tickletherapy

J. Paediatr. Child Health (2003)39, 719–721
Letters to the Editor
12 July 2003 Dear Editor,
DELAYED PRESENTATION OF A NEONATAL LONG
LINE INCIDENT
Central venous catheter insertion is technically an easy pro-cedure for a neonatal registrar, but to achieve optimal position for the catheter tip can be difficult. The recommended correct positions are either at the junction between the superior vena cava and the right atrium or immediately above the diaphragm if inserted into the inferior vena cava.1Extravasations from catheters in the inferior vena cava have been described to cause ascites and acute abdomen in neonates.2We report a case of malpositioning of a peripherally inserted central catheter (PICC), delayed presentation of an abdominal complication related to it and complete recovery on removal of the long line.
A 29-week-old gestation male infant, weighing 1.06 kg, was admitted and electively ventilated with administration of two doses of surfactant. Umbilical arterial and venous catheters were inserted on day 1. Non-nutritive orogastric feeding was started on day 5. On day 8, abdominal distension was noted and orogastric aspirates were bilious. Feeds were then stopped. An abdominal X-ray showed a thickened bowel wall. Blood counts were normal. He was treated for early necrotizing enterocolitis (NEC) after removal of umbilical lines.
A PICC 27-G (Vygon, Nutriline, Germany) was inserted on day 10, entering the saphenous vein just medial to the left knee for a length of 15 cm. Plain radiography of the abdomen reported the tip of long line in the common iliac vein. Total parenteral nutrition (TPN) was started through the long line. Abdominal distension improved and there was no more bilious aspirate. On day 14, a redness of the epigastric and hypochon-drial areas was noted. The periumbilical area was normal. A plain X-ray of the abdomen showed normal gaseous pattern. Cloxacillin was added and NEC measures continued as before. Surgical opinion sought at that time was that of a progressing NEC with associated abdominal wall erythema. The redness and induration of the anterior abdominal wall continued to increase. By day 18, a localized area of cellulitis was noted superior and to the left of the umbilicus. This became necrotic within 24 h and abdominal distension increased. Under sterile precautions, a thin eschar was removed and whitish fluid drained out. Microscopic examination of the fluid did not show any pus cells or bacteria and culture was sterile. Dressings applied over the drained area became continuously wet with a whitish discharge. A repeat microscopic examination of the fluid the next day showed flat globules. A deliberate diagnostic purge of intralipid being given through the long line resulted in visible oozing at the site of the ulcer on the abdomen. This confirmed a communication of the long line with the ulcer.
It was noted that there had been no obvious migration of the line as indicated by the PICC markings externally, and there was no resistance while the long line was removed. All abdominal X-rays had shown no change in the site of the catheter tip. There had been no syringe pump alarms of high pressure at any time. Throughout this period, blood counts and biochemical parameters were normal and the culture of blood and the catheter tip were negative.
The abdominal wall erythema completely resolved in 2 days. Feeds were then restarted. The ulcer healed quickly leaving only a small scar. The infant tolerated feeds well and started gaining weight. He was discharged home on day 51 with a weight of 2020 g.
A review of the abdominal X-ray of this case, taken on the day of insertion of the venous catheter, showed coiling of the catheter at the level of the head of femur. This would explain the length of 15 cm long line inserted. We think that the catheter had taken an abnormal route through a tributary of the external iliac vein. We considered possible factors contributing to the late appearance of signs and recognition of the problem. Upon insertion, we were able to aspirate blood back freely into the line before the line was secured. At some point in time the line must have perforated the vein. The high concentration of TPN in a small vein probably increases the risk of vessel erosion. The TPN solution used in this present case was a standard strength comprising of 10% dextrose with 40 g amino-acids/1000 mL and 20% intralipid with electrolytes and trace elements of unremarkable concentrations. The parenteral nutri-ents had extravasated, but abdominal signs only appeared 4 days after its insertion. It is likely that absorption of TPN by the peritoneal cavity allowed maintenance of normoglycaemia and contributed to the slow appearance of abdominal disten-sion. This contrasts with the case reported by Baker et al. where hypoglycemia was a problem.3
In conclusion, we should confirm the tip of long lines ideally using a contrast medium. If the appropriate amount of contrast is injected, the catheter would be outlined and a flare of contrast can be seen leaving the tip. If there is coiling of the long line, as in our case, the line should be pulled back until the lengths of coils are straightened. This will reduce the risk of migration. A long line has too much slack within these coils and could be pushed further along if the coils spontaneously straighten. This may occur with active movement of the limb. If a repositioning of the line is considered too difficult or the position of the tip is in doubt, the long line should be removed completely.
