418
anatomical relationship between prop forwards scrummaging suggests the probable causative factors. The authors' series demonstrates that these lesions are treatable with excision and direct closure, resulting in good cosmesis, patient satisfaction, minimal complications and no recurrence.
References 1. Dearing J. Soft tissue neck lumps in rugby union players. Br. J. Sports Med. 2006; 40: 317–319. 2. Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 4th edn. St Louis: Mosby, 2001. 3. Michal M, Fetsch JF, Hes O, Miettinen M. Nuchal‐type fibroma: a clinicopathological study of 52 cases. Cancer 1999; 85: 156–163. 4. Balachandran K, Allen PW, MacCormac LB. Nuchal fibroma. A clinicopathological study of nine cases. Am. J. Surg. Pathol. 1995; 19: 313–317. 5. O'Connell JX, Janzen DL, Hughes TR. Nuchal fibrocartilaginous pseudotumor: a distinctive soft‐tissue lesion associated with prior neck injury. Am. J. Surg. Pathol. 1997; 21: 836–840.
Images for surgeons
6. Laskin WB, Fetsch JF, Miettinen M. Nuchal fibrocartilaginous pseudotumor: a clinicopathologic study of five cases and review of the literature. Mod. Pathol. 1999; 12: 663–668. 7. Nicoletti GF, Platania N, Cicero S, Furnari M, Albanese V. Nuchal fibrocartilaginous pseudotumor. Case report and review of the literature. J. Neurosurg. Sci. 2003; 47: 173–175; discussion 175.
Duncan Robert Bayne,* FRCS (Plast) James Combes,† FRCS (Maxfac) Ankur Pandya,‡ FRCS (Plast) *Department of Plastic Surgery, Queen Victoria Hospital NHS Foundation Trust, East Grinstead, West Sussex, UK, †Maxillofacial Surgery, Royal Surrey County Hospital, Guildford, Surrey, UK and ‡Mountbatten Department of Plastic and Reconstructive Surgery, Queen Alexandra Hospital, Portsmouth, Hampshire, UK doi: 10.1111/ans.12697
Colonic perforation in a neonate with an anorectal malformation A 1‐day‐old male was transferred with abdominal distension secondary to an anorectal malformation (ARM). At 24 h of life he was spontaneously breathing in room air. He displayed features of Down's syndrome (confirmed on FISH). His soft, non‐tender abdomen was mildly distended, with palpable bowel loops. Perineal examination revealed no evidence of an anal opening nor meconium in the urine. Abdominal radiograph (26 h) demonstrated dilated bowel loops, with a prominent right lower quadrant loop (29 mm). Management overnight included antibiotics and nasogastric decompression, with laparotomy planned for the following morning. Examination in the morning was consistent with a distal bowel obstruction, with a moderately distended, non‐tender abdomen. Prone cross‐table radiograph (39 h) demonstrated a gas‐filled rectum, with 26‐mm maximal diameter (Fig. 1). Laparotomy, delayed by preoperative echocardiogram, occurred at 46 h. The delay resulted in subtle clinical changes 2 h pre‐laparotomy (unsettled with handling, difficult arterial access). At laparotomy, turbid peritoneal fluid was encountered, the sigmoid colon was not as dilated as expected, and the intraperitoneal rectum was perforated. The anti‐mesenteric taenia coli had split longitudinally for 4 cm, displaying prolapsed mucosa (Fig. 2). Following decompression and washout distally, the taenia coli was repaired (interrupted Vicryl). Divided stomas were formed and post‐operative course was uneventful. Distal colostogram, delayed 6 weeks to ensure healing, confirmed no rectourethral fistula (distal bowel 20 mm from perineal skin), colonic stricture nor diverticulum. Intestinal perforation in a neonate with an ARM is a rare but potentially life‐threatening event. Mortality rates may exceed 50%
in premature neonates, or neonates with associated anomalies.1 Spontaneous perforation rates of 2–6% are associated with home births, poor access to neonatal care, co‐morbidities (positive pressure ventilation), sepsis and/or fistula absence.2,3 However, the most common risk factor is delay in diagnosis (defined as greater than 48 h from birth), with rates up to 9.5%.2 This is particularly concerning as delayed diagnosis is common, affecting up to 50% of patients in some series.4,5 Reluctance to use rectal thermometers in neonates may have led to an increased rate of missed ARM.5
Fig. 1. Prone cross‐table abdominal radiograph performed at 39 h of age. A gas‐filled terminal rectum is demonstrated, with a maximal rectal diameter of 26 mm and a distance of 14 mm to the perineal marker.
