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Achalasia and sudden death: a case report
Forensic Science Intemtiii
Forensic Science International
ELSEVIER
75 (1995)
Achalasia
133-137
and sudden death: a case report
Giovanni
Fassina*,
Antonio
Osculati
Universitci di Pavia, Istituto di Medicina Legale e d&e Assicurazioni, 27100 Pavia Italy Received 6 February
via Forlanini
12,
1995; accepted 2 May 1995
Abstract
A 23-year-old pregnant woman was found dead at home. The anamnesis was completely negative, except for a slight dysphagia. The most important autopsy finding was a remarkable previously undiagnosed, megaesophagus. Conjunctival and pleural petechiae were also observed. The most likely etiopathogenetic factors are considered and discussed. Keywords:
Achalasia,
case report;
Megaesophagus;
Dysphagia;
Sudden
death
1. Introduction Achalasia is a rare idiopathic motility disorder of the esophagus, characterized by the lack of peristalsis at the esophageal body and by the incomplete opening of the lower esophageal sphincter. The most frequent symptom of the disease is a progressively worsening dysphagia involving both liquid and solid food. Moreover, heartburn, regurgitation, chest pain coughing, hoarseness, malnutrition, weight loss and respiratory distress, all secondary to tracheal compression, are sometimes noted [l-11].
A survey of literature revealed just one case of sudden death causally connected with such a disease [12]. In this paper, a case of unexpected death, or rather ‘not observed death’ associated with achalasia is presented. * Corresponding
author
0379-0738/95/$09.50 0 1995 Elsevier Science Ireland Ltd. All rights reserved SSDI 0379-0738(95)01777-G
134
G. Fassina, A. Osculati / Forensic Science International
75 (1995) 133- 137
2. Case report A 23-year-old, 6-month pregnant woman was found lifeless lying on the floor in her bedroom, at 1200 h. Traces of vomiting were evident around her mouth and on her left forearm. At 0800 h she had had a frugal breakfast. She had never shown any symptom before and during her pregnancy, apart from a disregarded slight dysphagia.
2.1. Autopsy jindings The external examination of the body showed conjunctival petechial hemorrhages and the aforesaid traces of vomiting. The inside inspection revealed a considerable enlargement of the esophagus along its whole length, which was also taut and bent (Fig. 1). The circumference of the esophagus was about 14 cm and its mucosa was similar to that found in the gastric tract. Some food material was recovered inside the larynx; the trachea and main bronchi were obstructed by semi-fluid material. The lungs were emphysematous and presented pleural petechial hemorrhages.
2.2. Histological study No ganglion cells were observed. The esophagus walls had hypertrophic muscolaris mucosae; the submucosa was fibrosclerotic; the chorion presented scattered inflammatory cells. The lungs presented an acute emphysema. The microscopic search for foreign material inside the bronchioles and the alveoli with normal and polarized light, as well as with UV light, was negative. This result was confirmed on cryostatic sections using PAS and Sudan III. 3. Discussion At first, the pathological findings did not show an evident cause of death. The obvious hypothesis of an asphyxic death caused by food aspiration was not confirmed, in fact, bronchioles and alveoli did not contain any food particles [13]. An acute respiratory distress due to the compression of the trachea can be proposed as a possible cause of death. Some clinical cases show the possibility of respiratory distress due to the same mechanism [14]. The compression has been recently recognized in patients with achalasia, who lose the normal ‘belch reflex’ and have poor upper esophageal sphincter relaxation in response to insufflation of air into the esophagus [ 15,161. Another hypothesis indicates that the death could be caused by the ‘Valsalva maneuver’ which may be utilized by patients with esophageal obstruction or motility disorders to move ingested food from the oropharynx to the stomach. Disturbances of cardiac rhythm provoked by the Valsalva maneuver are not uncommon [17,18]. In susceptible patients, the dysrhythmias that typically occur after this maneuver are generally attributed to increased myocardial irritability, caused by enhanced vagal tone and myocardial hypoxia. In our case, the anamnesis did not show cardiac rhythm disturbances.
G. Fassina, A. Osculati / Forensic Science International
75 (1995) 133-137
135
A partially satisfactory explanation for the death could be the direct stimulation of the nervus vagus by the walls of the enlarged esophagus, given the closenessof these anatomical formations. This stimulation would lead to severe arrhythmia and cardiac arrest. Moreover, the proximity of the esophagus to the other medyastinic organs (scheme 1 and 2) could also provoke a decreasedvenous return to the heart due to compression of the venae (vv. cavae, vv. pulomonares, etc.). This could produce an atria1 baroceptor stimulation followed by cardiac arrhythmia due to a vagal reflex.
