Images for surgeons
416
Table 1 Aetiology of cervical emphysema Raised intrathoracic pressure
Pulmonary disorders
Iatrogenic
Traumatic Idiopathic
Positive pressure ventilation Valsalva/Heimlich manoeuvre SCUBA diving Labour Excessive coughing/blowing Pneumonia Bronchiolitis Asthma Bronchoscopy Oesophagogastroscopy Tooth extraction Blunt trauma Penetrating trauma —
auscultation and Hamman's sign (precordial crepitation) when pneumomediastinum is also present.2,8 Initial assessment of the patient should include soft tissue radiographs of the neck and chest. This allows the volume and extension of gas to be observed and may reveal underlying pathological conditions. Computed tomography scan of the neck and chest gives greater detail. The clinical course for patients presenting with spontaneous cervical emphysema is usually self‐limiting.2,3,8 However, it is important to be aware that both these patients and those with other aetiologies have the potential to develop life‐threatening complications, for example, airway compromise or tension pneumothorax.3 Management requires only analgesia and avoidance of activities associated with increased intrathoracic pressure (straining, coughing, vigorous exercise) in the majority of cases. Treatment with supplemental oxygen has been suggested as being associated with a more rapid recovery.3 Antibiotic treatment is not indicated unless specific risk of infection exists.2,3,8 In severe or unresolving cases, emergent consultation with head and neck and intensive care specialists is indicated. In these cases,
tracheostomy with or without opening of fascial planes has been suggested.2,9
References 1. George P, Sundara Bhat K. Acute odynophagia – rare clinical presentation of spontaneous cervical emphysema. J. Clin. Diagn. Res. 2011; 5: 1496. 2. Choo MJ, Shin SO, Kim JS. A case of spontaneous cervical and mediastinal emphysema. J. Korean Med. Sci. 1998; 13: 223–226. 3. Parker GS, Mosborg DA, Foley RW, Stiernberg CM. Spontaneous cervical and mediastinal emphysema. Laryngoscope 1990; 100: 938–940. 4. Harley EH. Spontaneous cervical and mediastinal emphysema in asthma. Arch. Otolaryngol. Head Neck Surg. 1987; 113: 1111–1112. 5. Granich MS, Klotz RE, Lofgren RH, Partlow RC, DiGregorio LI. Spontaneous retropharyngeal and cervical subcutaneous emphysema in adults. Arch. Otolaryngol. 1983; 109: 701–704. 6. Munsell WP. Pneumomediastinum: a report of 28 cases and review of the literature. JAMA 1967; 202: 689–693. 7. Macklin CC. Transport of air along sheaths of pulmonary blood vessels from alveoli to mediastinum: clinical implications. Arch. Intern. Med. 1939; 64: 913–926. 8. Dekel B. Spontaneous pneumomediastinum in children; clinical and natural history. Eur. J. Paediar. 1996; 155: 695–697. 9. Herlan DB, Landreneau RJ, Ferson PF. Massive spontaneous subcutaneous emphysema. Acute management with infraclavicular ‘Blow Holes’. Chest 1992; 102: 503–512.