ACKNOWLEDGEMENTS
The authors thank Dr HT Wickramasinghe and Mr Chua Hock Beng of RIPAS Hospital for their advice in this case. REFERENCES
1Kelsall AWR. Practical procedures. In: Rennie JM, Roberton NRC, eds. Textbook of Neonatology, 3rd edn. Edinburgh, Scotland: Churchill Livingstone, 1999; 1376.
2Nour S, Puntis JWL, Stringer MD. Intra-abdominal extravasation complicating parenteral nutrition in infants. Arch. Dis. Child.
Fetal Neonatal Ed. 1995; 72: F205–6.
3Baker J, Imong S. A rare complication of neonatal central venous access. Arch. Dis. Child. Fetal Neonatal Ed. 2002; 86: F61–F62.
S Chandran
E Chong
Neonatal Unit
RIPAS Hospital
Brunei Darussalem
720
Letters to the Editor
28 July 2003
Dear Editor,
A NOVEL WAY TO DIAGNOSE CYSTIC FIBROSIS IN THE NEONATE WITH A BOWEL OBSTRUCTION AND
POSSIBLE MECONIUM ILEUS Paediatric surgeons and neonatologists are often confronted with a newborn baby with small bowel obstruction and sus-pected meconium ileus. Cystic fibrosis is an important and likely cause, but molecular diagnosis is rarely available in babies before intervention is required. If there were a simple clinical test for cystic fibrosis, it would confirm a diagnosis of meconium ileus even before a barium enema, and would alert the anaesthetist and surgeon.
There is a simple and reliable physical sign of cystic fibrosis in the male, that is, regression of the vas deferens. It has been well-documented that the majority of men with cystic fibrosis have congenital bilateral absence of the vas deferens (CBAVD).However, CBAVD has been considered in recent years as an attenuated form of cystic fibrosis. 1 This theory is based on the discovery of the cystic fibrosis transmembrane regulator (CFTR)gene mutation in CBAVD.
Cystic fibrosis and CBAVD share a common genetic and embryological origin. Cystic fibrosis is an autosomal recessive disease resulting from mutation in the CFTR, a gene located on
chromosome 7. The CFTR protein functions as a cyclic AMP-regulated chloride channel. CFTR mutations lead to degrada-tion or malfunctioning of the protein, resulting in absent or defective chloride ion transport. 2 Recently, these mutations have also been recognized in CBAVD, with 75–80% of CBAVD patients having a CFTR gene defect. 2–4 The remaining group, without any recognizable CFTR mutation, is believed to have renal tract anomalies not associated with cystic fibrosis,known as Mayer-Kuster–Hauser–Rokitansky syndrome. 3,4
There are two main theories to the pathogenesis of CBAVD in cystic fibrosis, based on the concept of defective chloride excretion. There is either growth disturbance of the Wolffian duct derivatives, which are more susceptible to defective chlor-ide transport, or there is a process of atrophy due to obstruction of the ductal structures by viscous secretions. In the normal fetus, the Wolffian duct forms the epididymis, vas deferens and seminal vesicle under the control of gonadal androgens. By contrast, in fetuses with cystic fibrosis, the caudal epididymis and vas deferens undergo involution in mid-trimester. 3 T he caput epididymis is spared from the pathophysiologic insult that affects the development of the distal two-thirds of the epididymis, the vas deferens, and the seminal vesicles, due to its derivation from a different embryologic precursor. 5
At birth, the head of the epididymis is palpable, but the caudal epididymis and vas deferens are absent. The latter is readily determined by careful palpation of the neck of the scrotum, where the vas and gonadal vessels normally can be felt as two separate structures. The vas feels like a thin cord which can be rolled between the finger and thumb (Fig. 1).Bilateral absence of the vas deferens at the scrotal neck,combined with absence of the caudal epididymis, is a simple,quick and reliable physical sign of cystic fibrosis. This is true especially when a neonate presents with bowel obstruction –one only has to feel the external male genitalia, and the diagnosis of meconium ileus can be made. However, there is one drawback – it can be applied to only half the population!