Fig. 1. View of the severely enlarged dilated esophagus. Pethechial hemorrhages on the lung surfaces are present.
136
G. Fassina, A. Osculati /Forensic
Science International
75 (1995) 133-137
. . . . . . . . Scheme 1. Transverse section at the level of fifth thoracic vertebra (TS, vertebra; L, lung; ao, aorta; t, trachea; e, esophagus; va, azygos vein; x, vagus nerve).
3
“P
Scheme 2. Transverse section at the level of seventh thoracic vertebra (T7, vertebra; nl, lymph nodes; b. bronchus; ap, pulmonary artery; vp. pulmonary vein).
G. Fassina, A. Osculati / Forensic Science International
75 (1995) 133-137
137
The suggested mechanism may act in synergism. A single factor cannot explain the full morphological picture. We, therefore postulate the concurrence of several factors, namely vagal stimulation and respiratory insufficiency, both produced by direct compression. This hypothesis could explain the presence of some signs of asphyxia without aspirated food particles. On the other hand, the role of the vagal reflex cannot be demonstrated, because it does not leave any morphological trace. References [l] G.D. Barr and V.S. McDonald, Management of achalasia and laryngo-tracheal compression. J. Laryngol. Otol., 103 (1989) 713-714. [2] B. Carlsson-Nordlander, Acute airway obstruction in a patient with achalasia. Arch. Otolaryngol. Head Neck Surg., 113 (1987) 885-887. [3] M.P. Collins and S. Rabie, Sudden airway obstruction in achalasia. J. Laryngol. Otol., 98 (1984) 2077211. [4] F. Dominguez, F. Hernandez-Ranz and D. Boixeda, Acute upper-airway obstruction in achalasia of the esophagus. Am. J. Gastroenterol., 82 (1987) 362-364. [5] CR. Evans, R. Cawodd and M.W. Dronfield, Achalasia: presentation with stridor and a new form of treatment. Br. Med. J., 285 (1982) 1704. [6] P.E. Giustra, P.J. Killoran and W.N. Wasgat, Acute stridor in achalasia of the esophagus (cardiospasm). Am. J. Gastroenterol., 60 (1973) 160- 164. [7] A.P. Kendall and E. Lin, Respiratory failure as presentation of achalasia of esophagus. Anaesfhesia 46 (1991) 1039-1040. [8] R.D.W. McLean, C.J. Stewart and D.G.C. Whyte, Acute thoracic inlet obstruction in achalasia of the esophagus. Thorax, 31 (1976) 456-459. [9] A. Morrow, T.J. Coady and D. Seatoa, Tracheal compression relieved by cardiomyotomy. Thorax, 37 (1982) 776-777. [IO] K.H. Travis, V.K. Saini and P.T. O’Sullivan Upper-airway obstruction and achalasia of the esophagus. Anesthesiology, 54 (1981) 87-88. [I l] J.L. Westbrook, Esophageal achalasia causing respiratory obstruction. Anaesthesia, 47 (1992) 38-40. [12] K. Sperry, Achalasia, the Valsalva maneuver and sudden death: A case report. J. Forensic Sci. 39 (1994) 547-551. [13] G. Canuto and L. Migliardi, Sulla penetrazione nei bronchi in vita e post-mortem del contenuto gastrico. Arch. Antropol. Crimin., 48 (1928) 870. [I41 L. Panzini and M. Traube, Stridor from tracheal obstruction in a patient with achalasia. Am. J. Gastroenterol., 88 (1993) 1097-2000. [15] D.J. Becker and D.O. Castell, Acute airway obstruction in achalasia: possible role of defective belch reflex. Gastroenterology, 97 (1989) 1323- 1326. [I61 B.T. Massey, W.J. Hogan, W.J. Dodds and R.O. Dantas, Alteration of the upper esophageal sphincter belch reflex in patients with achalasia. Gastroenterology, 103 (1992) 1574- 1579. [17] S. Schartum, Venticular arrest caused by the Valsalva maneuver in a patient with Adams-Stokes attacks acompanyng defecation. Acta Med. Stand., 184 (1968) 65568. [I81 K.G. Tolman and W.D. Ashwort, Syncope induced by disphagia: correction by esophageal dilatation. Am. J. Dig. Dis., I6 (1971) 102661031.
