in 3. Most patients (52) were stage I, 4 were stage II, 6 stage III, and 1, with liver metastases, stage IV. Sixteen patients had an emergency operation for tumor torsion. Unilateral salpingo-oophorectomy was the most frequently performed procedure (n = 46), and ovarian-sparing tumorectomy was performed in 9 patients (one bilaterally). Histologically, teratomas were found most frequently (mature: 45, immature: 9), followed by mixed tumors (n = 7), yolk sac tumors (n = 3), dysgerminoma (n = 2), gonadoblastoma (n = 2), and embryonal carcinoma (n = 1). Surgical removal of the tumor with or without the ovary and/or adnex was the sole treatment in 55 patients, chemotherapy was administered in 10 and radiotherapy + chemotherapy in one. Intra-operative spillage of tumoral fluid occurred in six; this did not influence outcome in five. Recurrence was observed in three patients. Two patients, with malignant disease, died. The 64 survivors are now between 8 months and 44 years after treatment. CONCLUSIONS: With a recurrence rate of 4.5% and a mortality rate of 3%, this series confirms the excellent prognosis for girls with ovarian germ cell tumor (GCT).

Retroperitoneal germ cell tumors: a clinical study of 12 patients.

PURPOSE: The aim of the study was to examine the clinical presentation, method(s) of treatment, complications, and results in newborns and infants with retroperitoneal germ cell tumors (GCTs). METHODS: A retrospective chart review of all patients treated between 1974 and 2002 for GCT located in the retroperitoneum in 2 institutions identified 12 patients with histologically proven retroperitoneal GCT. Vital data concerning pregnancy and delivery were analyzed. Age at diagnosis and symptoms were recorded, as well as possibly associated anomalies. Data concerning surgical treatment, perioperative and postoperative complications, histological staging, and final outcome were all analyzed. RESULTS: In 3 patients, the diagnosis had been made antenatally between 31 and 35 weeks of gestation. In 1 patient, the diagnosis was made at birth, and in 8 later in life (ages 3, 5, 7, 8, 8, 11, 18, and 24 months). Symptoms in these 8 boys and 4 girls were abdominal distension and a palpable upper abdominal mass, right-sided in 5, left-sided in 5, and central in 2; the tumor was usually big. Associated anomalies were noted in 4 patients and were chromosomal in 3 (Down syndrome in 2 and Klinefelter syndrome in 1). One baby died of uncontrollable bleeding during an emergency operation immediately after traumatic birth. The other 11 infants survived. Four other patients showed serious perioperative complications (1 caval vein tear, 1 choledochal tear, 1 cyst rupture, and 1 esophagogastric tear) which were managed without further consequences. Histologically, 4 tumors were mature teratomas, 6 were immature teratomas (grade I in 4, grade II in 1, and grade II-III in 1), and 2 were malignant yolk sac tumors (YSTs). The patients with YSTs underwent surgical biopsy, followed by chemotherapy and excision of the remaining tumor and of the metastases. No adjuvant treatment was administered in the patients with benign disease. Nine survivors with benign tumor are disease-free between 1 and 30 years after surgery. Two patients with YST have now been in remission for 6 and 5 years, respectively. CONCLUSIONS: Both this study and the literature review performed testify to the extreme rarity of GCTs in the retroperitoneum. Surgical removal of the tumors appeared to be hazardous because of the extent of the tumor, the displacement and elongation of adjacent structures and organs, and/or the adhesion of the tumor to surrounding tissues; this resulted in several perioperative complications. The long-term results are good, however, with 9 of 10 patients with benign tumors in good health after a mean follow-up of 12 years, and with the 2 patients with YST in remission for 6 and 5 years, respectively.

Strategy for management of newborns with cervical teratoma.

BACKGROUND: Cervical teratomas are extremely rare tumors with high perinatal mortality and morbidity rates. OBJECTIVE: To compare our experience and outcome in newborns with cervical teratoma with similar reports from the literature, in order to propose a structured approach. METHODS: A retrospective review of seven patients treated between 1986 and 2000 was performed. The results of these seven patients were compared with and added to a series of 44 well-documented patients retrieved from the literature. RESULTS: In four of the seven patients, the diagnosis was not suspected antenatally. Three of the patients survived, one died. In the other three, the diagnosis was made antenatally. Two were born using the ex-utero intrapartum treatment (EXIT) procedure, one by planned cesarean section. Only one of these three survived. Mortality in the total series of 51 patients was 33% overall, and 46% in the group in which the diagnosis had been made antenatally. Peri- and post-operative complications were reported in 27%. Although larger tumors caused polyhydramnios more frequently than smaller tumors, and were associated with more severe respiratory distress, the relationship between tumor volume at birth and final outcome could not be established. This makes difficult the identification of fetuses with a disastrous prognosis. CONCLUSION: Although mostly benign, cervical teratomas are still associated with high mortality rates. Timely antenatal diagnosis is indispensable in reducing morbidity and mortality caused by upper airway obstruction. A structured approach to the management of cervical teratoma is proposed.