REFERENCES
1Kugler A, Laccone F, Weider W, Kallerhoff M. Congenital agenesis of the vas deferens and cystic fibrosis. Urology 1995; 34 :348–50.
2
Dohle G, Veeze H, Overbeek S et al. The complex relationships between cystic fibrosis and congenital bilateral absence of the vas deferens: clinical, electrophysiological and genetic data. Human Reprod. 1992; 14 : 371–4.
3Chillon M, Casals T, Mercier B et al. Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens. N. Engl. J. Med. 1995; 332 : 1475–80.
4
McCallum T, Milunsky J, Munarriz R, Carson R, Sadeghi-Nejad H, Oates R. Unilateral renal agenesis associated with con-genital bilateral absence of the vas deferens: phenotypic findings and genetic considerations. Human Reprod. 2001; 16 : 282–8.
5
McCallum T, Milunsky J, Cunningham D, Harris D, Maher T,Oates R. Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes. Chest 2000; 118 : 1059–62.
V Sung JM Hutson
Department of General Surgery
Royal Children’s Hospital,
Parkville, Victoria
Australia
Fig. 1
Illustration of the scrotal examination of a neonate. (a) Normal scrotal neck examination, with the vas and the gonadal vessels palpable as two separate structures. (b) Scrotal examination of a neonate with cystic fibrosis, with the vas and the tail of the epididymis missing, and
the epididymal caput enlarged.
Letters to the Editor721
8 July 2003 Dear Editor,
TICKLE THERAPY
Most infants and children respond to tickling with a giggle and laughter.
There are only six references made to tickling in MEDLINE. Two of these relate to the tickle being used to reward rats for desirable behaviour in the laboratory.2,3
Four others address why we cannot successfully tickle ourselves. Apparently effective tickling requires the ‘sense of unpredictability’ in the subject. Thus only externally produced stimuli can elicit a tickle response.1,4,5,6
This is meagre research on a ‘tool’ that appears to have important diagnostic as well as therapeutic applications. I have found that tickling children can be used at least in the following two ways:
1An infant or child who has the capacity to respond posi-tively to a tickle cannot be too sick. The patient is unlikely to have a serious condition such as septicaemia, intussus-ception, meningitis, etc.
2Tickling is an engaging and effective way to carry out chest physiotherapy. Vigorous laughter is almost always followed by a good productive cough.
I recently came across a 6-year-old child with cystic fibrosis who has been having a lot of fun receiving twice a day tickling. Her mother had intuitively found that this was a good way to help her child cough.
It seems there are many other intuitive and engaging diag-nostic and therapeutic modalities that have received insufficient attention. The use of magic tricks, humour, charm, entertain-ment, jokes and story telling must find their rightful place in our human interactions with children.
REFERENCES
1Harris CR, Christenfeld N. Can a machine tickle? Psychon. Bull.
Rev. 1999; 6: 504–10.
2Burgdorf J, Panksepp J. Tickling induces reward in adolescent rats. Physiol. Behav. 2001; 72: 167–73.
3Panksepp J, Burgdorf J. 50-kHz chirping (laughter?) in response to conditioned and unconditioned tickle-induced rewards in rats: effects of social housing and genetic variables. Behav. Brain Res.
2000; 115: 25–38.
4Claxton G. Why can’t we tickle ourselves? Percept. Mot. Skills 1975; 41: 335–8.
5Chronicle EP, Glover J. A ticklish question: does magnetic stimu-lation of the primary motor cortex give rise to an ‘efference copy’?
Cortex 2003; 39: 105–10.
6Blakemore SJ, Wolpert DM, Frith CD. Central cancellation of self-produced tickle sensation. Nat. Neurosci. 1998; 1: 635–40.
H Zehnwirth
Consultant Paediatrician
Ballarat, Victoria
Australia。