Forensic Science International
ELSEVIER
75 (1995)
Achalasia
133-137
and sudden death: a case report
Giovanni
Fassina*,
Antonio
Osculati
Universitci di Pavia, Istituto di Medicina Legale e d&e Assicurazioni, 27100 Pavia Italy Received 6 February
via Forlanini
12,
1995; accepted 2 May 1995
Abstract
A 23-year-old pregnant woman was found dead at home. The anamnesis was completely negative, except for a slight dysphagia. The most important autopsy finding was a remarkable previously undiagnosed, megaesophagus. Conjunctival and pleural petechiae were also observed. The most likely etiopathogenetic factors are considered and discussed. Keywords:
Achalasia,
case report;
Megaesophagus;
Dysphagia;
Sudden
death
1. Introduction Achalasia is a rare idiopathic motility disorder of the esophagus, characterized by the lack of peristalsis at the esophageal body and by the incomplete opening of the lower esophageal sphincter. The most frequent symptom of the disease is a progressively worsening dysphagia involving both liquid and solid food. Moreover, heartburn, regurgitation, chest pain coughing, hoarseness, malnutrition, weight loss and respiratory distress, all secondary to tracheal compression, are sometimes noted [l-11].
A survey of literature revealed just one case of sudden death causally connected with such a disease [12]. In this paper, a case of unexpected death, or rather ‘not observed death’ associated with achalasia is presented. * Corresponding
author
0379-0738/95/$09.50 0 1995 Elsevier Science Ireland Ltd. All rights reserved SSDI 0379-0738(95)01777-G
134
G. Fassina, A. Osculati / Forensic Science International
75 (1995) 133- 137
2. Case report A 23-year-old, 6-month pregnant woman was found lifeless lying on the floor in her bedroom, at 1200 h. Traces of vomiting were evident around her mouth and on her left forearm. At 0800 h she had had a frugal breakfast. She had never shown any symptom before and during her pregnancy, apart from a disregarded slight dysphagia.
2.1. Autopsy jindings The external examination of the body showed conjunctival petechial hemorrhages and the aforesaid traces of vomiting. The inside inspection revealed a considerable enlargement of the esophagus along its whole length, which was also taut and bent (Fig. 1). The circumference of the esophagus was about 14 cm and its mucosa was similar to that found in the gastric tract. Some food material was recovered inside the larynx; the trachea and main bronchi were obstructed by semi-fluid material. The lungs were emphysematous and presented pleural petechial hemorrhages.
2.2. Histological study No ganglion cells were observed. The esophagus walls had hypertrophic muscolaris mucosae; the submucosa was fibrosclerotic; the chorion presented scattered inflammatory cells. The lungs presented an acute emphysema. The microscopic search for foreign material inside the bronchioles and the alveoli with normal and polarized light, as well as with UV light, was negative. This result was confirmed on cryostatic sections using PAS and Sudan III. 3. Discussion At first, the pathological findings did not show an evident cause of death. The obvious hypothesis of an asphyxic death caused by food aspiration was not confirmed, in fact, bronchioles and alveoli did not contain any food particles [13]. An acute respiratory distress due to the compression of the trachea can be proposed as a possible cause of death. Some clinical cases show the possibility of respiratory distress due to the same mechanism [14]. The compression has been recently recognized in patients with achalasia, who lose the normal ‘belch reflex’ and have poor upper esophageal sphincter relaxation in response to insufflation of air into the esophagus [ 15,161. Another hypothesis indicates that the death could be caused by the ‘Valsalva maneuver’ which may be utilized by patients with esophageal obstruction or motility disorders to move ingested food from the oropharynx to the stomach. Disturbances of cardiac rhythm provoked by the Valsalva maneuver are not uncommon [17,18]. In susceptible patients, the dysrhythmias that typically occur after this maneuver are generally attributed to increased myocardial irritability, caused by enhanced vagal tone and myocardial hypoxia. In our case, the anamnesis did not show cardiac rhythm disturbances.
G. Fassina, A. Osculati / Forensic Science International
75 (1995) 133-137
135
A partially satisfactory explanation for the death could be the direct stimulation of the nervus vagus by the walls of the enlarged esophagus, given the closenessof these anatomical formations. This stimulation would lead to severe arrhythmia and cardiac arrest. Moreover, the proximity of the esophagus to the other medyastinic organs (scheme 1 and 2) could also provoke a decreasedvenous return to the heart due to compression of the venae (vv. cavae, vv. pulomonares, etc.). This could produce an atria1 baroceptor stimulation followed by cardiac arrhythmia due to a vagal reflex.
