[Thoracoscopic Sympathectomy for Palmar and Axillary Hyperhidrosis]. / Thorakoskopische Sympathektomie bei palmarer und axillärer Hyperhidrosis.

OBJECTIVE: This video is a step-by-step description of thoracoscopic sympathectomy. INDICATION: Sweating is essential for thermoregulation. Hyperhidrosis is a condition of excess sweating from the eccrine glands and is associated with severe suffering for patients of all ages. It often worsens during adolescence. A generalised and focal type of hyperhidrosis which affects mainly the face, armpits, hands and feet can be distinguished from the focal variant. Thoracic sympathectomy has become the standard treatment for palmar and axillary hyperhidrosis worldwide. METHODS: The procedure is performed in the supine position with the upper body elevated about 30° in an adolescent patient. Both arms are abducted at 90° and single tube endotracheal ventilation is employed. A 3 mm trocar is placed in the anterior axillary line for a 3 mm 30° optic. A 5 mm trocar placed on the anterior axillary line (or breast fold in female patients) of the 4th or 5th intercostal space is used for the bipolar forceps. The sympathetic trunk and ganglia T 2 - 4 are identified and coagulated over the heads of ribs. CONCLUSION: The thoracoscopic approach to focal palmar and axillary hyperhidrosis allows clear identification of the sympathetic structures on each side. Under direct vision, selective ablation of the ganglia and sympathetic trunk provides long-term benefit for patients.

Human exome and mouse embryonic expression data implicate ZFHX3, TRPS1, and CHD7 in human esophageal atresia.

INTRODUCTION: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) occurs approximately 1 in 3.500 live births representing the most common malformation of the upper digestive tract. Only half a century ago, EA/TEF was fatal among affected newborns suggesting that the steady birth prevalence might in parts be due to mutational de novo events in genes involved in foregut development. METHODS: To identify mutational de novo events in EA/TEF patients, we surveyed the exome of 30 case-parent trios. Identified and confirmed de novo variants were prioritized using in silico prediction tools. To investigate the embryonic role of genes harboring prioritized de novo variants we performed targeted analysis of mouse transcriptome data of esophageal tissue obtained at the embryonic day (E) E8.5, E12.5, and postnatal. RESULTS: In total we prioritized 14 novel de novo variants in 14 different genes (APOL2, EEF1D, CHD7, FANCB, GGT6, KIAA0556, NFX1, NPR2, PIGC, SLC5A2, TANC2, TRPS1, UBA3, and ZFHX3) and eight rare de novo variants in eight additional genes (CELSR1, CLP1, GPR133, HPS3, MTA3, PLEC, STAB1, and PPIP5K2). Through personal communication during the project, we identified an additional EA/TEF case-parent trio with a rare de novo variant in ZFHX3. In silico prediction analysis of the identified variants and comparative analysis of mouse transcriptome data of esophageal tissue obtained at E8.5, E12.5, and postnatal prioritized CHD7, TRPS1, and ZFHX3 as EA/TEF candidate genes. Re-sequencing of ZFHX3 in additional 192 EA/TEF patients did not identify further putative EA/TEF-associated variants. CONCLUSION: Our study suggests that rare mutational de novo events in genes involved in foregut development contribute to the development of EA/TEF.

A New Operative Approach for Long-Gap Esophageal Atresia.

Surgical management of long-gap esophageal atresia (LGEA) remains challenging. Yet, there is a consensus among pediatric surgeons to preserve native esophagus. We used a new surgical technique to successfully manage three children diagnosed with LGEA. This technique consists of a combined thoracic and cervical approach to the EA repair using the patient's native esophagus. All patients initially had had gastrostomy and continuous upper pouch suction while awaiting surgery. This new technique has the potential to become the choice method in LGEA management.

Esophageal Atresia with or without Tracheoesophageal Fistula (EA/TEF): Association of Different EA/TEF Subtypes with Specific Co-occurring Congenital Anomalies and Implications for Diagnostic Workup.

BACKGROUND: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) represents the most common developmental malformation of the upper digestive tract. It is classified into six subtypes according to the classification of Vogt, depending on anatomical variation of this malformation. Around 50% of the patients with EA/TEF present additional anomalies, which often influence, next to the EA/TEF subtype, the overall prognosis of EA/TEF newborns. Here, we investigated the association of the different EA/TEF subtypes with co-occurring congenital anomalies in EA/TEF patients and demonstrate their implications for postnatal diagnostic workup. MATERIALS AND METHODS: We investigated 333 patients of a large German multicenter study born between 1980 and 2012. After evaluation of all available clinical records, 235 patients were included in our analysis. We compared our results with existing data. RESULTS: The highest risk for co-occurring anomalies was seen in patients with most common Vogt 3b (p = 0.024), especially for additional gastrointestinal anomalies (p = 0.04). Co-occurring anomalies of the skin were significantly more common in patients with subtype Vogt 2 (p = 0.024). A significant correlation was observed for an impaired neurodevelopmental outcome and EA/TEF Vogt 3a (p = 0.041). Patients with EA/TEF showed a higher risk to present with any additional congenital anomaly compared with the general population (p < 0.001). CONCLUSION: Our results warrant thorough clinical workup for gastrointestinal anomalies especially in patients with Vogt 3b. Moreover, it might be necessary to focus on a thorough aftercare for neurocognitive development in patients with Vogt 3a. The here presented observations need to be confirmed by future studies.

[Laparoscopic Resection of Urachal Cysts]. / Die laparoskopische Resektion einer Urachuszyste.