Neil Lowrie,* MBChB, MRCS James Smith,† MBBS Christopher Low,‡ FRCS *General Surgery, Lakes DHB, Rotorua, New Zealand, †Emergency Medicine, Lakes DHB, Rotorua, New Zealand and ‡ENT, Lakes DHB, Rotorua, New Zealand doi: 10.1111/ans.12719
Nuchal fibrolipoma gives rugby players the ‘hump’ Four healthy male rugby prop forwards aged 38–48 were seen by the authors within a period of 8 months, all with similar looking ‘humps’ present for many years on the back of their necks (Fig. 1). These were cosmetically disfiguring, being visible through their clothing and causing mild discomfort after playing; their symptoms partially regressed in the off‐season. Clinically, the lesions were firm to palpate and attached to the skin over the C6‐T1 region ranging from 8 to 14 cm in diameter. The first patient had an ultrasound scan yielding no specific diagnostic data, followed by magnetic resonance imaging (MRI) demonstrating an increased thickness of subcutaneous fat (Fig. 2). The MRI of another patient was remarkably similar with neither showing nuchal ligament involvement. Liposuction was considered but the lesions firmness indicated a significant fibrous component that would disrupt the cannula
passage, yield and predictability of this technique. Open excisions cosmetically placed below the collar line were performed under general anaesthetic, the tough fibrous tissue, lack of capsule and unexpected vascularity required sharp and diathermy dissection contrasting greatly from a standard lipoma removal. Excess overlying skin was excised in two cases, optimizing their cosmetic correction. There were no major post‐operative complications, one patient had a seroma aspirated twice; in all cases, the scarring was acceptable (Fig. 3), with no recurrence beyond 12 months. All four masses were confirmed as fibrolipoma at primary and secondary histology review with exclusion of nuchal fibromas and nuchal fibrocartilaginous pseudo‐tumours. In 2006, Dearing reported abandoning mid‐operation a similar ‘humpectomy’ because of his concerns that removal would leave a
© 2014 Royal Australasian College of Surgeons
Images for surgeons
417
Fig. 3. Post‐operative oblique view showing minimal scarring.
Fig. 1. Lateral preoperative view of typical rugby nuchal ‘hump’.
Fig. 2. Sagittal magnetic resonance imaging of nuchal ‘hump’.
defect needing skin grafting, which might end the players career. He also surveyed in a national rugby magazine prop forwards identifying nine further players who had surgery; two‐thirds of their lesions were lipomas, the others fibromas.1 Two other distinct lesions occur almost exclusively in the midline of the posterior cervical region; the nuchal fibroma and the nuchal fibrocartilaginous pseudo‐tumour. © 2014 Royal Australasian College of Surgeons
In 1988, Enzinger and Weiss2 described the nuchal fibroma as a rare lesion usually occurring between 25 and 60 years old in the paraspinal and interscapular regions. Histologically, these show broad bundles of collagen with few fibrocytes, the absence of a capsule and minimal adipose tissue distinguishes them from a fibrolipoma. Michal et al.3 retrospectively analysed 52 cases of nuchal fibroma, 44% of the patients in their series had diabetes mellitus. Balachandran et al.4 reviewed this lesion in nine patients, presenting as non‐tender, firm, fibrofatty masses from 2.5 to 8 cm in diameter, with entrapped nerve fibres in eight out of the nine cases, so excluding a diagnosis of fibrolipoma. None of these nuchal fibroma patients had a history of trauma. Nuchal fibrocartilaginous pseudo‐tumours arise from proliferation of the nuchal ligament, probably in response to injury, termed by O'Connell et al.5 The lesions are smaller, sometimes painful and almost invariably associated with a history of soft tissue injury such as whiplash; histologically, they arise within the nuchal ligament5,6 and are thought to be post‐traumatic metaplasia by Nicoletti et al.7 The ‘buffalo hump’ deformity seen in states with raised