Fig. 1. View of the severely enlarged dilated esophagus. Pethechial hemorrhages on the lung surfaces are present.
136
G. Fassina, A. Osculati /Forensic
Science International
75 (1995) 133-137
. . . . . . . . Scheme 1. Transverse section at the level of fifth thoracic vertebra (TS, vertebra; L, lung; ao, aorta; t, trachea; e, esophagus; va, azygos vein; x, vagus nerve).
3
“P
Scheme 2. Transverse section at the level of seventh thoracic vertebra (T7, vertebra; nl, lymph nodes; b. bronchus; ap, pulmonary artery; vp. pulmonary vein).
G. Fassina, A. Osculati / Forensic Science International
75 (1995) 133-137
137
The suggested mechanism may act in synergism. A single factor cannot explain the full morphological picture. We, therefore postulate the concurrence of several factors, namely vagal stimulation and respiratory insufficiency, both produced by direct compression. This hypothesis could explain the presence of some signs of asphyxia without aspirated food particles. On the other hand, the role of the vagal reflex cannot be demonstrated, because it does not leave any morphological trace. References [l] G.D. Barr and V.S. McDonald, Management of achalasia and laryngo-tracheal compression. J. Laryngol. Otol., 103 (1989) 713-714. [2] B. Carlsson-Nordlander, Acute airway obstruction in a patient with achalasia. Arch. Otolaryngol. Head Neck Surg., 113 (1987) 885-887. [3] M.P. Collins and S. Rabie, Sudden airway obstruction in achalasia. J. Laryngol. Otol., 98 (1984) 2077211. [4] F. Dominguez, F. Hernandez-Ranz and D. Boixeda, Acute upper-airway obstruction in achalasia of the esophagus. Am. J. Gastroenterol., 82 (1987) 362-364. [5] CR. Evans, R. Cawodd and M.W. Dronfield, Achalasia: presentation with stridor and a new form of treatment. Br. Med. J., 285 (1982) 1704. [6] P.E. Giustra, P.J. Killoran and W.N. Wasgat, Acute stridor in achalasia of the esophagus (cardiospasm). Am. J. Gastroenterol., 60 (1973) 160- 164. [7] A.P. Kendall and E. Lin, Respiratory failure as presentation of achalasia of esophagus. Anaesfhesia 46 (1991) 1039-1040. [8] R.D.W. McLean, C.J. Stewart and D.G.C. Whyte, Acute thoracic inlet obstruction in achalasia of the esophagus. Thorax, 31 (1976) 456-459. [9] A. Morrow, T.J. Coady and D. Seatoa, Tracheal compression relieved by cardiomyotomy. Thorax, 37 (1982) 776-777. [IO] K.H. Travis, V.K. Saini and P.T. O’Sullivan Upper-airway obstruction and achalasia of the esophagus. Anesthesiology, 54 (1981) 87-88. [I l] J.L. Westbrook, Esophageal achalasia causing respiratory obstruction. Anaesthesia, 47 (1992) 38-40. [12] K. Sperry, Achalasia, the Valsalva maneuver and sudden death: A case report. J. Forensic Sci. 39 (1994) 547-551. [13] G. Canuto and L. Migliardi, Sulla penetrazione nei bronchi in vita e post-mortem del contenuto gastrico. Arch. Antropol. Crimin., 48 (1928) 870. [I41 L. Panzini and M. Traube, Stridor from tracheal obstruction in a patient with achalasia. Am. J. Gastroenterol., 88 (1993) 1097-2000. [15] D.J. Becker and D.O. Castell, Acute airway obstruction in achalasia: possible role of defective belch reflex. Gastroenterology, 97 (1989) 1323- 1326. [I61 B.T. Massey, W.J. Hogan, W.J. Dodds and R.O. Dantas, Alteration of the upper esophageal sphincter belch reflex in patients with achalasia. Gastroenterology, 103 (1992) 1574- 1579. [17] S. Schartum, Venticular arrest caused by the Valsalva maneuver in a patient with Adams-Stokes attacks acompanyng defecation. Acta Med. Stand., 184 (1968) 65568. [I81 K.G. Tolman and W.D. Ashwort, Syncope induced by disphagia: correction by esophageal dilatation. Am. J. Dig. Dis., I6 (1971) 102661031.