© 2014 Royal Australasian College of Surgeons
Images for surgeons
419
One of the monozygotic twins (both with ARM without fistula) perforated within 24 h, thus highlighting the need for extra vigilance in syndromic patients. Perhaps hypotonia and poor reactivity seen in syndromic patients may disguise the early clinical signs of perforation. Clinicians must be aware of the signs of deterioration in any neonate and, if suspicious, laparotomy and formation of stomas should be expedited.
References
Fig. 2. Intraoperative photograph of the divided sigmoid colon. A 4‐cm longitudinal split in the anti‐mesenteric taenia coli has resulted in prolapse of the underlying mucosa. The proximal sigmoid colon stoma is situated medially.
Abdominal radiographs are the mainstay of diagnosis in perforation. Typical features are pneumoperitoneum, absence of intraluminal gas, Rigler's sign and/or pneumoscrotum. However, as in our case, radiographic features may be absent if perforation occurs in the period of waiting for laparotomy. Careful abdominal examination, particularly assessing for signs of distension, is critical. If possible, stomas should be formed within 36 h of birth.6 In concordance with our patient, other investigators have noted that the rectosigmoid is affected in more than 60% of cases.3 Proposed aetiologies for distal perforation include a competent ileocaecal valve forming a closed loop obstruction (with resultant increase in wall pressure in the most dilated segment of bowel, as per Laplace's law) or a ‘field defect’ abnormality in the distal intestine. Interestingly, the actual calibre of the distal intestine in our patient was not consistent with a previously overly distended loop. Intestinal perforation in the setting of ARM and Down's syndrome has been previously described by de Buys Roessingh et al.7
© 2014 Royal Australasian College of Surgeons
1. Digray NC, Mengi Y, Goswamy HL, Thappa DR. Colorectal perforations in neonates with anorectal malformations. Pediatr. Surg. Int. 2001; 17: 42–44. 2. Turowski C, Dingemann J, Gillick J. Delayed diagnosis of imperforate anus: an unacceptable mortality. Pediatr. Surg. Int. 2010; 26: 1083–1086. 3. Raveenthiran V. Spontaneous perforation of the colon and rectum complicating anorectal malformations in neonates. J. Pediatr. Surg. 2012; 47: 720–726. 4. Haider N, Fisher R. Mortality and morbidity associated with late diagnosis of anorectal malformations in children. Surgeon. 2007; 5: 327–330. 5. Wilson BE, Etheridge CE, Soundappan SVS, Holland AJA. Delayed diagnosis of anorectal malformations: are current guidelines sufficient? J. Paediatr. Child Health 2010; 46: 268–272. 6. Levitt MA, Pena A. Imperforate anus and cloacal malformations. In: Holcomb GW, Murphy JP (eds). Ashcraft's Pediatric Surgery, 5th edn. Philadelphia: Saunders Elsevier, 2010; 468–490. 7. de Buys Roessingh AS, Mueller C, Wiesenauer C, Bensoussan AL, Beaunoyer M. Anorectal malformation and Down's syndrome in monozygotic twins. J. Pediatr. Surg. 2009; 44: E13–16.
Sebastian K. King,*† PhD, FRACS Rebecca Cooksey,* MBBS John Atkinson,* MBChB Elizabeth McLeod,* MD, FRACS Marc Levitt,‡ MD, FACS *Department of Paediatric and Neonatal Surgery, The Royal Children's Hospital, Melbourne, Victoria, Australia, †Department of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia and ‡Colorectal Center, Cincinnati Children's Hospital, Cincinnati, Ohio, USA doi: 10.1111/ans.12704
anatomical relationship between prop forwards scrummaging suggests the probable causative factors. The authors' series demonstrates that these lesions are treatable with excision and direct closure, resulting in good cosmesis, patient satisfaction, minimal complications and no recurrence.
