SERCA directs cell migration and branching across species and germ layers.

Branching morphogenesis underlies organogenesis in vertebrates and invertebrates, yet is incompletely understood. Here, we show that the sarco-endoplasmic reticulum Ca2+ reuptake pump (SERCA) directs budding across germ layers and species. Clonal knockdown demonstrated a cell-autonomous role for SERCA in Drosophila air sac budding. Live imaging of Drosophila tracheogenesis revealed elevated Ca2+ levels in migratory tip cells as they form branches. SERCA blockade abolished this Ca2+ differential, aborting both cell migration and new branching. Activating protein kinase C (PKC) rescued Ca2+ in tip cells and restored cell migration and branching. Likewise, inhibiting SERCA abolished mammalian epithelial budding, PKC activation rescued budding, while morphogens did not. Mesoderm (zebrafish angiogenesis) and ectoderm (Drosophila nervous system) behaved similarly, suggesting a conserved requirement for cell-autonomous Ca2+ signaling, established by SERCA, in iterative budding.

Single incision prone retroperitoneoscopic paediatric nephrectomy.

OBJECTIVE: We have previously reported our early experience (2 patients) of single-incision nephrectomy via the retroperitoneal prone route using an advanced access platform (GelPOINT Mini). Here, we review our series to date and also present a detailed video demonstrating the technique. METHOD: In the prone position, a single transverse incision was made at the midpoint on a line along the lateral border of erector spinae bounded by the lower border of the 12th rib and iliac crest. Posterior abdominal muscles were split and the deep lumbodorsal fascia incised. The Alexis retractor was positioned and the Gel-Seal cap with low-profile sleeves locked in place. Hilar vessels were divided by endoclip application or harmonic scalpel. The kidney was retrieved directly or via an endobag. The Alexis retractor was removed and wound closed with absorbable sutures. RESULTS: Between July 2013 and March 2015, we have used this approach to perform 10 nephrectomies in nine patients (4 male and 5 female). The median age at nephrectomy was 10.9 years (range 2.7-15.9 years). The median kidney length was 7.5 cm (range 3.7-11.5 cm). No complications occurred and none converted to open procedure. CONCLUSION: Single incision retroperitoneal nephrectomy is feasible, safe, and provides excellent cosmesis.

Spiral rotational flap for the creation of a new umbilicus in bladder exstrophy.

OBJECTIVE: A range of surgical techniques have been described to create a new umbilicus in children with bladder exstrophy. We present a detailed video demonstrating our technique for the creation of a new umbilicus. METHOD: The position of the planned umbilicus and flap were marked on the abdomen. Incisions were made using monopolar diathermy. The flap was raised and excess fat thinned. Subcutaneous tissues were apposed, and the flap rotated and sutured in spiral fashion with interrupted 6/0 monocryl forming a cone which was subsequently inverted. RESULTS: Between May 1999 and August 2014, we used this technique for the creation of a new umbilicus in 47 bladder exstrophy patients (31 boys and 16 girls). No complications occurred. All were followed up as part of our bladder exstrophy programme. CONCLUSION: The technique allows reproducible excellent cosmetic results. Aesthetic results are durable and it should be considered as an alternative to existing reported techniques.

Paediatric sutureless circumcision and modified circumcision: video demonstration.

OBJECTIVE: Circumcision is undertaken using a variety of surgical methods. Modified circumcision for the hooded prepuce or mild glanular hypospadias mandates an excellent cosmetic result. Tissue glue is an alternative to sutures for circumcision in children and has significant advantages in terms of cosmesis and postoperative pain. We present two detailed videos demonstrating our technique for sutureless circumcision and modified circumcision. METHOD: The incisions were marked on the outer and inner preputial skin. Incisions were made with a scalpel. Complete haemostasis was ensured. Tissue adhesive was placed onto the wound using a 32-gauge needle to allow controlled application. It is important to ensure that the coronal sulcus is free of glue at the end of the procedure. Once the circumcision is complete the penis can be cleaned and temporarily dressed. RESULTS: Between November 2008 and September 2011, we have used this technique for 57 circumcisions and 30 modified circumcisions. No complications occurred. All were followed up for a minimum of 3-6 months. CONCLUSION: The technique allows reproducible excellent cosmetic results, can be easily taught to junior surgical staff and should be considered as an alternative to a standard sutured circumcision.

Spider-man, magnets, and urethral-cutaneous fistula.