cortisol levels is an enlargement of the dorsocervical fat pad, consisting of mature fat, and is unlike the fibrolipomatous lesions described.2 None of these lesions fits with the clinical and histological findings from the four patients in this series. There is relatively little protection for the spinous processes of the lower cervical vertebrae especially in flexion when propping, despite the aim of the prop to straighten his or her neck as much as possible to disrupt the opposition scrum. We hypothesize that the repetitive force and frictional movements involved in scrummaging generates shearing and trauma that provokes a reactive response by the posterior neck subcutaneous tissue, leading to the lesions. These four patients have helped to establish a definitive clinical entity of nuchal fibrolipoma, resulting from the playing of rugby football union in the position of prop forward. Their distinct characteristics differentiate it from simple lipomas, fibromas, nuchal fibromas and nuchal fibrocartilaginous pseudo‐tumours. The intricate
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
416
Table 1 Aetiology of cervical emphysema Raised intrathoracic pressure
Pulmonary disorders
Iatrogenic
Traumatic Idiopathic
Positive pressure ventilation Valsalva/Heimlich manoeuvre SCUBA diving Labour Excessive coughing/blowing Pneumonia Bronchiolitis Asthma Bronchoscopy Oesophagogastroscopy Tooth extraction Blunt trauma Penetrating trauma —
auscultation and Hamman's sign (precordial crepitation) when pneumomediastinum is also present.2,8 Initial assessment of the patient should include soft tissue radiographs of the neck and chest. This allows the volume and extension of gas to be observed and may reveal underlying pathological conditions. Computed tomography scan of the neck and chest gives greater detail. The clinical course for patients presenting with spontaneous cervical emphysema is usually self‐limiting.2,3,8 However, it is important to be aware that both these patients and those with other aetiologies have the potential to develop life‐threatening complications, for example, airway compromise or tension pneumothorax.3 Management requires only analgesia and avoidance of activities associated with increased intrathoracic pressure (straining, coughing, vigorous exercise) in the majority of cases. Treatment with supplemental oxygen has been suggested as being associated with a more rapid recovery.3 Antibiotic treatment is not indicated unless specific risk of infection exists.2,3,8 In severe or unresolving cases, emergent consultation with head and neck and intensive care specialists is indicated. In these cases,
tracheostomy with or without opening of fascial planes has been suggested.2,9
References 1. George P, Sundara Bhat K. Acute odynophagia – rare clinical presentation of spontaneous cervical emphysema. J. Clin. Diagn. Res. 2011; 5: 1496. 2. Choo MJ, Shin SO, Kim JS. A case of spontaneous cervical and mediastinal emphysema. J. Korean Med. Sci. 1998; 13: 223–226. 3. Parker GS, Mosborg DA, Foley RW, Stiernberg CM. Spontaneous cervical and mediastinal emphysema. Laryngoscope 1990; 100: 938–940. 4. Harley EH. Spontaneous cervical and mediastinal emphysema in asthma. Arch. Otolaryngol. Head Neck Surg. 1987; 113: 1111–1112. 5. Granich MS, Klotz RE, Lofgren RH, Partlow RC, DiGregorio LI. Spontaneous retropharyngeal and cervical subcutaneous emphysema in adults. Arch. Otolaryngol. 1983; 109: 701–704. 6. Munsell WP. Pneumomediastinum: a report of 28 cases and review of the literature. JAMA 1967; 202: 689–693. 7. Macklin CC. Transport of air along sheaths of pulmonary blood vessels from alveoli to mediastinum: clinical implications. Arch. Intern. Med. 1939; 64: 913–926. 8. Dekel B. Spontaneous pneumomediastinum in children; clinical and natural history. Eur. J. Paediar. 1996; 155: 695–697. 9. Herlan DB, Landreneau RJ, Ferson PF. Massive spontaneous subcutaneous emphysema. Acute management with infraclavicular ‘Blow Holes’. Chest 1992; 102: 503–512.