References 1. Dearing J. Soft tissue neck lumps in rugby union players. Br. J. Sports Med. 2006; 40: 317–319. 2. Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 4th edn. St Louis: Mosby, 2001. 3. Michal M, Fetsch JF, Hes O, Miettinen M. Nuchal‐type fibroma: a clinicopathological study of 52 cases. Cancer 1999; 85: 156–163. 4. Balachandran K, Allen PW, MacCormac LB. Nuchal fibroma. A clinicopathological study of nine cases. Am. J. Surg. Pathol. 1995; 19: 313–317. 5. O'Connell JX, Janzen DL, Hughes TR. Nuchal fibrocartilaginous pseudotumor: a distinctive soft‐tissue lesion associated with prior neck injury. Am. J. Surg. Pathol. 1997; 21: 836–840.
Images for surgeons
6. Laskin WB, Fetsch JF, Miettinen M. Nuchal fibrocartilaginous pseudotumor: a clinicopathologic study of five cases and review of the literature. Mod. Pathol. 1999; 12: 663–668. 7. Nicoletti GF, Platania N, Cicero S, Furnari M, Albanese V. Nuchal fibrocartilaginous pseudotumor. Case report and review of the literature. J. Neurosurg. Sci. 2003; 47: 173–175; discussion 175.
Duncan Robert Bayne,* FRCS (Plast) James Combes,† FRCS (Maxfac) Ankur Pandya,‡ FRCS (Plast) *Department of Plastic Surgery, Queen Victoria Hospital NHS Foundation Trust, East Grinstead, West Sussex, UK, †Maxillofacial Surgery, Royal Surrey County Hospital, Guildford, Surrey, UK and ‡Mountbatten Department of Plastic and Reconstructive Surgery, Queen Alexandra Hospital, Portsmouth, Hampshire, UK doi: 10.1111/ans.12697
Colonic perforation in a neonate with an anorectal malformation A 1‐day‐old male was transferred with abdominal distension secondary to an anorectal malformation (ARM). At 24 h of life he was spontaneously breathing in room air. He displayed features of Down's syndrome (confirmed on FISH). His soft, non‐tender abdomen was mildly distended, with palpable bowel loops. Perineal examination revealed no evidence of an anal opening nor meconium in the urine. Abdominal radiograph (26 h) demonstrated dilated bowel loops, with a prominent right lower quadrant loop (29 mm). Management overnight included antibiotics and nasogastric decompression, with laparotomy planned for the following morning. Examination in the morning was consistent with a distal bowel obstruction, with a moderately distended, non‐tender abdomen. Prone cross‐table radiograph (39 h) demonstrated a gas‐filled rectum, with 26‐mm maximal diameter (Fig. 1). Laparotomy, delayed by preoperative echocardiogram, occurred at 46 h. The delay resulted in subtle clinical changes 2 h pre‐laparotomy (unsettled with handling, difficult arterial access). At laparotomy, turbid peritoneal fluid was encountered, the sigmoid colon was not as dilated as expected, and the intraperitoneal rectum was perforated. The anti‐mesenteric taenia coli had split longitudinally for 4 cm, displaying prolapsed mucosa (Fig. 2). Following decompression and washout distally, the taenia coli was repaired (interrupted Vicryl). Divided stomas were formed and post‐operative course was uneventful. Distal colostogram, delayed 6 weeks to ensure healing, confirmed no rectourethral fistula (distal bowel 20 mm from perineal skin), colonic stricture nor diverticulum. Intestinal perforation in a neonate with an ARM is a rare but potentially life‐threatening event. Mortality rates may exceed 50%
in premature neonates, or neonates with associated anomalies.1 Spontaneous perforation rates of 2–6% are associated with home births, poor access to neonatal care, co‐morbidities (positive pressure ventilation), sepsis and/or fistula absence.2,3 However, the most common risk factor is delay in diagnosis (defined as greater than 48 h from birth), with rates up to 9.5%.2 This is particularly concerning as delayed diagnosis is common, affecting up to 50% of patients in some series.4,5 Reluctance to use rectal thermometers in neonates may have led to an increased rate of missed ARM.5
Fig. 1. Prone cross‐table abdominal radiograph performed at 39 h of age. A gas‐filled terminal rectum is demonstrated, with a maximal rectal diameter of 26 mm and a distance of 14 mm to the perineal marker.