Self-inserted foreign bodies in the genitourinary tract or externally attached to the male genitalia can be challenging to the urologist. These cases often necessitate urgent assessment and intervention. We present the case of a 12-year-old boy who inserted 1 magnet into his urethra and placed a second on the ventral surface of the penis. This led to the development of a urethral-cutaneous fistula. To our knowledge, this is the first reported case illustrating the development of an iatrogenic fistula after self-insertion of a foreign body in the genitourinary tract.

Meconium per vagina: a rare presentation of meconium peritonitis.

Meconium peritonitis results from antenatal perforation of the gastrointestinal tract; it presents as gastrointestinal obstruction, intraabdominal masses, or calcification. The presentation with passage of meconium per vagina secondary to meconium peritonitis is rare. We describe the radiologic and surgical findings in a neonate who had passage of meconium per vagina secondary to ileal atresia and meconium peritonitis. Initial clinical and radiologic examination suggested rectal atresia with an associated rectovaginal fistula, although subsequently, this was not the case. Possible explanations for the passage of meconium per vagina include decompression of a meconium cyst via the left fallopian tube or direct perforation of a collection into the vagina from the peritoneal cavity.

Airway smooth muscle dysfunction precedes teratogenic congenital diaphragmatic hernia and may contribute to hypoplastic lung morphogenesis.

Fetal intervention aims to improve lung growth and survival in congenital diaphragmatic hernia (CDH). Airway smooth muscle (ASM) is important in lung development: ASM progenitors produce a key growth factor for lung morphogenesis (fibroblast growth factor 10); ASM contractility is also coupled to growth. ASM hyperreactivity occurs in postnatal CDH and may exacerbate barotrauma via impaired lung compliance. We hypothesize that ASM hyperreactivity and its sequelae are based on an early developmental lesion of ASM activity in hypoplastic lung. Sprague-Dawley rats were fed 100 mg nitrofen on Day 9.5 of pregnancy to induce lung hypoplasia in offspring (controls had vehicle alone). Normal and hypoplastic lung primordia were cultured from Day 13.5 of gestation at 37 degrees C in 5% CO(2) and loaded at 54 or 78 h with Ca(2+)-sensitive indicators: Fluo-4 for confocal imaging and Indo-1 or Fura-2 for photometric measurements of [Ca(2+)](i). Hypoplastic lung features spontaneous propagating ASM Ca(2+) transients with reduced frequency, increased amplitude, and significantly prolonged plateau duration, relative to control lung. Nonetheless, hypoplastic lung exhibits normal requirement for extracellular calcium entry and intracellular calcium release in initiation and regulation of ASM Ca(2+) waves. Early ASM dysfunction in lung hypoplasia is apparent as specific anomalies of Ca(2+) transients that indicate a problem with plasmalemmal ion channels/action potential generation. Elucidation of such an ASM lesion may allow pharmacologic amelioration not only of ASM hyperreactivity and its sequelae, but also of hypoplastic lung growth itself.

Hidden pathologies associated with (and concealed by) early gestational isolated fetal hydrothorax.

Isolated fetal hydrothorax is an uncommon finding on routine prenatal ultrasound and is associated with a variable prognosis. We believe this is the first report of a patient whose antenatal hydrothorax was associated with both congenital diaphragmatic hernia and congenital pulmonary lymphangiectasia. This rare combination caused significant problems in diagnosis and subsequent treatment.

Spontaneous propagating calcium waves underpin airway peristalsis in embryonic rat lung.

Prenatal airways from diverse species exhibit spontaneous peristaltic contractions (airway peristalsis). These contractile waves appear coupled to and may function to regulate prenatal lung growth. They are unaffected by atropine or tetrodotoxin but abolished by nifedipine. Nevertheless, the mechanisms by which these contractile waves are generated, regulated, and propagated remain obscure. Using calcium imaging and whole embryonic lung organ culture, we demonstrate for the first time that peristalsis of the embryonic airway is driven by spontaneous, regenerative, temperature-sensitive calcium (Ca2+) waves. These Ca2+ waves propagate between individual airway smooth muscle cells coupled via gap junctions, are likely to be action potential-mediated, and are dependent on not only extracellular calcium entry via L-type voltage-gated channels but also intracellular Ca2+ stores. Thus, if airway peristalsis regulates lung growth, these findings mean that airway smooth muscle Ca2+ waves in turn regulate prenatal lung morphogenesis.