Neil Lowrie,* MBChB, MRCS James Smith,† MBBS Christopher Low,‡ FRCS *General Surgery, Lakes DHB, Rotorua, New Zealand, †Emergency Medicine, Lakes DHB, Rotorua, New Zealand and ‡ENT, Lakes DHB, Rotorua, New Zealand doi: 10.1111/ans.12719
Nuchal fibrolipoma gives rugby players the ‘hump’ Four healthy male rugby prop forwards aged 38–48 were seen by the authors within a period of 8 months, all with similar looking ‘humps’ present for many years on the back of their necks (Fig. 1). These were cosmetically disfiguring, being visible through their clothing and causing mild discomfort after playing; their symptoms partially regressed in the off‐season. Clinically, the lesions were firm to palpate and attached to the skin over the C6‐T1 region ranging from 8 to 14 cm in diameter. The first patient had an ultrasound scan yielding no specific diagnostic data, followed by magnetic resonance imaging (MRI) demonstrating an increased thickness of subcutaneous fat (Fig. 2). The MRI of another patient was remarkably similar with neither showing nuchal ligament involvement. Liposuction was considered but the lesions firmness indicated a significant fibrous component that would disrupt the cannula
passage, yield and predictability of this technique. Open excisions cosmetically placed below the collar line were performed under general anaesthetic, the tough fibrous tissue, lack of capsule and unexpected vascularity required sharp and diathermy dissection contrasting greatly from a standard lipoma removal. Excess overlying skin was excised in two cases, optimizing their cosmetic correction. There were no major post‐operative complications, one patient had a seroma aspirated twice; in all cases, the scarring was acceptable (Fig. 3), with no recurrence beyond 12 months. All four masses were confirmed as fibrolipoma at primary and secondary histology review with exclusion of nuchal fibromas and nuchal fibrocartilaginous pseudo‐tumours. In 2006, Dearing reported abandoning mid‐operation a similar ‘humpectomy’ because of his concerns that removal would leave a
© 2014 Royal Australasian College of Surgeons
Images for surgeons
417
Fig. 3. Post‐operative oblique view showing minimal scarring.
Fig. 1. Lateral preoperative view of typical rugby nuchal ‘hump’.
Fig. 2. Sagittal magnetic resonance imaging of nuchal ‘hump’.
defect needing skin grafting, which might end the players career. He also surveyed in a national rugby magazine prop forwards identifying nine further players who had surgery; two‐thirds of their lesions were lipomas, the others fibromas.1 Two other distinct lesions occur almost exclusively in the midline of the posterior cervical region; the nuchal fibroma and the nuchal fibrocartilaginous pseudo‐tumour. © 2014 Royal Australasian College of Surgeons
In 1988, Enzinger and Weiss2 described the nuchal fibroma as a rare lesion usually occurring between 25 and 60 years old in the paraspinal and interscapular regions. Histologically, these show broad bundles of collagen with few fibrocytes, the absence of a capsule and minimal adipose tissue distinguishes them from a fibrolipoma. Michal et al.3 retrospectively analysed 52 cases of nuchal fibroma, 44% of the patients in their series had diabetes mellitus. Balachandran et al.4 reviewed this lesion in nine patients, presenting as non‐tender, firm, fibrofatty masses from 2.5 to 8 cm in diameter, with entrapped nerve fibres in eight out of the nine cases, so excluding a diagnosis of fibrolipoma. None of these nuchal fibroma patients had a history of trauma. Nuchal fibrocartilaginous pseudo‐tumours arise from proliferation of the nuchal ligament, probably in response to injury, termed by O'Connell et al.5 The lesions are smaller, sometimes painful and almost invariably associated with a history of soft tissue injury such as whiplash; histologically, they arise within the nuchal ligament5,6 and are thought to be post‐traumatic metaplasia by Nicoletti et al.7 The ‘buffalo hump’ deformity seen in states with raised cortisol levels is an enlargement of the dorsocervical fat pad, consisting of mature fat, and is unlike the fibrolipomatous lesions described.2 None of these lesions fits with the clinical and histological findings from the four patients in this series. There is relatively little protection for the spinous processes of the lower cervical vertebrae especially in flexion when propping, despite the aim of the prop to straighten his or her neck as much as possible to disrupt the opposition scrum. We hypothesize that the repetitive force and frictional movements involved in scrummaging generates shearing and trauma that provokes a reactive response by the posterior neck subcutaneous tissue, leading to the lesions. These four patients have helped to establish a definitive clinical entity of nuchal fibrolipoma, resulting from the playing of rugby football union in the position of prop forward. Their distinct characteristics differentiate it from simple lipomas, fibromas, nuchal fibromas and nuchal fibrocartilaginous pseudo‐tumours. The intricate
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