Objective This video is a step-by-step description of the laparoscopic technique for the resection of urachal cysts. Indication The urachus is an embryonic extraperitoneal structure between the urinary bladder and the umbilicus. Failure of the allantois to obliterate in utero leads to urachal anomalies such as fistulas or cysts. Because of the risk of infection and malignant degeneration later in life, urachal fistulas or cysts are usually removed by surgical excision. Method The procedure is performed in supine position with 3 mm trocars in the epigastric angle, the left upper abdomen and the left middle abdomen. During the operation, the trocar in the epigastric angle will be upsized to 5 mm in order to introduce a 5 mm polymer clip applier. We use a 5 mm 30° optic and a 3 mm monopolar hook for dissection. Conclusion This laparoscopic approach to removing urachal remnants allows for the involved structures to be clearly identified and accurately dissected. A complete resection of urachal remnants is facilitated by direct visualization of the entire anomaly down to the bladder dome.

[Laparoscopic-assisted mobilisation and resection of a sacrococcygeal teratoma Altman type III]. / Die laparoskopisch assistierte Mobilisation und Resektion eines Steißbeinteratoms Altman Typ III.

Objective This video is a step-by-step illustration of the laparoscopic-assisted mobilisation and resection of a sacrococcygeal teratoma Altman type III. Indication Laparoscopic supralevatory tumour mobilisation is an established method in selected centres and can be the initial step in approaching sacrococcygeal teratomas with an intrapelvic portion, facilitating a complete (R0) resection. Method Laparoscopic surgery is performed in supine position with a 5 mm umbilical trocar, a 5 mm trocar in the right upper abdomen, and two 3 mm trocars in the left middle and lower abdomen. We use a 5 mm 45° optic and 3 mm laparoscopic grasping forceps as well as Overholt clamps. For coagulation, a laparoscopic 5 mm diathermy Sealer (LigaSure™, Covidien, Neustadt) is used. Conclusion In selected tumours, laparoscopic assisted tumour mobilisation enables surgeons to clearly identify and ligate the primary tumour-supplying vascular structures at the beginning of the operation, thereby avoiding the risk of major bleeding throughout the subsequent tumour mobilisation. The laparoscopic approach also allows an accurate dissection and mobilisation of the supralevatory tumour parts under direct vision. After repositioning the patient into the prone position, infralevatory preparation can be carried out safely and fast in the conventional sacral approach without any major risk of bleeding.

Quality of Life after Surgical Treatment for Esophageal Atresia: Long-Term Outcome of 154 Patients.

Background The short- and long-term surgical results in patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) have been described in depth from a physician's perspective. Contrarily, the perception and coping strategies of affected patients and their parents have rarely been reported. The aim of this study was to generate data on this matter. Patients and Methods A total of 154 patients who had operative reconstruction for EA between 1971 and 2012 were evaluated for demographic data, surgical technique, affection of daily life, and coping strategies. Results Gastroesophageal reflux (GER) symptoms were reported in 59% of cases with 33% requiring fundoplication. Regular bougienages of anastomotic strictures were necessary in 68% with 36% requiring repeated dilatations in the first postoperative year. Enteral nutrition via a nasogastric tube was performed in 66% after surgery. In 40%, the tube was needed until their sixth week of life. In 25%, nutritional support was necessary more than 1 year out of surgery. Quality of life in general was felt to be impaired according to the patients' parents in 50%. Regarding medical advice about long-term morbidities, more than 50% of the parents felt insufficiently advised. There were no statistical differences between the EA/TEF subtypes regarding GER symptoms, frequency of esophageal dilatations, eating behaviors, or support of the parents in feeding management. Conclusion Our observations indicate that a high percentage of EA/TEF patients and families require more intensive aftercare and support during the first year following primary reconstruction than previously thought. We observed a higher need for adequate parental information on long-term complications of their children compared with current practice.

More than fetal urine: enteral uptake of amniotic fluid as a major predictor for fetal growth during late gestation.

UNLABELLED: The purpose of our study was to investigate the importance of amniotic fluid (AF) for fetal growth during late gestation using esophageal atresia (EA) patients as a model. In this retrospective cohort study, we compared the z-scores adapted for birth weights (BW z-scores) for each of 517 European newborns with congenital pre-gastric intestinal atresia, i.e., EA, to a European reference population. To account for the influence of the intestinal atresia on fetal growth per se, we compared adapted birth weights for each of 504 European newborns with post colonic intestinal atresia (anorectal malformation (ARM) with atresia of the anus) to the same European reference population. Analysis of the complete cohort showed (i) a significantly higher rate of small for gestational age newborns among EA compared to ARM newborns (p < 0.001) and (ii) significantly lower BW z-scores among EA compared to ARM newborns (p < 0.001). BW z-scores of EA newborns were significantly lower in term compared to preterm newborns with an inverse correlation with gestational age (GA) (Spearman correlation coefficient, r = -0.185, p < 0.001). CONCLUSIONS: Enteral uptake of AF seems to play a pivotal role in fetal growth during late gestation. WHAT IS KNOWN: • Peak velocity of fetal weight gain occurs at 33 weeks of gestation and continues until birth. During this period, fetal growth is mainly characterized by cellular hypertrophy. • Amniotic fluid (AF) comprises large amounts of hormones and growth regulators. What is New: • A significantly higher rate of small for gestational age and lower birth weights and z-scores are observed among newborn infants with congenital pre-gastric intestinal atresia. • These findings suggest that enteral uptake of AF is a major predictor for fetal growth during late gestation.