© 2014 Royal Australasian College of Surgeons
Images for surgeons
419
One of the monozygotic twins (both with ARM without fistula) perforated within 24 h, thus highlighting the need for extra vigilance in syndromic patients. Perhaps hypotonia and poor reactivity seen in syndromic patients may disguise the early clinical signs of perforation. Clinicians must be aware of the signs of deterioration in any neonate and, if suspicious, laparotomy and formation of stomas should be expedited.
References
Fig. 2. Intraoperative photograph of the divided sigmoid colon. A 4‐cm longitudinal split in the anti‐mesenteric taenia coli has resulted in prolapse of the underlying mucosa. The proximal sigmoid colon stoma is situated medially.
Abdominal radiographs are the mainstay of diagnosis in perforation. Typical features are pneumoperitoneum, absence of intraluminal gas, Rigler's sign and/or pneumoscrotum. However, as in our case, radiographic features may be absent if perforation occurs in the period of waiting for laparotomy. Careful abdominal examination, particularly assessing for signs of distension, is critical. If possible, stomas should be formed within 36 h of birth.6 In concordance with our patient, other investigators have noted that the rectosigmoid is affected in more than 60% of cases.3 Proposed aetiologies for distal perforation include a competent ileocaecal valve forming a closed loop obstruction (with resultant increase in wall pressure in the most dilated segment of bowel, as per Laplace's law) or a ‘field defect’ abnormality in the distal intestine. Interestingly, the actual calibre of the distal intestine in our patient was not consistent with a previously overly distended loop. Intestinal perforation in the setting of ARM and Down's syndrome has been previously described by de Buys Roessingh et al.7
© 2014 Royal Australasian College of Surgeons
1. Digray NC, Mengi Y, Goswamy HL, Thappa DR. Colorectal perforations in neonates with anorectal malformations. Pediatr. Surg. Int. 2001; 17: 42–44. 2. Turowski C, Dingemann J, Gillick J. Delayed diagnosis of imperforate anus: an unacceptable mortality. Pediatr. Surg. Int. 2010; 26: 1083–1086. 3. Raveenthiran V. Spontaneous perforation of the colon and rectum complicating anorectal malformations in neonates. J. Pediatr. Surg. 2012; 47: 720–726. 4. Haider N, Fisher R. Mortality and morbidity associated with late diagnosis of anorectal malformations in children. Surgeon. 2007; 5: 327–330. 5. Wilson BE, Etheridge CE, Soundappan SVS, Holland AJA. Delayed diagnosis of anorectal malformations: are current guidelines sufficient? J. Paediatr. Child Health 2010; 46: 268–272. 6. Levitt MA, Pena A. Imperforate anus and cloacal malformations. In: Holcomb GW, Murphy JP (eds). Ashcraft's Pediatric Surgery, 5th edn. Philadelphia: Saunders Elsevier, 2010; 468–490. 7. de Buys Roessingh AS, Mueller C, Wiesenauer C, Bensoussan AL, Beaunoyer M. Anorectal malformation and Down's syndrome in monozygotic twins. J. Pediatr. Surg. 2009; 44: E13–16.
Sebastian K. King,*† PhD, FRACS Rebecca Cooksey,* MBBS John Atkinson,* MBChB Elizabeth McLeod,* MD, FRACS Marc Levitt,‡ MD, FACS *Department of Paediatric and Neonatal Surgery, The Royal Children's Hospital, Melbourne, Victoria, Australia, †Department of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia and ‡Colorectal Center, Cincinnati Children's Hospital, Cincinnati, Ohio, USA doi: 10.1111/ans.12704
